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Question 1
Correct
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A 55-year-old man is hospitalized after experiencing haematemesis. Upon examination, his prothrombin time is found to be elevated. What could be a possible reason for this anomaly?
Your Answer: Chronic liver disease
Explanation:The inheritance of Haemophilia A and B is crucial in identifying individuals who are at risk of developing the condition. Haemophilia A and B are genetic disorders that are inherited in an X-linked recessive manner. Haemophilia A is caused by a deficiency in clotting factor VIII, while haemophilia B is caused by a deficiency in clotting factor IX.
On the other hand, haemophilia C, which is caused by a deficiency in clotting factor XI, is primarily inherited in an autosomal recessive manner. In X-linked recessive conditions like haemophilia B, males are more likely to be affected than females. This is because males only need one abnormal copy of the gene, which is carried on the X chromosome, to be affected.
Females, on the other hand, can be carriers of the condition if they carry one normal and one abnormal copy of the gene. While carriers can have clotting abnormalities, these are usually milder than those seen in affected individuals. Men cannot pass the condition to their sons, but they will pass on the abnormal X chromosome to all their daughters, who will be carriers.
Female carriers can pass on the condition to around half their sons, and half their daughters will be carriers. Females can only be affected if they are the offspring of an affected male and a carrier female. In summary, the inheritance of haemophilia A and B is crucial in identifying individuals who are at risk of developing the condition. It also helps in providing appropriate genetic counseling and management for affected individuals and their families.
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This question is part of the following fields:
- Haematology And Oncology
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Question 2
Incorrect
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A 50-year-old man comes to surgical outpatients complaining of recurrent abdominal pain and vomiting. During examination, a peripheral motor neuropathy is observed. What is the probable diagnosis?
Your Answer: Myeloma
Correct Answer: Acute intermittent porphyria
Explanation:Unless proven otherwise, the presence of neurological symptoms along with abdominal pain may indicate either acute intermittent porphyria or lead poisoning.
Understanding Acute Intermittent Porphyria
Acute intermittent porphyria (AIP) is a rare genetic disorder that affects the biosynthesis of haem due to a defect in the porphobilinogen deaminase enzyme. This results in the accumulation of delta aminolaevulinic acid and porphobilinogen, leading to a range of symptoms. AIP typically presents in individuals aged 20-40 years, with females being more commonly affected.
The condition is characterized by a combination of abdominal, neurological, and psychiatric symptoms. Abdominal symptoms include pain and vomiting, while neurological symptoms may manifest as motor neuropathy. Psychiatric symptoms may include depression. Hypertension and tachycardia are also common.
Diagnosis of AIP involves a range of tests, including urine analysis, assay of red cells for porphobilinogen deaminase, and measurement of serum levels of delta aminolaevulinic acid and porphobilinogen. A classic sign of AIP is the deep red color of urine on standing.
Management of AIP involves avoiding triggers and treating acute attacks with IV haematin/haem arginate. In cases where these treatments are not immediately available, IV glucose may be used. With proper management, individuals with AIP can lead healthy and fulfilling lives.
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This question is part of the following fields:
- Haematology And Oncology
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Question 3
Incorrect
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A patient presents to the emergency department with shortness of breath and fatigue. Upon examination, a purpuric rash is discovered on their torso, arms, and legs. The initial blood test results are as follows:
Hb 78 g/L Male: (135-180)
Female: (115 - 160)
Platelets 43 * 109/L (150 - 400)
WBC 9.3 * 109/L (4.0 - 11.0)
A blood film reveals numerous fragmented red cells (schistocytes) and marked thrombocytopenia, indicating intravascular hemolysis with high levels of free hemoglobin. To confirm this diagnosis, which of the following additional test results would be helpful?Your Answer: Low lactate dehydrogenase (LDH)
Correct Answer: Low haptoglobins
Explanation:Haptoglobin is a liver-produced protein that binds to free haemoglobin in blood plasma, allowing the reticuloendothelial system to remove it. This consumption of haptoglobin reduces its detectable levels in the blood, making it a useful indicator of haemolysis.
If an individual has a functioning liver, conjugated bilirubin levels will increase in haemolysis. This is because the liver generates conjugated bilirubin from unconjugated bilirubin, which is produced from the porphyrin rings of haemoglobin. Conjugated bilirubin is more soluble in water and can be secreted through the kidneys.
Lactate dehydrogenase is an intracellular enzyme that is leaked from cells, including erythrocytes, which are broken down. Its levels increase due to cell breakdown, not only in haemolysis but also in cardiomyocyte damage due to infarction and lymphocyte turnover due to leukaemia.
Potassium is an intracellular ion that can increase in levels due to haemolysis and cell breakdown. This can lead to cardiac arrhythmias such as ventricular tachycardia and fibrillation.
Low platelets and a purpuric rash suggest that the likely form of intravascular haemolysis is a microangiopathic haemolytic anaemia (MAHA) such as thrombotic thrombocytopenic purpura (TTP) or haemolytic uraemic syndrome (HUS). These rare conditions result in the accumulation of intravascular thrombosis, leading to platelet and clotting factor consumption.
Understanding Haemolytic Anaemias by Site
Haemolytic anaemias can be classified by the site of haemolysis, either intravascular or extravascular. In intravascular haemolysis, free haemoglobin is released and binds to haptoglobin. As haptoglobin becomes saturated, haemoglobin binds to albumin forming methaemalbumin, which can be detected by Schumm’s test. Free haemoglobin is then excreted in the urine as haemoglobinuria and haemosiderinuria. Causes of intravascular haemolysis include mismatched blood transfusion, red cell fragmentation due to heart valves, TTP, DIC, HUS, paroxysmal nocturnal haemoglobinuria, and cold autoimmune haemolytic anaemia.
On the other hand, extravascular haemolysis occurs when red blood cells are destroyed by macrophages in the spleen or liver. This type of haemolysis is commonly seen in haemoglobinopathies such as sickle cell anaemia and thalassaemia, hereditary spherocytosis, haemolytic disease of the newborn, and warm autoimmune haemolytic anaemia.
It is important to understand the site of haemolysis in order to properly diagnose and treat haemolytic anaemias. While both intravascular and extravascular haemolysis can lead to anaemia, the underlying causes and treatment approaches may differ.
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This question is part of the following fields:
- Haematology And Oncology
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Question 4
Correct
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A 28-year-old woman visits her doctor with complaints of fatigue. Upon further inquiry, the doctor learns that she has been experiencing heavy menstrual bleeding for the past 8 months. To investigate further, the doctor orders a complete blood count and iron level test. What is the typical lifespan of a red blood cell?
Your Answer: 120 days
Explanation:The bone marrow in large bones is responsible for the production of human red blood cells through erythropoiesis. Stem cells undergo a 7-day development process to become red blood cells, which then circulate for around 120 days before being eliminated by the spleen. Eryptosis, or programmed red cell death, occurs at the same rate as production.
However, certain diseases can increase the rate of eryptosis, resulting in a shorter lifespan for red blood cells. These diseases include haemolytic uraemic syndrome, sepsis, malaria, sickle cell disease, thalassaemia, iron deficiency, and Wilson’s disease.
Iron deficiency anaemia is a prevalent condition worldwide, with preschool-age children being the most affected. The lack of iron in the body leads to a decrease in red blood cells and haemoglobin, resulting in anaemia. The primary causes of iron deficiency anaemia are excessive blood loss, inadequate dietary intake, poor intestinal absorption, and increased iron requirements. Menorrhagia is the most common cause of blood loss in pre-menopausal women, while gastrointestinal bleeding is the most common cause in men and postmenopausal women. Vegans and vegetarians are more likely to develop iron deficiency anaemia due to the lack of meat in their diet. Coeliac disease and other conditions affecting the small intestine can prevent sufficient iron absorption. Children and pregnant women have increased iron demands, and the latter may experience dilution due to an increase in plasma volume.
The symptoms of iron deficiency anaemia include fatigue, shortness of breath on exertion, palpitations, pallor, nail changes, hair loss, atrophic glossitis, post-cricoid webs, and angular stomatitis. To diagnose iron deficiency anaemia, a full blood count, serum ferritin, total iron-binding capacity, transferrin, and blood film tests are performed. Endoscopy may be necessary to rule out malignancy, especially in males and postmenopausal females with unexplained iron-deficiency anaemia.
The management of iron deficiency anaemia involves identifying and treating the underlying cause. Oral ferrous sulfate is commonly prescribed, and patients should continue taking iron supplements for three months after the iron deficiency has been corrected to replenish iron stores. Iron-rich foods such as dark-green leafy vegetables, meat, and iron-fortified bread can also help. It is crucial to exclude malignancy by taking an adequate history and appropriate investigations if warranted.
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This question is part of the following fields:
- Haematology And Oncology
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Question 5
Incorrect
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A 28-year-old male gives a blood unit that is stored at 4 oC. Which clotting factor will be most impacted after 72 hours?
Your Answer: Factor XI
Correct Answer: Factor V
Explanation:FFP is frozen shortly after collection due to the temperature sensitivity of factors V and VIII.
Blood Products and Cell Saver Devices
Blood products are essential in various medical procedures, especially in cases where patients require transfusions due to anaemia or bleeding. Packed red cells, platelet-rich plasma, platelet concentrate, fresh frozen plasma, and cryoprecipitate are some of the commonly used whole blood fractions. Fresh frozen plasma is usually administered to patients with clotting deficiencies, while cryoprecipitate is a rich source of Factor VIII and fibrinogen. Cross-matching is necessary for all blood products, and cell saver devices are used to collect and re-infuse a patient’s own blood lost during surgery.
Cell saver devices come in two types, those that wash the blood cells before re-infusion and those that do not. The former is more expensive and complicated to operate but reduces the risk of re-infusing contaminated blood. The latter avoids the use of donor blood and may be acceptable to Jehovah’s witnesses. However, it is contraindicated in malignant diseases due to the risk of facilitating disease dissemination.
In some surgical patients, the use of warfarin can pose specific problems and may require the use of specialised blood products. Warfarin reversal can be achieved through the administration of vitamin K, fresh frozen plasma, or human prothrombin complex. Fresh frozen plasma is used less commonly now as a first-line warfarin reversal, and human prothrombin complex is preferred due to its rapid action. However, it should be given with vitamin K as factor 6 has a short half-life.
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This question is part of the following fields:
- Haematology And Oncology
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Question 6
Incorrect
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A 47-year-old woman presents to the Emergency Department with pleuritic chest pain and dyspnoea. Upon examination, an area of painful swelling is found in her right calf, indicating a possible deep vein thrombosis. Her Wells' score is calculated to be 4.2. The patient's vital signs are as follows:
Blood pressure: 105/78 mmHg
Pulse: 118 bpm
Temperature: 37.1ºC
Respiratory rate: 20/min
A CT pulmonary angiography confirms the presence of a right pulmonary embolism. What medication is most likely to be prescribed to this patient?Your Answer: Dalteparin
Correct Answer: Rivaroxaban
Explanation:Rivaroxaban is a direct inhibitor of factor Xa, which is the correct answer. Pulmonary emboli can be caused by various factors, and symptoms include chest pain, dyspnoea, and haemoptysis. Factor Xa inhibitors, such as rivaroxaban, have replaced warfarin as the first-line treatment for stroke prevention in patients with atrial fibrillation.
Dabigatran is a direct thrombin inhibitor and has a different mechanism of action compared to rivaroxaban. It is commonly used for venous thromboembolism prophylaxis after total knee or hip replacement surgery.
Dalteparin is a type of low molecular weight heparin (LMWH) and has a different mechanism of action compared to factor Xa inhibitors. It is used for prophylaxis against venous thromboembolism in patients who are immobile or have recently had surgery.
Fondaparinux is an indirect inhibitor of factor Xa and is not the correct answer. It is used for the treatment of deep-vein thrombosis, pulmonary embolism, and acute coronary syndrome.
Direct oral anticoagulants (DOACs) are medications used to prevent stroke in non-valvular atrial fibrillation (AF), as well as for the prevention and treatment of venous thromboembolism (VTE). To be prescribed DOACs for stroke prevention, patients must have certain risk factors, such as a prior stroke or transient ischaemic attack, age 75 or older, hypertension, diabetes mellitus, or heart failure. There are four DOACs available, each with a different mechanism of action and method of excretion. Dabigatran is a direct thrombin inhibitor, while rivaroxaban, apixaban, and edoxaban are direct factor Xa inhibitors. The majority of DOACs are excreted either through the kidneys or the liver, with the exception of apixaban and edoxaban, which are excreted through the feces. Reversal agents are available for dabigatran and rivaroxaban, but not for apixaban or edoxaban.
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This question is part of the following fields:
- Haematology And Oncology
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Question 7
Incorrect
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A 68-year-old woman has a left ankle ulcer that has been present for nine months. She had a DVT in her right leg five years ago. Upon examination, there is a 6 cm diameter slough-based ulcer on the medial malleolus without cellulitis. What investigation is required before applying compression bandaging?
Your Answer: Bacteriological swab of the ulcer
Correct Answer: Ankle-brachial pressure index
Explanation:Venous Ulceration and the Importance of Identifying Arterial Disease
Venous ulcerations are a common type of ulcer that affects the lower extremities. The underlying cause of venous congestion, which can promote ulceration, is venous insufficiency. The treatment for venous ulceration involves controlling oedema, treating any infection, and compression. However, compressive dressings or devices should not be applied if the arterial circulation is impaired. Therefore, it is crucial to identify any arterial disease, and the ankle-brachial pressure index is a simple way of doing this. If indicated, one may progress to a lower limb arteriogram.
It is important to note that there is no clinical sign of infection, and although a bacterial swab would help to rule out pathogens within the ulcer, arterial insufficiency is the more important issue. If there is a clinical suspicion of DVT, then duplex (or rarely a venogram) is indicated to decide on the indication for anticoagulation. By identifying arterial disease, healthcare professionals can ensure that appropriate treatment is provided and avoid potential complications from compressive dressings or devices.
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This question is part of the following fields:
- Haematology And Oncology
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Question 8
Incorrect
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A 70-year-old man has just undergone an emergency repair for a ruptured abdominal aortic aneurysm. Preoperatively, he was taking aspirin, clopidogrel, and warfarin. Intraoperatively, he received 5000 units of unfractionated heparin before the application of the aortic cross clamp. Upon admission to the critical care unit, his blood results are as follows:
Full blood count
Hb 8 g/dl
Platelets 40 * 109/l
WBC 7.1 * 109/l
His fibrin degradation products are measured and found to be markedly elevated. What is the likely cause of these results?Your Answer: Heparin induced thrombocytopenia
Correct Answer: Disseminated intravascular coagulation
Explanation:DIC is the most probable diagnosis due to the presence of low platelet counts and elevated FDP in this scenario.
Understanding Disseminated Intravascular Coagulation
Under normal conditions, the coagulation and fibrinolysis processes work together to maintain hemostasis. However, in cases of disseminated intravascular coagulation (DIC), these processes become dysregulated, leading to widespread clotting and bleeding. One of the critical factors in the development of DIC is the release of tissue factor (TF), a glycoprotein found on the surface of various cell types. TF is normally not in contact with the circulation but is exposed after vascular damage or in response to cytokines and endotoxins. Once activated, TF triggers the extrinsic pathway of coagulation, leading to the activation of the intrinsic pathway and the formation of clots.
DIC can be caused by various factors, including sepsis, trauma, obstetric complications, and malignancy. Diagnosis of DIC typically involves a blood test that shows decreased platelet count and fibrinogen levels, prolonged prothrombin time and activated partial thromboplastin time, and increased fibrinogen degradation products. Microangiopathic hemolytic anemia may also be present, leading to the formation of schistocytes.
Overall, understanding the pathophysiology and diagnosis of DIC is crucial for prompt and effective management of this potentially life-threatening condition.
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This question is part of the following fields:
- Haematology And Oncology
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Question 9
Correct
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A 65-year-old man presents to the clinic with a complaint of losing 1 stone in weight over the past three months. Apart from this, he has no significant medical history. During the physical examination, his abdomen is soft, and no palpable masses are detected. A normal PR examination is also observed. The patient's blood tests reveal a haemoglobin level of 80 g/L (120-160) and an MCV of 70 fL (80-96). What is the most appropriate initial investigation for this patient?
Your Answer: Upper GI endoscopy and colonoscopy
Explanation:Possible GI Malignancy in a Man with Weight Loss and Microcytic Anaemia
This man is experiencing weight loss and has an unexplained microcytic anaemia. The most probable cause of his blood loss is from the gastrointestinal (GI) tract, as there is no other apparent explanation. This could be due to an occult GI malignancy, which is why the recommended initial investigations are upper and lower GI endoscopy. These tests will help to identify any potential sources of bleeding in the GI tract and determine if there is an underlying malignancy. It is important to diagnose and treat any potential malignancy as early as possible to improve the patient’s prognosis. Therefore, prompt investigation and management are crucial in this case.
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This question is part of the following fields:
- Haematology And Oncology
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Question 10
Incorrect
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A 75-year-old man has a tumour located in the central area of the posterior third of his tongue. Which lymph node group is most likely to be affected by metastasis?
Your Answer:
Correct Answer: Bilateral deep cervical nodes
Explanation:Metastasis to the bilateral deep cervical lymph nodes is a common occurrence in tumours located in the posterior third of the tongue. This is particularly true for tumours located near the midline, as lymph vessels may cross the median plane at this location. Additionally, centrally located tumours are also more likely to exhibit early metastasis.
Lymphatic Drainage of the Tongue
The lymphatic drainage of the tongue varies depending on the location of the tumour. The anterior two-thirds of the tongue have minimal communication of lymphatics across the midline, resulting in metastasis to the ipsilateral nodes being more common. On the other hand, the posterior third of the tongue has communicating networks, leading to early bilateral nodal metastases being more common in this area.
The tip of the tongue drains to the submental nodes and then to the deep cervical nodes, while the mid portion of the tongue drains to the submandibular nodes and then to the deep cervical nodes. If mid tongue tumours are laterally located, they will usually drain to the ipsilateral deep cervical nodes. However, those from more central regions may have bilateral deep cervical nodal involvement. Understanding the lymphatic drainage of the tongue is crucial in determining the spread of tumours and planning appropriate treatment.
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This question is part of the following fields:
- Haematology And Oncology
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Question 11
Incorrect
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A 27-year-old man comes to his doctor for a routine check-up before participating in a local 20-mile cycling race. He has been training for over a year and is determined to win. He has been experiencing occasional headaches on both sides of his head for the past three weeks, but they come and go and are not accompanied by aura, photophobia, or phonophobia. He has some redness and tenderness on his abdomen, but no masses are felt. His bowel and bladder function are normal. He had flu-like symptoms last week but is feeling much better now. His blood test results are as follows, and his hematocrit level is higher than normal:
Hemoglobin: 198 g/L
Platelets: 250 * 10^9/L
White blood cells: 6 * 10^9/L
Which of the following best explains his symptoms and blood test results?Your Answer:
Correct Answer: Secondary polycythemia due to erythropoietin use
Explanation:Athletes who use EPO are at risk of developing polycythemia. Cyclists are known to frequently use EPO, which can cause localized erythema on the abdomen from repeated injections. The patient’s headaches are not migrainous as they lack associated symptoms such as aura, photophobia, or phonophobia. Renal cell carcinoma is the primary type of kidney cancer in adults and typically presents with flank pain, haematuria, and a flank mass. Other symptoms may include weight loss, night sweats, fever, and malaise.
Polycythaemia is a condition that can be classified as relative, primary (polycythaemia rubra vera), or secondary. Relative polycythaemia can be caused by dehydration or stress, such as in Gaisbock syndrome. Primary polycythaemia rubra vera is a rare blood disorder that causes the bone marrow to produce too many red blood cells. Secondary polycythaemia can be caused by conditions such as COPD, altitude, obstructive sleep apnoea, or excessive erythropoietin production due to certain tumors or growths. To distinguish between true polycythaemia and relative polycythaemia, red cell mass studies may be used. In true polycythaemia, the total red cell mass in males is greater than 35 ml/kg and in women is greater than 32 ml/kg. Uterine fibroids may also cause polycythaemia indirectly by causing menorrhagia, but this is rarely a clinical problem.
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This question is part of the following fields:
- Haematology And Oncology
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Question 12
Incorrect
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A 67-year-old woman visits the oncology clinic after being diagnosed with non-metastatic breast cancer. She is started on neoadjuvant chemotherapy using docetaxel.
What is the mechanism of action for this form of chemotherapy?Your Answer:
Correct Answer: Prevents microtubule depolymerisation and disassembly
Explanation:Docetaxel, a taxane chemotherapy agent, works by reducing the amount of free tubulin through the prevention of microtubule depolymerisation and disassembly during the metaphase stage of cell division, ultimately hindering mitosis.
Cytotoxic agents are drugs that are used to kill cancer cells. There are several types of cytotoxic agents, each with their own mechanism of action and potential adverse effects. Alkylating agents, such as cyclophosphamide, work by causing cross-linking in DNA. However, they can also cause haemorrhagic cystitis, myelosuppression, and transitional cell carcinoma. Cytotoxic antibiotics, like bleomycin and anthracyclines, degrade preformed DNA and stabilize DNA-topoisomerase II complex, respectively. However, they can also cause lung fibrosis and cardiomyopathy. Antimetabolites, such as methotrexate and fluorouracil, inhibit dihydrofolate reductase and thymidylate synthesis, respectively. However, they can also cause myelosuppression, mucositis, and liver or lung fibrosis. Drugs that act on microtubules, like vincristine and docetaxel, inhibit the formation of microtubules and prevent microtubule depolymerisation & disassembly, respectively. However, they can also cause peripheral neuropathy, myelosuppression, and paralytic ileus. Topoisomerase inhibitors, like irinotecan, inhibit topoisomerase I, which prevents relaxation of supercoiled DNA. However, they can also cause myelosuppression. Other cytotoxic drugs, such as cisplatin and hydroxyurea, cause cross-linking in DNA and inhibit ribonucleotide reductase, respectively. However, they can also cause ototoxicity, peripheral neuropathy, hypomagnesaemia, and myelosuppression.
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This question is part of the following fields:
- Haematology And Oncology
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Question 13
Incorrect
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A 10-year-old boy has been admitted to the hospital due to a swollen and painful right knee. His mother reports that he has always had large bruises after minor injuries. About six months ago, he had bleeding that lasted for ten days after biting his tongue, which required a blood transfusion. The patient has not taken any medication except for Paracetamol for knee pain two hours ago. In the family history, the patient's uncle required a blood transfusion after tonsillectomy and had bleeding after a dental extraction, while their grandfather had severe bleeding after a routine knee operation. Which of the following test results would be expected? The tests are BT (bleeding time), APTT (activated partial thromboplastin time), PT (prothrombin time), and TT (thrombin time).
Your Answer:
Correct Answer: BT - Normal; APTT - Abnormal; PT - Normal; TT - Normal
Explanation:Haemophilia and its Laboratory Findings
Haemophilia is a genetic disorder that affects males in the family. It can either be haemophilia A or B, which are both sex-linked recessive disorders. Haemophilia A is caused by a deficiency of factor VIII, while haemophilia B is caused by a deficiency of factor IX. Females are carriers of the gene, but only males express the disease. The hallmark symptoms of haemophilia include haemorrhage into the joints, bleeding with tooth extraction, and skin bruising.
Laboratory findings in haemophilia include normal prothrombin time and bleeding time, as well as normal fibrinogen levels. However, there is a prolongation of the partial thromboplastin time. It is important to differentiate haemophilia from other bleeding disorders, such as Von Willebrand’s disease. While the bleeding phenotype in Von Willebrand’s disease is generally less severe, the family history is more in keeping with haemophilia. Coagulation tests in Von Willebrand’s disease are often normal.
In summary, haemophilia is a genetic disorder that affects males in the family and can either be haemophilia A or B. The hallmark symptoms include haemorrhage into the joints, bleeding with tooth extraction, and skin bruising. Laboratory findings in haemophilia include normal prothrombin time and bleeding time, normal fibrinogen levels, and a prolongation of the partial thromboplastin time. It is important to differentiate haemophilia from other bleeding disorders, such as Von Willebrand’s disease, which has different coagulation test results.
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This question is part of the following fields:
- Haematology And Oncology
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Question 14
Incorrect
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A 65-year-old man presents with shortness of breath and a haemoglobin level of 72 g/dL. The haematology lab performed a blood film and found numerous schistocytes and occasional reticulocytes, with no other erythrocyte abnormalities. Neutrophils and platelets were normal. The patient has a mid-line sternotomy scar, bruising to the arms, a metallic click to the first heart sound, and a resting tremor in the left hand. What is the most likely cause of his anaemia?
Your Answer:
Correct Answer: Intravascular haemolysis
Explanation:Schistocytes on a blood film are indicative of intravascular haemolysis, which is the most likely cause in this clinical scenario. The presence of a mid-line sternotomy scar, metallic click to the first heart sound, and warfarin prescription suggests a metal heart valve, which can cause sheering of red blood cells and subsequent intravascular haemolysis. Vasculitis, thrombotic thrombocytopenic purpura (TTP), and B12 deficiency are less likely causes in this case.
Pathological Red Cell Forms in Blood Films
Blood films are used to examine the morphology of red blood cells and identify any abnormalities. Pathological red cell forms are associated with various conditions and can provide important diagnostic information. Some of the common pathological red cell forms include target cells, tear-drop poikilocytes, spherocytes, basophilic stippling, Howell-Jolly bodies, Heinz bodies, schistocytes, pencil poikilocytes, burr cells (echinocytes), and acanthocytes.
Target cells are seen in conditions such as sickle-cell/thalassaemia, iron-deficiency anaemia, hyposplenism, and liver disease. Tear-drop poikilocytes are associated with myelofibrosis, while spherocytes are seen in hereditary spherocytosis and autoimmune hemolytic anaemia. Basophilic stippling is a characteristic feature of lead poisoning, thalassaemia, sideroblastic anaemia, and myelodysplasia. Howell-Jolly bodies are seen in hyposplenism, while Heinz bodies are associated with G6PD deficiency and alpha-thalassaemia. Schistocytes or ‘helmet cells’ are seen in conditions such as intravascular haemolysis, mechanical heart valve, and disseminated intravascular coagulation. Pencil poikilocytes are seen in iron deficiency anaemia, while burr cells (echinocytes) are associated with uraemia and pyruvate kinase deficiency. Acanthocytes are seen in abetalipoproteinemia.
In addition to these red cell forms, hypersegmented neutrophils are seen in megaloblastic anaemia. Identifying these pathological red cell forms in blood films can aid in the diagnosis and management of various conditions.
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This question is part of the following fields:
- Haematology And Oncology
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Question 15
Incorrect
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A 10-year-old boy with a history of sickle cell anaemia arrives at the Emergency department complaining of a painful and swollen left leg that has been bothering him for the past two days. He has also been experiencing fevers and overall malaise. Upon examination, he is found to have a high fever and an extremely tender lower leg. What is the most probable organism responsible for his current condition?
Your Answer:
Correct Answer:
Explanation:Salmonella and Staphylococcus aureus as Causes of Osteomyelitis
Salmonella species are responsible for more than half of osteomyelitis cases in patients with sickle cell disease. The higher incidence of salmonella infections is due to various factors. The gut wall’s micro-infarcts allow the bacteria to enter the bloodstream, causing infection. Additionally, impaired splenic function leads to a weakened immune response against the pathogen.
On the other hand, Staphylococcus aureus is the most common organism that causes osteomyelitis in the general population. Although other organisms can also cause osteomyelitis, they are less frequently implicated.
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This question is part of the following fields:
- Haematology And Oncology
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Question 16
Incorrect
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Which of the following is the least probable cause of an extended prothrombin time?
Your Answer:
Correct Answer: Acquired factor 12 deficiency
Explanation:Cholestatic jaundice and prolonged antibiotic therapy can lead to a deficiency in vitamin K.
Abnormal coagulation can be caused by various factors such as heparin, warfarin, disseminated intravascular coagulation (DIC), and liver disease. Heparin prevents the activation of factors 2, 9, 10, and 11, while warfarin affects the synthesis of factors 2, 7, 9, and 10. DIC affects factors 1, 2, 5, 8, and 11, and liver disease affects factors 1, 2, 5, 7, 9, 10, and 11.
When interpreting blood clotting test results, different disorders can be identified based on the levels of activated partial thromboplastin time (APTT), prothrombin time (PT), and bleeding time. Haemophilia is characterized by increased APTT levels, normal PT levels, and normal bleeding time. On the other hand, von Willebrand’s disease is characterized by increased APTT levels, normal PT levels, and increased bleeding time. Lastly, vitamin K deficiency is characterized by increased APTT and PT levels, and normal bleeding time. Proper interpretation of these results is crucial in diagnosing and treating coagulation disorders.
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This question is part of the following fields:
- Haematology And Oncology
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Question 17
Incorrect
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What type of cell is found in higher quantities in the blood sample of an individual who has a viral infection?
Your Answer:
Correct Answer: Lymphocytes
Explanation:Blood Cell Types and Their Presence in Various Disorders
Lymphocytes are a type of blood cell that can be found in higher numbers during viral infections. Eosinophils, on the other hand, are present in response to allergies, drug reactions, or infections caused by flatworms and strongyloides. Monocytes are another type of blood cell that can be found in disorders such as EBV infection, CMML, and other atypical infections. Neutrophils are present in bacterial infections or in disorders such as CML or AML where their more immature blastoid form is seen. Lastly, platelets can be increased in infections, iron deficiency, or myeloproliferative disorders.
In summary, different types of blood cells can indicate various disorders or infections. By analyzing the presence of these cells in the blood, doctors can better diagnose and treat patients. It is important to note that the presence of these cells alone is not enough to make a diagnosis, and further testing may be necessary.
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This question is part of the following fields:
- Haematology And Oncology
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Question 18
Incorrect
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Which of the following processes enables phagocytosis to occur?
Your Answer:
Correct Answer: Opsonisation
Explanation:Phagocytosis is facilitated by opsonisation, which involves coating the micro-organism with antibody, C3b, and specific acute phase proteins. This leads to an increase in phagocytic cell surface receptors on macrophages and neutrophils, which is mediated by pro-inflammatory cytokines. As a result, these cells are able to engulf the micro-organism.
Phagocytosis: The Process of Cell Ingestion
Phagocytosis is the process by which cells ingest foreign materials or pathogens. The first step in this process is opsonisation, where the organism is coated by an antibody. The second step is adhesion to the cell surface, followed by pseudopodial extension to form a phagocytic vacuole. Finally, lysosomes fuse with the vacuole and degrade its contents.
Phagocytosis is an essential process for the immune system to fight off infections and diseases. It is a complex process that involves multiple steps, including opsonisation, adhesion, and pseudopodial extension. The end result is the degradation of the foreign material or pathogen by lysosomes. Understanding the process of phagocytosis is crucial for developing treatments for diseases that involve the immune system.
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This question is part of the following fields:
- Haematology And Oncology
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Question 19
Incorrect
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A 3-year-old child is experiencing recurrent infections that are difficult to clear, leading to suspicion of an immunodeficiency. The clinical immunologist orders an immunological profile, which reveals low CD3+ cells, normal CD20+ cells, and normal CD68+ cells. The child's medical history includes surgical repair of a cleft palate and Tetralogy of Fallot within the first year of life. Which pharyngeal pouches are involved in the development of the structure implicated in this child's immunodeficiency?
Your Answer:
Correct Answer: Third and fourth
Explanation:The thymus is derived from the third and fourth pharyngeal pouches during development.
In a child with normal levels of B-cells and monocytes but no T-cells, the underlying issue is likely located in the thymus as this is where T-cells are produced. This suggests that the thymus is the structure responsible for the child’s condition.
The child’s medical history, including a conotruncal heart defect and cleft palate, suggests a possible diagnosis of DiGeorge syndrome.
During development, the first pouch gives rise to the Eustachian tube, middle ear, mastoid antrum, and inner tympanic membrane. The second pouch forms the middle ear and palatine tonsils. The third pouch develops into the thymus and inferior parathyroid glands, while the fourth pouch gives rise to the superior parathyroid glands, thymus, thyroid C-cells, muscles, and cartilage of the larynx. The fifth pouch is a rudimentary structure that becomes part of the fourth pouch, and the sixth pouch forms the muscles and cartilage of the larynx.
The Thymus Gland: Development, Structure, and Function
The thymus gland is an encapsulated organ that develops from the third and fourth pharyngeal pouches. It descends to the anterior superior mediastinum and is subdivided into lobules, each consisting of a cortex and a medulla. The cortex is made up of tightly packed lymphocytes, while the medulla is mostly composed of epithelial cells. Hassall’s corpuscles, which are concentrically arranged medullary epithelial cells that may surround a keratinized center, are also present.
The inferior parathyroid glands, which also develop from the third pharyngeal pouch, may be located with the thymus gland. The thymus gland’s arterial supply comes from the internal mammary artery or pericardiophrenic arteries, while its venous drainage is to the left brachiocephalic vein. The thymus gland plays a crucial role in the development and maturation of T-cells, which are essential for the immune system’s proper functioning.
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This question is part of the following fields:
- Haematology And Oncology
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Question 20
Incorrect
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A 25-year-old man is playing rugby and sustains a compound fracture of the distal third of his clavicle with arterial bleeding. During surgical exploration, which vessel is likely to be encountered first?
Your Answer:
Correct Answer: Thoracoacromial artery
Explanation:The thoracoacromial artery originates from the axillary artery’s second part. It is a broad, brief trunk that penetrates the clavipectoral fascia and terminates by dividing into four branches, located deep to pectoralis major.
The Thoracoacromial Artery and its Branches
The thoracoacromial artery is a short trunk that originates from the axillary artery and is usually covered by the upper edge of the Pectoralis minor. It projects forward to the upper border of the Pectoralis minor and pierces the coracoclavicular fascia, dividing into four branches: pectoral, acromial, clavicular, and deltoid.
The pectoral branch descends between the two Pectoral muscles and supplies them and the breast, anastomosing with the intercostal branches of the internal thoracic artery and the lateral thoracic artery. The acromial branch runs laterally over the coracoid process and under the Deltoid, giving branches to it before piercing the muscle and ending on the acromion in an arterial network formed by branches from the suprascapular, thoracoacromial, and posterior humeral circumflex arteries. The clavicular branch runs upwards and medially to the sternoclavicular joint, supplying this articulation and the Subclavius. The deltoid branch arises with the acromial branch, crosses over the Pectoralis minor, and passes in the same groove as the cephalic vein, giving branches to both the Pectoralis major and Deltoid muscles.
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This question is part of the following fields:
- Haematology And Oncology
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Question 21
Incorrect
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From which of the following cell types do giant cells typically arise?
Your Answer:
Correct Answer: Macrophages
Explanation:Macrophages are still the most frequent cell type that can generate giant cells, despite the possibility of other cell types doing so.
Giant cells are masses that result from the fusion of various types of cells. Typically, these masses are composed of macrophages. It is important to note that giant cells are not the same as granulomas, although the agents that cause them may be similar. In fact, giant cells are often a reaction to foreign materials, such as suture material, and can be seen in histological sections stained with haematoxylin and eosin. Overall, giant cells are a unique phenomenon in cellular biology that can provide insight into the body’s response to foreign substances.
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This question is part of the following fields:
- Haematology And Oncology
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Question 22
Incorrect
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A 14-year-old girl with beta thalassaemia major is receiving counselling from her haematologist regarding the potential complications of her condition. The doctor explains that frequent blood transfusions may result in iron overload, which can result in liver damage and heart failure. What is an example of an iron chelation medication?
Your Answer:
Correct Answer: Deferiprone
Explanation:To prevent complications from iron overload caused by frequent transfusions in beta-thalassaemia major, iron chelation therapy is crucial. Iron chelation agents such as Deferiprone, Deferoxamine, and Deferasirox are commonly used for this purpose. Trientine is a copper chelator used in Wilson’s disease, while Dimercaptosuccinic acid is used as a lead chelator. Penicillamine is primarily used to treat copper toxicity.
Understanding Beta-Thalassaemia Major
Beta-thalassaemia major is a genetic disorder that results from the absence of beta globulin chains on chromosome 11. This condition typically presents in the first year of life with symptoms such as failure to thrive and hepatosplenomegaly. Microcytic anaemia is also a common feature, with raised levels of HbA2 and HbF, but absent HbA.
Management of beta-thalassaemia major involves repeated transfusions, which can lead to iron overload and organ failure. Therefore, iron chelation therapy, such as desferrioxamine, is crucial to prevent complications. It is important to understand the features and management of this condition to provide appropriate care for affected individuals.
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This question is part of the following fields:
- Haematology And Oncology
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Question 23
Incorrect
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A 50-year-old woman goes for a cervical screening test and is found to have HPV subtypes 6 & 11. She has no other health issues. What is her primary risk factor?
Your Answer:
Correct Answer: Genital warts
Explanation:HPV Infection and Cervical Cancer
Human papillomavirus (HPV) infection is the primary risk factor for cervical cancer, with subtypes 16, 18, and 33 being the most carcinogenic. Other common subtypes, such as 6 and 11, are associated with genital warts but are not carcinogenic. When endocervical cells become infected with HPV, they may undergo changes that lead to the development of koilocytes. These cells have distinct characteristics, including an enlarged nucleus, irregular nuclear membrane contour, hyperchromasia (darker staining of the nucleus), and a perinuclear halo. These changes are important diagnostic markers for cervical cancer and can be detected through Pap smears or other screening methods. Early detection and treatment of HPV infection and cervical cancer can greatly improve outcomes and reduce the risk of complications.
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This question is part of the following fields:
- Haematology And Oncology
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Question 24
Incorrect
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A 55-year-old male presents with exertional fatigue. He has no significant past medical history and is not taking any medications. His blood test results show abnormal readings of Hb 125 g/L (normal range: 135-180 g/L) and calcium 2.9 mmol/L (normal range: 2.1-2.6 mmol/L). The rest of his blood test results, including mean corpuscular volume, platelet count, and white cell count, are normal. Additionally, his serum ferritin, vitamin B12, and folic acid levels are normal. Upon conducting a urine protein electrophoresis, the presence of immunoglobulin light chains is detected. What is the most likely diagnosis?
Your Answer:
Correct Answer: Multiple myeloma
Explanation:The diagnosis of multiple myeloma can be supported by the presence of Bence-Jones protein, which is a monoclonal globulin protein produced by neoplastic plasma cells. Anaemia and hypercalcemia, along with the presence of Bence-Jones protein in the urine, make multiple myeloma the most likely diagnosis.
Gout can be diagnosed by examining the contents of a joint fluid aspirate under polarised red light. The urate crystals will appear needle-shaped and negatively birefringent.
Megaloblastic anaemia occurs due to inhibition of DNA synthesis during red blood cell production. A normal mean corpuscular volume (MCV) and serum vitamin B12 level can rule out megaloblastic anaemia.
While patients with non-Hodgkin lymphoma may present with anaemia, it can be ruled out for the time being as the white cell count and platelet count are normal.
Understanding Multiple Myeloma: Features and Investigations
Multiple myeloma is a type of cancer that affects the plasma cells in the bone marrow. It is most commonly found in patients aged 60-70 years. The disease is characterized by a range of symptoms, which can be remembered using the mnemonic CRABBI. These include hypercalcemia, renal damage, anemia, bleeding, bone lesions, and increased susceptibility to infection. Other features of multiple myeloma include amyloidosis, carpal tunnel syndrome, neuropathy, and hyperviscosity.
To diagnose multiple myeloma, a range of investigations are required. Blood tests can reveal anemia, renal failure, and hypercalcemia. Protein electrophoresis can detect raised levels of monoclonal IgA/IgG proteins in the serum, while bone marrow aspiration can confirm the diagnosis if the number of plasma cells is significantly raised. Imaging studies, such as whole-body MRI or X-rays, can be used to detect osteolytic lesions.
The diagnostic criteria for multiple myeloma require one major and one minor criteria or three minor criteria in an individual who has signs or symptoms of the disease. Major criteria include the presence of plasmacytoma, 30% plasma cells in a bone marrow sample, or elevated levels of M protein in the blood or urine. Minor criteria include 10% to 30% plasma cells in a bone marrow sample, minor elevations in the level of M protein in the blood or urine, osteolytic lesions, or low levels of antibodies in the blood. Understanding the features and investigations of multiple myeloma is crucial for early detection and effective treatment.
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This question is part of the following fields:
- Haematology And Oncology
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Question 25
Incorrect
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What is the lymphatic drainage of the ovaries?
Your Answer:
Correct Answer: Para-aortic nodes
Explanation:The para-aortic nodes receive lymphatic drainage from the ovary through the gonadal vessels.
Lymphatic Drainage of Female Reproductive Organs
The lymphatic drainage of the female reproductive organs is a complex system that involves multiple nodal stations. The ovaries drain to the para-aortic lymphatics via the gonadal vessels. The uterine fundus has a lymphatic drainage that runs with the ovarian vessels and may thus drain to the para-aortic nodes. Some drainage may also pass along the round ligament to the inguinal nodes. The body of the uterus drains through lymphatics contained within the broad ligament to the iliac lymph nodes. The cervix drains into three potential nodal stations; laterally through the broad ligament to the external iliac nodes, along the lymphatics of the uterosacral fold to the presacral nodes and posterolaterally along lymphatics lying alongside the uterine vessels to the internal iliac nodes. Understanding the lymphatic drainage of the female reproductive organs is important for the diagnosis and treatment of gynecological cancers.
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This question is part of the following fields:
- Haematology And Oncology
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Question 26
Incorrect
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What is the primary location for haematopoiesis during the first and second trimesters of foetal development?
Your Answer:
Correct Answer: Liver
Explanation:The Development of Haematopoiesis in the Foetus
The development of haematopoiesis in the foetus is a complex process that involves several organs. Initially, the yolk sac is the primary site of haematopoiesis until around two months gestation when the liver takes over. The liver remains the most important site of haematopoiesis until about month seven when the bone marrow becomes the predominant site throughout life.
After the age of 20, haematopoiesis occurs mainly in the proximal bones, with production in the distal lone bones decreasing. However, in certain disease states such as β-thalassaemia, haematopoiesis can occur outside of the bone marrow, known as extra-medullary haematopoiesis. the development of haematopoiesis in the foetus is important for identifying potential abnormalities and diseases that may arise during this process.
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This question is part of the following fields:
- Haematology And Oncology
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Question 27
Incorrect
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As a medical student working with a geriatric care team, we recently conducted a blood test on a patient with a history of microcytic anemia. Our goal was to determine if a blood transfusion was necessary. At what Hb level is a transfusion typically recommended for elderly patients?
Your Answer:
Correct Answer:
Explanation:According to the NICE guidelines, patients who require red blood cell transfusions but do not have major bleeding, acute coronary syndrome, or chronic anemia requiring regular transfusions should receive transfusions with a restrictive threshold. This threshold should be set at 7g/dl, with a target hemoglobin concentration of 7-9 g/dl after transfusion. For patients with acute coronary syndrome, a threshold of 8g/dl and a target hemoglobin concentration of 8-10g/dl after transfusion should be considered. For patients with chronic anemia requiring regular transfusions, individual thresholds and hemoglobin concentration targets should be established.
Understanding Microcytic Anaemia
Microcytic anaemia is a condition characterized by small red blood cells that result in a decrease in the amount of oxygen carried in the blood. There are several causes of microcytic anaemia, including iron-deficiency anaemia, thalassaemia, congenital sideroblastic anaemia, and lead poisoning. In some cases, microcytosis may be associated with a normal haemoglobin level, which could indicate the possibility of polycythaemia rubra vera. It is important to note that new onset microcytic anaemia in elderly patients should be urgently investigated to exclude underlying malignancy.
Beta-thalassaemia minor is a type of microcytic anaemia where the microcytosis is often disproportionate to the anaemia. It is important to identify the underlying cause of microcytic anaemia to determine the appropriate treatment. Iron-deficiency anaemia is the most common cause of microcytic anaemia and can be treated with iron supplements. Thalassaemia may require blood transfusions or bone marrow transplantation. Congenital sideroblastic anaemia may require treatment with vitamin B6 supplements. Lead poisoning can be treated by removing the source of lead exposure and chelation therapy. Overall, early diagnosis and treatment of microcytic anaemia can improve outcomes and prevent complications.
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This question is part of the following fields:
- Haematology And Oncology
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Question 28
Incorrect
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A 57-year-old man with stable angina undergoes an angiogram and is found to have a 60% stenosis of the left main artery. The surgeons recommend a coronary artery bypass procedure. Which structure is likely to be supplied by the vessel used in this procedure?
Your Answer:
Correct Answer: Thymus gland
Explanation:The thymus receives its arterial supply from either the internal mammary artery or the pericardiophrenic arteries.
During coronary artery bypass surgery, the internal thoracic artery, also referred to as the internal mammary artery, is utilized.
The Thymus Gland: Development, Structure, and Function
The thymus gland is an encapsulated organ that develops from the third and fourth pharyngeal pouches. It descends to the anterior superior mediastinum and is subdivided into lobules, each consisting of a cortex and a medulla. The cortex is made up of tightly packed lymphocytes, while the medulla is mostly composed of epithelial cells. Hassall’s corpuscles, which are concentrically arranged medullary epithelial cells that may surround a keratinized center, are also present.
The inferior parathyroid glands, which also develop from the third pharyngeal pouch, may be located with the thymus gland. The thymus gland’s arterial supply comes from the internal mammary artery or pericardiophrenic arteries, while its venous drainage is to the left brachiocephalic vein. The thymus gland plays a crucial role in the development and maturation of T-cells, which are essential for the immune system’s proper functioning.
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This question is part of the following fields:
- Haematology And Oncology
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Question 29
Incorrect
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An 81-year-old male visits his primary care physician with concerns about his medication. He has been diagnosed with Hodgkin's lymphoma and his oncologist has recommended a trial of chemotherapy with doxorubicin.
What is the mechanism of action of doxorubicin?Your Answer:
Correct Answer: Inhibits the formation of microtubules
Explanation:Vincristine inhibits the formation of microtubules, which are essential for separating chromosomes during cell division. This mechanism is also shared by paclitaxel, a member of the taxane family. Alkylating agents, such as cyclophosphamide, disrupt the double helix of DNA by adding an alkyl group to guanine bases. Methotrexate inhibits dihydrofolate reductase, an enzyme that supports folate in DNA synthesis. Pyrimidine antagonists, like cytarabine, prevent the use of pyrimidines in DNA synthesis.
Cytotoxic agents are drugs that are used to kill cancer cells. There are several types of cytotoxic agents, each with their own mechanism of action and potential adverse effects. Alkylating agents, such as cyclophosphamide, work by causing cross-linking in DNA. However, they can also cause haemorrhagic cystitis, myelosuppression, and transitional cell carcinoma. Cytotoxic antibiotics, like bleomycin and anthracyclines, degrade preformed DNA and stabilize DNA-topoisomerase II complex, respectively. However, they can also cause lung fibrosis and cardiomyopathy. Antimetabolites, such as methotrexate and fluorouracil, inhibit dihydrofolate reductase and thymidylate synthesis, respectively. However, they can also cause myelosuppression, mucositis, and liver or lung fibrosis. Drugs that act on microtubules, like vincristine and docetaxel, inhibit the formation of microtubules and prevent microtubule depolymerisation & disassembly, respectively. However, they can also cause peripheral neuropathy, myelosuppression, and paralytic ileus. Topoisomerase inhibitors, like irinotecan, inhibit topoisomerase I, which prevents relaxation of supercoiled DNA. However, they can also cause myelosuppression. Other cytotoxic drugs, such as cisplatin and hydroxyurea, cause cross-linking in DNA and inhibit ribonucleotide reductase, respectively. However, they can also cause ototoxicity, peripheral neuropathy, hypomagnesaemia, and myelosuppression.
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This question is part of the following fields:
- Haematology And Oncology
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Question 30
Incorrect
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A 65-year-old man visits his doctor complaining of fatigue and weight loss that has persisted for the past year. He has also been experiencing fevers and night sweats lately. During the physical examination, the doctor observes that the patient has bruises on his shins and forearms and hepatosplenomegaly. The doctor orders blood tests.
Hemoglobin: 100 g/L
White blood cells: 18.0 x 109/L
Neutrophils: 10.0 x 109/L
The patient is referred to the hospital, where a bone marrow biopsy is performed, and he is subsequently treated with imatinib.
Based on the most probable diagnosis, which of the following cell types is also likely to be elevated?Your Answer:
Correct Answer: Eosinophils
Explanation:The origin of eosinophils is from common myeloid progenitor cells. A patient with neutrophilia and low haemoglobin is likely to have chronic myeloid leukaemia (CML). CML is characterized by increased levels of all cells derived from the myeloid lineage, including basophils, monocytes, and eosinophils. The bone marrow biopsy is diagnostic for CML and typically shows the t(9;22) chromosomal translocation, also known as the Philadelphia chromosome. Imatinib, an inhibitor of the BCR-ABL fusion protein created with this translocation, is a common treatment for CML. Cells derived from common lymphoid progenitor cells are not affected in CML.
Haematopoiesis: The Generation of Immune Cells
Haematopoiesis is the process by which immune cells are produced from haematopoietic stem cells in the bone marrow. These stem cells give rise to two main types of progenitor cells: myeloid and lymphoid progenitor cells. All immune cells are derived from these progenitor cells.
The myeloid progenitor cells generate cells such as macrophages/monocytes, dendritic cells, neutrophils, eosinophils, basophils, and mast cells. On the other hand, lymphoid progenitor cells give rise to T cells, NK cells, B cells, and dendritic cells.
This process is essential for the proper functioning of the immune system. Without haematopoiesis, the body would not be able to produce the necessary immune cells to fight off infections and diseases. Understanding haematopoiesis is crucial in developing treatments for diseases that affect the immune system.
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This question is part of the following fields:
- Haematology And Oncology
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