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Question 1
Correct
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A 15-year-old girl presents to the clinic with her mother, reporting an increase in abdominal size and no menstrual periods for the past three months. Despite denying any sexual activity, you suspect she may be pregnant. What is the specific measurement of a urinary pregnancy test?
Your Answer: Beta-HCG
Explanation:The Role of Hormone Assays in Confirming Pregnancy
Beta-HCG is a hormone produced by the placenta during pregnancy. There are highly sensitive assays available to detect the presence of beta-HCG, which can confirm pregnancy. In fact, some manufacturers of pregnancy tests claim that their tests are more accurate than ultrasound dating in determining gestation during the early stages of pregnancy.
While alpha-fetoprotein may also be elevated in pregnancy, particularly in cases of neural tube defects, it is not the primary focus of pregnancy testing. Hormone assays for oestrogen, progesterone, or testosterone levels are not reliable methods for confirming pregnancy. Therefore, beta-HCG remains the most reliable hormone to test for when confirming pregnancy.
It is important to note that while hormone assays can confirm pregnancy, they cannot determine the viability of the pregnancy or the presence of any complications. Ultrasound imaging and other diagnostic tests may be necessary to assess the health of the pregnancy and the developing fetus.
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This question is part of the following fields:
- Haematology
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Question 2
Correct
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A 28-year-old woman presents to her general practitioner after suffering from several miscarriages and is afraid her husband will leave her. The patient gave the history of bruising even with minor injuries and several spontaneous miscarriages. On examination, the patient is noted to have a rash in a butterfly distribution on the nose and cheeks. Tests reveal 1+ proteinuria only.
What is the most likely cause of her main concern?Your Answer: Antiphospholipid syndrome (APLS)
Explanation:Understanding Antiphospholipid Syndrome (APLS) and its Link to Recurrent Spontaneous Abortions
When a young woman experiences multiple spontaneous abortions, it may indicate an underlying disorder. One possible cause is antiphospholipid syndrome (APLS), a hypercoagulable state with autoantibodies against phospholipid components. This disorder can lead to recurrent spontaneous abortions during the first 20 weeks of pregnancy, and approximately 9% of APLS patients also have renal abnormalities.
Other potential causes of recurrent spontaneous abortions include poorly controlled diabetes, nephritic syndrome, dermatomyositis, and anatomic defects like a bicornuate uterus. However, the examination and test results in this case suggest a systemic etiology, making APLS a strong possibility.
Diagnosing systemic lupus erythematosus (SLE), which can also cause nephritic or nephrotic syndrome, requires meeting at least 4 out of 11 criteria established by the American Rheumatism Association (ARA).
Understanding these potential causes and their links to recurrent spontaneous abortions can help healthcare providers identify and treat underlying disorders in women of reproductive age.
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This question is part of the following fields:
- Haematology
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Question 3
Correct
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A 19-year-old Afro-Caribbean male with sickle cell disease complains of right upper abdominal pain and exhibits tenderness upon palpation. What diagnostic test should be performed to confirm the diagnosis?
Your Answer: Abdominal ultrasound scan
Explanation:Pigment Gallstones and High Haem Turnover
In cases of chronic haemolysis, such as sickle cell disease or thalassaemia, the presence of unconjugated bilirubin in bile can lead to the formation of pigment gallstones. These stones are black in color and are caused by the precipitation of calcium bilirubinate from solution. The high concentration of unconjugated bilirubin in bile is a result of the increased turnover of haemoglobin. This can cause pain and discomfort for the patient. It is important to manage the underlying condition causing the high haem turnover to prevent the formation of pigment gallstones.
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This question is part of the following fields:
- Haematology
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Question 4
Correct
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A 67-year-old man presents to his General Practitioner with a 5-month history of night sweats and weight loss. He has no history of cough, shortness of breath or foreign travel. He denies any back or joint pain. He is normally fit and well, except for high blood pressure for which he takes enalapril. He continues to drink ten units of alcohol per week.
On examination, he is noted to have cervical and groin lymphadenopathy, with an enlarged spleen. The remainder of the examination and observations are normal.
Which of the following is most likely to be used in the management of this patient?Your Answer: Rituximab
Explanation:The patient in question is displaying symptoms of non-Hodgkin’s lymphoma, including night sweats, weight loss, lymphadenopathy, and splenomegaly. While other symptoms may include pruritus, fever, and shortness of breath, the most common treatment for progressive non-Hodgkin’s lymphoma is a combination chemotherapy called R-CHOP, which includes rituximab. Rituximab is an anti-CD20 monoclonal antibody used for non-Hodgkin’s lymphoma and rheumatoid arthritis. The other options, including infliximab, lenalidomide, radiotherapy, and rifampicin, are used for different conditions such as Crohn’s disease, multiple myeloma, Hodgkin’s lymphoma, and tuberculosis, respectively.
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This question is part of the following fields:
- Haematology
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Question 5
Incorrect
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A 36-year-old Afro-Caribbean woman presents to the Emergency Department complaining of shortness of breath, fever and a productive cough. She has suffered multiple severe infections over the past five years; however, she has no other past medical history.
On examination, you note intercostal recessions and the use of accessory muscles of respiration. She has significant coarse crepitations in her right lower lobe.
You take some basic observations, which are as follows:
Temperature: 39.8 °C
Heart rate: 120 bpm
Respiratory rate: 26 breaths/min
Blood pressure: 150/94 mmHg (lying) 146/90 mmHg (standing)
Oxygen saturation: 86% on room air
Her initial investigation findings are as follows:
Investigation Result Normal value
White cell count (WCC) 14.4 × 109/l 4–11. × 109/l
Neutrophils 12800 × 106/l 3000–5800 × 106/l
Lymphocytes 1400 × 106/l 1500–3000 × 106/l
Haemoglobin (Hb) 110 g/dl 115–155 g/dl
Mean corpuscular volume (MCV) 94 fl 76–98 fl
Platelets 360 × 109/l 150–400 × 109/l
Her chest X-ray shows significant consolidation in the right lower lobe.
A blood film comes back and shows the following: sickled erythrocytes and Howell–Jolly bodies.
A sputum culture is grown and shows Streptococcus pneumoniae, and the patient’s pneumonia is managed successfully with antibiotics and IV fluid therapy.
What condition is predisposing this patient to severe infections?Your Answer: Aplastic crisis
Correct Answer: Splenic dysfunction
Explanation:The patient has sickle cell disease and a history of recurrent infections, indicating long-term damage to the spleen. The blood film shows signs of splenic disruption, such as Howell-Jolly bodies, and a low lymphocyte level, which may be due to reduced lymphocyte storage capacity in the shrunken spleen. This is different from a splenic sequestration crisis, which is an acute pediatric emergency. The current admission may be an acute chest pain crisis, but it is not the cause of the recurrent infections. The patient does not have acute lymphoblastic leukemia, as there is no evidence of blastic cells or pancytopenia. Advanced HIV is a possibility, but the blood film suggests sickle cell disease. While the patient is at risk of an aplastic crisis, it typically occurs in younger patients after a parvovirus B19 infection, which is not present in this case.
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This question is part of the following fields:
- Haematology
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Question 6
Correct
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A 50-year-old female patient visits the clinic with complaints of abdominal discomfort and bloating that have been progressively worsening over the past few months. Upon examination, her GP discovered a complex left ovarian cyst. Which blood test would be the most useful in determining if the cyst is cancerous?
Your Answer: CA125
Explanation:Tumor Markers and Their Association with Different Cancers
Tumor markers are substances that are produced by cancer cells or by normal cells in response to cancer. These markers can be used to detect the presence of cancer, monitor the progress of treatment, and detect the recurrence of cancer. One such tumor marker is CA125, which is associated with ovarian carcinoma. However, elevations in CA125 can also be seen in uterine cancer, pancreas, stomach, and colonic tumors. In some cases, benign ovarian tumors can also cause a rise in CA125 levels. If a patient has a CA125 level of over 200U/ml and an abnormal ultrasound scan, it is highly likely that they have ovarian carcinoma.
Other tumor markers include CA19-9, which is primarily associated with pancreatic tumors, beta-HCG, which is associated with germ cell tumors, alpha-fetoprotein, which is associated with hepatocellular carcinoma, and carcinoembryonic antigen, which is associated with colonic carcinoma. While AFP and beta-HCG can rarely be secreted by ovarian tumors, it is important to check CA125 levels first, as they are much more frequently elevated. By monitoring tumor markers, doctors can detect cancer early and provide appropriate treatment.
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This question is part of the following fields:
- Haematology
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Question 7
Correct
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A 30-year-old donor experiences a sudden fainting spell during blood donation. What should be the immediate course of action to manage this adverse event?
Your Answer: Temporarily cease the donation, consider fluid replacement and elevate the donor's legs
Explanation:Donor safety is important in blood donation. Fainting is a common adverse event and should be treated by stopping the donation and reviving the donor. Elevation of the legs and monitoring vitals is necessary. Donors should be counseled on pre-donation expectations and encouraged to drink fluids after recovery. Hemoglobin checks are no longer required.
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This question is part of the following fields:
- Haematology
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Question 8
Incorrect
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A 70-year-old man presents with fatigue, pallor and shortness of breath. He has been battling with an indolent colon carcinoma for the past 5 years. He also suffers from insulin-dependent diabetes, hypertension, coronary artery disease and rheumatoid arthritis. He has been feeling unwell for the past few weeks. He denies any history of melaena or haematochezia and has been amenorrhoeic for decades. A bedside stool guaiac test is negative for any blood in the stool. He is well nourished, reports taking daily supplements and is not a vegetarian. He reports that his haematocrit is 0.28 (0.35–0.55) and haemoglobin level 100 g/l (115–155 g/l).
What additional findings would you expect to observe in his full blood count?Your Answer: Macrocytosis
Correct Answer: Increased ferritin
Explanation:Understanding Anaemia of Chronic Disease: Increased Ferritin and Decreased TIBC
Anaemia of chronic disease is a type of anaemia that is commonly seen in patients with chronic inflammatory conditions. It is characterised by a low haemoglobin level and low haematocrit, but unlike iron deficiency anaemia, it is associated with increased ferritin levels and decreased total iron-binding capacity (TIBC). This is because ferritin is a serum reactive protein that is elevated in response to the underlying inflammatory process.
Diagnosis of anaemia of chronic disease requires the presence of a chronic inflammatory condition and anaemia, which can be either normocytic or microcytic. It is important to note that a haemoglobin level of <80 g/l is very rarely associated with this type of anaemia. Treatment involves addressing the underlying disorder causing the anaemia and monitoring the haemoglobin level. Blood transfusion is only used in severe cases. It is important to differentiate anaemia of chronic disease from other types of anaemia. For example, it is characterised by a low reticulocyte count, and not reticulocytosis. Serum transferrin receptor is not affected in anaemia of chronic disease and would therefore be normal. Additionally, TIBC is reduced in anaemia of chronic disease, whereas it is increased in iron deficiency anaemia. Finally, anaemia of chronic disease is associated with either microcytosis or normocytosis, whereas macrocytosis is associated with other types of anaemia such as folate deficiency, vitamin B12 deficiency, alcohol excess, and myelodysplastic disease. In summary, understanding the unique features of anaemia of chronic disease, such as increased ferritin and decreased TIBC, can aid in its diagnosis and management.
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This question is part of the following fields:
- Haematology
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Question 9
Incorrect
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A 67-year-old man was admitted with pain in the left groin. He had fallen from his chair, after which the pain started. He was unable to walk and his left leg was externally rotated. X-rays revealed a neck of femur fracture. He lives alone in a flat and is a vegetarian. His past medical history includes shortness of breath, body itching and occasional swelling of legs. His blood tests revealed:
Investigation Result Normal range
Haemoglobin 120 g/l 135–175 g/l
Calcium 3.5 mmol/l 2.20–2.60 mmol/l
Phosphate 1.52 mmol/l 0.70–1.40 mmol/l
Erythrocyte sedimentation rate (ESR) 184 mm/h 0–15 mm/h
What is the most likely diagnosis?Your Answer: Malignancy with metastasis
Correct Answer: Multiple myeloma
Explanation:Diagnosis of Multiple Myeloma in a Patient with Pathological Fracture
A man has sustained a pathological fracture after a minor trauma, which is likely due to lytic bone lesions. He also presents with anemia, raised calcium, and ESR, all of which are consistent with a diagnosis of multiple myeloma. This is further supported by his age group for presentation.
Other possible diagnoses, such as osteoporosis, vitamin D deficiency, acute leukemia, and malignancy with metastasis, are less likely based on the absence of specific symptoms and laboratory findings. For example, in osteoporosis, vitamin D and phosphate levels are normal, and ESR and hemoglobin levels are not affected. In vitamin D deficiency, calcium and phosphate levels are usually normal or low-normal, and ESR is not raised. Acute leukemia typically presents with systemic symptoms and normal serum calcium levels. Malignancy with metastasis is possible but less likely without preceding symptoms suggestive of an underlying solid tumor malignancy.
In summary, the patient’s clinical presentation and laboratory findings suggest a diagnosis of multiple myeloma.
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This question is part of the following fields:
- Haematology
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Question 10
Correct
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A 43-year-old woman was diagnosed with acute myeloid leukaemia (AML) with 71% of bone marrow blasts. She declined bone marrow transplant and was started on appropriate chemotherapy. After 2 months, a repeat bone marrow revealed 8% of blasts. Peripheral blood was blast-free and blood tests revealed:
Investigation Result Normal value
Haemoglobin 106 g/l 115–155 g/l
White cell count (WCC) 8.1 × 109/l 4–11 × 109/l
Neutrophils 5.2 × 109/l 2.5–7.58 × 109/l
Lymphocytes 1.8 × 109/l 1.5–3.5 × 109/l
Platelets 131 × 109/l 150–400 × 109/l
What is her clinical status?Your Answer: Partial remission
Explanation:Partial remission occurs when a patient meets all the criteria for complete remission except for having more than 5% bone marrow blasts. To be diagnosed with partial remission, the blast cells can be between 5% and 25% and must have decreased by at least 50% from their levels before treatment.
Complete remission is achieved when a patient meets specific criteria, including having a neutrophil count of over 1.0 × 109/l and a platelet count of over 100 × 109/l, not requiring red cell transfusions, having normal cellular components on bone marrow biopsy, having less than 5% blasts in the bone marrow without Auer rods present, and having no signs of leukemia anywhere else in the body.
Complete remission with incomplete recovery is when a patient meets all the criteria for complete remission except for continuing to have neutropenia or thrombocytopenia.
Resistant disease occurs when a patient fails to achieve complete or partial remission and still has leukemia cells in their peripheral blood or bone marrow seven days after completing initial therapy.
A morphologic leukemia-free state is when a patient has less than 5% bone marrow blasts without blasts with Auer rods present and no extramedullary disease, but they do not meet the criteria for neutrophils, platelets, and blood transfusions.
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This question is part of the following fields:
- Haematology
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Question 11
Incorrect
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A 55-year-old woman presents at the clinic for evaluation. She has not experienced menstrual periods for the past six months and has taken two pregnancy tests, both of which were negative. Upon clinical examination, no abnormalities were found. The patient desires a blood test to determine if she has entered menopause. What is the most sensitive hormone to test for this purpose?
Your Answer: Oestrogen
Correct Answer: FSH
Explanation:Hormone Levels and Menopausal Status
Follicle-stimulating hormone (FSH) levels that are greater than 30 IU/l, repeated over a period of four to eight weeks, are typically indicative of menopause. It is important to ensure that FSH is tested when the patient is not on contraception, although this is not relevant in the current scenario. While oestrogen and progesterone levels decrease after menopause, their assay is less reliable in determining menopausal status compared to FSH levels. Beta-HCG levels are elevated during pregnancy and trophoblastic disease, while prolactin levels increase in response to certain drug therapies and the presence of a pituitary tumour.
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This question is part of the following fields:
- Haematology
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Question 12
Correct
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As a part of a haematology rotation, a final-year medical student is asked to give a PowerPoint presentation to the team about a 20-year-old patient who presented with sickle-cell crisis.
With regard to sickle-cell disease, which of the following statements is correct?Your Answer: Aplastic crisis can be precipitated by parvovirus B19
Explanation:Understanding Aplastic Crisis and Sickle-Cell Disease
Aplastic crisis is a condition of transient bone marrow failure that can be precipitated by parvovirus B19, Epstein–Barr virus (EBV), or Streptococcus. In sickle-cell disease, aplastic crisis is usually caused by parvovirus B19 and is characterized by reticulocytopenia, symptomatic anemia, and the presence of parvovirus immunoglobulin M (IgM) antibodies. It is managed by monitoring and symptomatic relief with blood transfusion until normal erythrocyte function returns. Aplastic crisis is most common in individuals of Mediterranean descent.
Sickle-cell disease is most common in individuals of Black Afro-Caribbean descent and, to a lesser extent, in individuals of Mediterranean or Middle Eastern descent. It occurs as a result of the production of an abnormal beta (β) chain in haemoglobin, caused by a mutation that changes adenine to thymine in the sixth codon of the β chain gene. This results in the formation of HbS, which circulates in the blood and forms polymers in the deoxygenated state, causing sickling of red blood cells. The resulting blood film shows elongated, thin, sickled red blood cells, target cells, and Howell–Jolly bodies.
Splenomegaly is most usually seen in childhood, as most children with sickle-cell disease have a splenic infarction event in late childhood and develop hyposplenism. Spherocytes, on the other hand, are abnormal red blood cells with a spherical shape, seen on the blood film of spherocytosis, a form of haemolytic anaemia.
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This question is part of the following fields:
- Haematology
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Question 13
Incorrect
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A 35-year-old woman comes to the clinic for follow-up three months after undergoing evacuation of a hydatidiform mole. She reports feeling well and has not experienced any significant issues since the procedure.
What blood test is the most effective in monitoring for the recurrence of trophoblastic disease?Your Answer: Alpha-fetoprotein (AFP)
Correct Answer: Beta-HCG
Explanation:Hydatidiform Mole and Trophoblastic Disease
A hydatidiform mole is a type of abnormal pregnancy that only generates placental tissue. However, approximately 10% of cases of hydatidiform mole can transform into malignant trophoblastic disease. To assess if there is any retained tissue or recurrence/malignant transformation, the best way is to measure the levels of HCG, which is primarily produced by the placenta. On the other hand, alpha-fetoprotein, CEA, and CA-125 are tumour markers associated with hepatocellular carcinoma, colonic carcinoma, and ovarian carcinoma, respectively. It is important to note that progesterone levels are not useful in determining the prognosis of trophoblastic disease. the characteristics and markers of hydatidiform mole and trophoblastic disease is crucial in the diagnosis and management of these conditions.
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This question is part of the following fields:
- Haematology
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Question 14
Correct
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Which autoantibody is correctly matched with its associated disease in the following list?
Your Answer: Pernicious anaemia and anti-intrinsic factor antibodies
Explanation:Autoimmune Disorders and Associated Antibodies
Autoimmune disorders occur when the immune system mistakenly attacks healthy cells and tissues in the body. These disorders are often associated with the presence of specific antibodies that can help diagnose and monitor the disease. Here are some examples:
Pernicious Anaemia and Anti-Intrinsic Factor Antibodies
Pernicious anaemia is a type of anaemia caused by a deficiency in vitamin B12. It is associated with the presence of anti-intrinsic factor antibodies, which bind to intrinsic factor and prevent the absorption of vitamin B12 in the gut.Primary Biliary Cholangitis and Anti-Jo-1 Antibodies
Primary biliary cholangitis is an autoimmune disorder that affects the liver. It is associated with the presence of anti-mitochondrial antibodies, but not anti-Jo-1 antibodies, which are associated with other autoimmune disorders like polymyositis and dermatomyositis.Myasthenia Gravis and Voltage-Gated Calcium Channel Antibodies
Myasthenia gravis is a neuromuscular disorder that causes muscle weakness and fatigue. It is associated with the presence of anti-acetylcholine receptor antibodies, but not anti-striated muscle antibodies, which are found in other autoimmune disorders.Granulomatosis with Polyangiitis (GPA) and Anti-Myeloperoxidase (p-ANCA) Antibody
GPA is a type of vasculitis that affects small and medium-sized blood vessels. It is associated with the presence of cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCA), but not p-ANCA, which are found in other types of vasculitis.Hashimoto’s Thyroiditis and Thyroid-Stimulating Antibodies
Hashimoto’s thyroiditis is an autoimmune disorder that affects the thyroid gland. It is associated with the presence of anti-thyroglobulin and anti-thyroperoxidase antibodies, which attack the thyroid gland and cause inflammation. -
This question is part of the following fields:
- Haematology
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Question 15
Correct
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A plasma donor on a continuous flow separator machine experiences light headedness, muscle cramps, and circumoral paraesthesia. What should be the next course of action in managing this patient?
Your Answer: Investigate and treat citrate toxicity
Explanation:Citrate Toxicity and Hypocalcaemia in Apheresis Patients
This patient is experiencing symptoms of citrate toxicity, which has led to hypocalcaemia. While it is possible for haemorrhage to occur at the site of venepuncture or venous access, this is typically easy to identify through clinical examination. Sepsis is an uncommon occurrence if proper aseptic precautions have been taken, and the symptoms described here are not indicative of an infection. Immediate treatment is necessary, and this can be achieved by slowing or stopping the apheresis process. Treatment options include the administration of oral or intravenous calcium replacement.
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This question is part of the following fields:
- Haematology
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Question 16
Correct
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A 35-year-old man presents with progressive weakness in his right upper limb and both lower limbs over the past four months. He has also developed digital infarcts affecting the second and third fingers on the right hand and the fifth finger on the left. On examination, his blood pressure is 160/140 mm Hg, all peripheral pulses are palpable, and there is an asymmetrical neuropathy. Laboratory investigations reveal a haemoglobin level of 120 g/L (130-170), a white cell count of 12.5 ×109/L (4-10), a platelet count of 430 ×109/L (150-450), and an ESR of 50 mm/hr (0-15). Urine examination shows proteinuria and 10-15 red blood cells per high power field without casts. What is the most likely diagnosis?
Your Answer: Polyarteritis nodosa
Explanation:Polyarteritis nodosa (PAN) is a systemic disease that affects small or medium-sized arteries in various organs, leading to a wide range of symptoms such as nerve damage, skin issues, joint and muscle pain, kidney problems, and heart issues. Laboratory findings include anemia, increased white blood cells and platelets, and elevated inflammatory markers. ANCA testing can help differentiate PAN from other vasculitis diseases.
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This question is part of the following fields:
- Haematology
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Question 17
Correct
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Which structure, containing both white and red pulp, is responsible for trapping foreign substances present in the blood?
Your Answer: Spleen
Explanation:Comparison of Lymphoid Organs and Non-Lymphoid Organs
The human body contains various organs that serve different functions. Among these are the lymphoid organs, which play a crucial role in the immune system. In this article, we will compare the characteristics of three lymphoid organs (spleen, lymph node, and thymus) with two non-lymphoid organs (heart and thyroid gland).
Spleen
The spleen is the largest secondary lymphoid organ. It is located in the left upper quadrant of the abdomen and contains both white pulp (lymphatic tissue and macrophages) and red pulp (sinusoids and red blood cells). One of its functions is to filter foreign substances from the blood.Lymph Node
Lymph nodes are secondary lymphoid organs that contain structures where mature lymphocytes are stimulated by antigens to undergo further division and differentiation. They do not contain white and red pulp.Thymus
The thymus is a primary lymphoid organ where T lymphocytes mature, differentiate, and proliferate. It does not contain white and red pulp.Heart
The heart is the main organ of the circulatory system and does not contain white and red pulp.Thyroid Gland
The thyroid gland is located in the anterior neck and is part of the endocrine system. It does not contain white and red pulp.In summary, lymphoid organs play a crucial role in the immune system, while non-lymphoid organs serve other functions. Understanding the characteristics of these organs can help us appreciate the complexity and diversity of the human body.
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This question is part of the following fields:
- Haematology
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Question 18
Correct
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A 75-year-old woman comes in with a swollen and hot right calf. She had undergone a right knee arthroscopy and had been immobile for a while. She only takes paracetamol and codeine for osteoarthritis as needed. An above-knee deep vein thrombosis (DVT) is detected on ultrasound, which is her first DVT. What is the recommended treatment?
Your Answer: Three months of apixaban
Explanation:Management of Deep Vein Thrombosis and Pulmonary Embolism
Apixaban and rivaroxaban are the preferred medications for treating deep vein thrombosis (DVT), except for patients with renal impairment or antiphospholipid syndrome. Low molecular weight heparin (LMWH) and warfarin are alternative options for those who cannot take apixaban or rivaroxaban. Thrombolysis is used to manage pulmonary embolism (PE) in patients with haemodynamic instability.
The duration of anticoagulation treatment depends on the type of DVT. For provoked DVTs, which have an identifiable cause, treatment is recommended for at least three months. After this period, the risks and benefits of continuing anticoagulation treatment must be assessed to determine further treatment. For unprovoked DVTs, which have no identifiable cause, treatment is recommended for at least six months. After this period, a risk and benefit assessment is required to determine further treatment.
NICE has provided a helpful visual summary to assist in the management of DVT and PE.
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This question is part of the following fields:
- Haematology
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Question 19
Correct
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A 28-year-old woman on vacation complained of a swollen left calf. She mentioned having the 'factor V Leiden mutation'. Can you explain the pathophysiology of this mutation?
Your Answer: Activated protein C resistance
Explanation:Factor V Leiden: A Genetic Condition Affecting Blood Clotting
Factor V is a protein that acts as a cofactor to allow the generation of an enzyme called thrombin, which is responsible for cleaving fibrinogen to fibrin. This process leads to the formation of a dense meshwork that makes up the majority of a clot. Activated protein C (aPC) is a natural anticoagulant that limits clotting by degrading factor V. However, in individuals with Factor V Leiden, a genetic condition that is inherited in an autosomal dominant manner, the coagulation factor cannot be destroyed by aPC.
Factor V Leiden is caused by a single nucleotide substitution of adenine for guanine in the gene encoding factor V. This mutation changes the protein’s 506th amino acid from arginine to glutamine, which prevents efficient inactivation of factor V. As a result, factor V remains active, leading to overproduction of thrombin and excess fibrin generation, which in turn causes excess clotting.
In summary, Factor V Leiden is a genetic condition that affects blood clotting by preventing the efficient inactivation of factor V. This leads to excess clotting, which can increase the risk of developing blood clots and related complications.
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This question is part of the following fields:
- Haematology
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Question 20
Incorrect
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A 25-year-old patient presented with red rashes on their feet. Upon examination, they were found to be pale with purpuric spots on their lower legs. Their temperature was 38.3 °C and they also complained of nausea. On the second day of admission, their fever increased and they became disoriented. New bleeding spots started appearing on their face. Blood reports revealed low hemoglobin, high white cell count, low platelets, and high creatinine levels. A peripheral blood smear showed helmet cells and anisocytosis. The CSF study was normal. What test should be done next for this patient?
Your Answer: Antiplatelet antibody
Correct Answer: Urinary β-human chorionic gonadotrophin (hCG)
Explanation:The patient is presenting with thrombotic thrombocytopenic purpura (TTP), which is characterized by low platelet count due to clotting and platelet sequestration in small vessels. TTP is associated with haemolytic anaemia, thrombocytopenic purpura, fever, and neurological and renal abnormalities. The patient’s risk factors for TTP include being female, obese, pregnant, and of Afro-Caribbean origin. To determine the appropriate management, a urinary β-hCG test should be performed to establish pregnancy status. The first-line treatment for TTP is plasma exchange with fresh frozen plasma. Blood cultures should also be performed to check for underlying septicaemia. Antiplatelet antibody titres can be raised in idiopathic thrombocytopenic purpura (ITP), but ITP does not cause renal failure. A bone marrow study is appropriate to rule out leukaemia. Illicit drug use should also be considered as a cause of disseminated intravascular coagulation (DIC).
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This question is part of the following fields:
- Haematology
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Question 21
Incorrect
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What is the correct information regarding the storage requirements and lifespan of blood products?
Your Answer: Fresh frozen plasma is stored at −30°C for 12 months
Correct Answer: Fresh frozen plasma is stored at −25°C for up to 36 months
Explanation:Storage Guidelines for Blood Products
Blood products such as fresh frozen plasma, red cells, and platelets have specific storage guidelines to ensure their safety and efficacy. Fresh frozen plasma can be stored for up to 36 months at a temperature of −25°C. On the other hand, red cells are stored at a temperature of 4°C for a maximum of 35 days, while platelets are stored at a temperature of 22°C for up to 5 days on a platelet shaker/agitator.
These guidelines are important to follow to maintain the quality of blood products and prevent any adverse reactions in patients who receive them. It is crucial to store blood products at the appropriate temperature and for the recommended duration to ensure their effectiveness when used in transfusions. Healthcare professionals should be aware of these guidelines and ensure that they are followed to provide safe and effective blood transfusions to patients.
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This question is part of the following fields:
- Haematology
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Question 22
Correct
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A 50-year-old woman presents with headaches and nosebleeds and is found to have a raised platelet count. She is diagnosed with essential thrombocytosis by the haematologist.
Which of the following might be used to treat essential thrombocytosis?Your Answer: Hydroxyurea
Explanation:Common Medications and Their Uses
Thrombocytosis and Hydroxyurea
Thrombocytosis is a condition characterized by an elevated platelet count, which can lead to bleeding or thrombosis. Primary or essential thrombocytosis is a myeloproliferative disorder that results in overproduction of platelets by the bone marrow. Hydroxyurea is the first-line treatment for essential thrombocytosis, as it inhibits an enzyme involved in DNA synthesis and reduces the rate of platelet production.Interferon Gamma for Immunomodulation
Interferon gamma is an immunomodulatory medication used to reduce the frequency of infections in patients with chronic granulomatous disease and severe malignant osteopetrosis. It is administered by subcutaneous injection.Cromoglycate for Inflammation
Sodium cromoglycate is a synthetic non-steroidal anti-inflammatory drug used in the treatment of asthma, allergic rhinitis, and various food allergies.Interferon β for Multiple Sclerosis
Interferon β is a cytokine used in the treatment of relapsing-remitting multiple sclerosis. It is administered subcutaneously.Ranitidine for Acid Reduction
Ranitidine is a H2 (histamine) receptor blocker that inhibits the production of acid in the stomach. It can be used in the treatment of gastro-oesophageal reflux disease, peptic ulcer disease, and gastritis. -
This question is part of the following fields:
- Haematology
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Question 23
Incorrect
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A 50-year-old patient presents to the general practitioner with a complaint of darkening of urine, particularly noticeable in the morning. The patient has no family history of bleeding disorders and was recently hospitalized for deep venous thrombosis in the right leg. Upon examination, the patient's heart and lungs appear normal. Blood tests reveal anemia, elevated levels of lactate dehydrogenase (LDH), high bilirubin levels, and a high reticulocyte count. What is the most likely cause of this patient's condition?
Your Answer: Glucose-6-phosphate dehydrogenase deficiency
Correct Answer: Phosphatidylinositol glycan A defect in red blood cells
Explanation:Understanding Different Causes of Haemolytic Anaemia
Haemolytic anaemia is a condition where red blood cells are destroyed faster than they can be produced, leading to a shortage of oxygen-carrying cells in the body. There are various causes of haemolytic anaemia, including phosphatidylinositol glycan A defect, vitamin B12 deficiency, glucose-6-phosphate dehydrogenase deficiency, loss of spectrin in the red blood cell membrane, and immunoglobulin M (IgM) antibody against red blood cells.
Phosphatidylinositol glycan A defect, also known as nocturnal haemoglobinuria, is an acquired condition caused by a mutation in the gene encoding for phosphatidylinositol glycan A. This leads to an increased susceptibility of red blood cells to complement proteins in an acidotic environment, resulting in haemolysis. Patients typically present with haematuria in the morning, and treatment involves managing symptoms and using medication such as eculizumab.
Vitamin B12 deficiency causes megaloblastic anaemia and is not related to haemolysis. Glucose-6-phosphate dehydrogenase deficiency is an inherited X-linked recessive condition that results in red blood cell breakdown. Loss of spectrin in the red blood cell membrane is seen in hereditary spherocytosis, where red blood cells become spherical and are trapped in the spleen, leading to haemolysis. IgM antibody against red blood cells causes autoimmune haemolytic anaemia, where the antibody binds to the I antigen on the membrane of red blood cells, leading to haemolysis at low temperatures.
Understanding the different causes of haemolytic anaemia is crucial for proper diagnosis and treatment.
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This question is part of the following fields:
- Haematology
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Question 24
Incorrect
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A 60-year-old man comes to his doctor with complaints of night sweats and unintended weight loss. He has a medical history of axillary lymphadenopathy. The doctor suspects that he may have non-Hodgkin's lymphoma. What is the most probable test to confirm this diagnosis?
Your Answer: Protein electrophoresis and urine Bence-Jones protein
Correct Answer: Excisional biopsy of an enlarged lymph node
Explanation:Diagnostic Investigations for Non-Hodgkin’s Lymphoma
Non-Hodgkin’s lymphoma is a type of cancer that affects the lymphatic system. There are several diagnostic investigations that can be used to diagnose this condition.
Excisional Biopsy of an Enlarged Lymph Node: This is the most common diagnostic investigation for suspected non-Hodgkin’s lymphoma. It involves removing all of the abnormal tissue from an enlarged lymph node.
Computed Tomography (CT) of the Chest, Neck, Abdomen, and Pelvis: CT scanning can indicate features suggestive of lymphoma, such as lymphadenopathy and hepatosplenomegaly. However, it cannot provide a tissue diagnosis.
Core Needle Biopsy of an Enlarged Lymph Node: If a surgical excisional biopsy is not appropriate, a core needle biopsy can be performed. However, if this does not reveal a definite diagnosis, then an excisional biopsy should be undertaken.
Full Blood Count: A full blood count can be a helpful first-line investigation if a haematological malignancy is suspected, but it is not sufficient to be diagnostic for non-Hodgkin’s lymphoma.
Protein Electrophoresis and Urine Bence-Jones Protein: Protein electrophoresis can be helpful in screening for multiple myeloma, but it is not helpful for diagnosing non-Hodgkin’s lymphoma.
In conclusion, a combination of these diagnostic investigations can be used to diagnose non-Hodgkin’s lymphoma. However, excisional biopsy remains the gold standard for diagnosis.
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This question is part of the following fields:
- Haematology
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Question 25
Correct
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A 48-year-old man was admitted with unconsciousness.
On examination, his left plantar response was extensor, and deep tendon jerks were increased on the left side. A computerised tomography (CT) scan of the brain revealed a right-middle cerebral artery territory infarct. He was not known to have diabetes or hypertension. He was not receiving any drugs, either.
His blood count revealed:
Investigation Result Normal value
Haemoglobin 110 g/l 135–175 g/l
White cell count (WCC) 331 × 109/l 4–11 × 109/l
Neutrophil count 145 × 109/l 2.5–7.58 × 109/l
Metamyelocyte 3000/mm3
Platelet 490 × 109/l 150–400 × 109/l
Peripheral smear Many band forms, myelocytes, basophils
What is the next appropriate therapy?Your Answer: Leukapheresis
Explanation:Leukapheresis and Other Treatment Options for Chronic Myeloid Leukaemia with High White Blood Cell Count and Ischaemic Stroke
Chronic myeloid leukaemia can cause an extremely high white blood cell count, leading to hyperviscosity of the blood and an increased risk of ischaemic events such as stroke. While anticoagulation medications are important, they do not address the underlying issue of the high cell count. Leukapheresis is a procedure that can reduce the white cell volume by 30-60%, making it a crucial emergency treatment option. Other treatments, such as hydroxyurea and imatinib, can also be used to control disease burden. Imatinib is a tyrosine kinase inhibitor that is effective in treating chronic myeloid leukaemia with the Philadelphia chromosome translocation. Aspirin and heparin have limited roles in this scenario. While aspirin is recommended for long-term therapy after an ischaemic stroke, it does not address the hypercoagulable state caused by the high white blood cell count. Heparin is not used in the treatment of ischaemic strokes. Overall, leukapheresis should be the first step in emergency management for chronic myeloid leukaemia with a high white blood cell count and ischaemic stroke.
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This question is part of the following fields:
- Haematology
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Question 26
Incorrect
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A 33-year-old woman who gave birth three weeks ago is feeling very fatigued after walking to the park and back. The birth was via a caesarean section and she needed one unit of blood transfusion. She visits her family doctor and denies experiencing any chest pain, heart palpitations, shortness of breath, or further bleeding.
During the examination, there are no indications of ongoing bleeding, and the caesarean section incision is healing properly. The blood test results are as follows:
- Hb 95 g/L Female: (115 - 160)
- Platelets 240 * 109/L (150 - 400)
- WBC 7.0 * 109/L (4.0 - 11.0)
- Ferritin 6 µg/L (15 - 300)
What is the minimum haemoglobin level for this patient to be prescribed iron supplements?Your Answer: <105 g/L
Correct Answer:
Explanation:The appropriate cut-off for determining if iron supplementation is necessary in the postpartum period is <100 g/L. It is important to continue oral iron for three months after normalizing ferritin levels to ensure adequate stores for efficient oxygen delivery to the tissues. Cut-offs of <105 g/L, <110 g/L, and <120 g/L are incorrect for iron supplementation in the second or third trimester of pregnancy, first trimester of pregnancy, and postpartum period, respectively. However, the decision to administer iron for anaemia should be based on the doctor's discretion and the patient's symptoms. During pregnancy, women are checked for anaemia twice – once at the initial booking visit (usually around 8-10 weeks) and again at 28 weeks. The National Institute for Health and Care Excellence (NICE) has set specific cut-off levels to determine if a pregnant woman requires oral iron therapy. These levels are less than 110 g/L in the first trimester, less than 105 g/L in the second and third trimesters, and less than 100 g/L postpartum. If a woman’s iron levels fall below these cut-offs, she will be prescribed oral ferrous sulfate or ferrous fumarate. It is important to continue this treatment for at least three months after the iron deficiency has been corrected to allow the body to replenish its iron stores. By following these guidelines, healthcare professionals can help ensure that pregnant women receive the appropriate care to prevent and manage anaemia during pregnancy.
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This question is part of the following fields:
- Haematology
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Question 27
Correct
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A patient on the ward receiving a blood transfusion after knee replacement surgery is noted to have rigors.
On examination, their temperature is 39°C (baseline 36.5°C). They also complain of abdominal pain and their blood pressure is 90/55 mmHg (baseline 126/84 mmHg).
What is the next step in managing this patient?Your Answer: Stop the transfusion immediately and commence IV fluids
Explanation:Management of Acute Haemolytic Transfusion Reaction
When a patient experiences a temperature rise of more than 2°C, abdominal pain, and hypotension after a blood transfusion, an acute haemolytic transfusion reaction should be suspected. In such cases, the transfusion must be stopped immediately, and the set should be taken down. Saline infusion should be initiated to maintain the patient’s blood pressure.
The blood bank should be notified of the suspected reaction, and a sample may need to be collected for further investigation. However, the priority is to manage the patient’s symptoms and prevent further complications. If the reaction is severe, the transfusion should not be continued.
In summary, prompt recognition and management of acute haemolytic transfusion reactions are crucial to prevent serious complications. Healthcare providers should be vigilant in monitoring patients who receive blood transfusions and act quickly if any adverse reactions occur.
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This question is part of the following fields:
- Haematology
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Question 28
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A 35-year-old woman is brought to the Emergency Department after consuming 30 tablets of warfarin which belonged to her mother. She has diarrhoea but has no mucosal bleeding. She admits to previously attending a psychiatric unit for self-harming behaviour. She has no remarkable medical history. Her mother has a history of recurrent venous thrombosis for which she is taking warfarin.
What will the patient’s coagulation screen likely be?Your Answer: Elevated prothrombin time, international normalised ratio, activated partial thromboplastin time and normal platelet counts
Explanation:Warfarin poisoning is characterized by elevated prothrombin time (PT), international normalized ratio (INR), and activated partial thromboplastin time (APTT), along with normal platelet counts. This is due to the drug’s ability to block the function of vitamin K epoxide reductase, leading to a depletion of the reduced form of vitamin K that serves as a cofactor for gamma carboxylation of vitamin-K-dependent coagulation factors. As a result, the vitamin-K-dependent factors cannot function properly, leading to elevated PT and INR, normal or elevated APTT, and normal platelet counts. Thrombocytopenia with normal PT, INR, and APTT can be caused by drugs like methotrexate and carboplatin isotretinoin, which induce direct myelosuppression. Decreased factor VIII levels are seen in haemophilia A, disseminated intravascular coagulation (DIC), and von Willebrand disease, but not in warfarin overdose. Isolated APTT elevation is seen in heparin overdose, while elevated fibrinogen levels can be seen in inflammation, acute coronary syndrome, and stroke, but not in warfarin overdose.
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This question is part of the following fields:
- Haematology
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Question 29
Incorrect
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A 17-year-old male patient comes in with a history of fevers, night sweats, and a weight loss of 20 pounds over the course of several months. Upon conducting a CT scan, mediastinal lymphadenopathy is discovered, and a biopsy is performed. The genetic analysis of the lesion reveals a translocation between chromosomes 14 and 18. What protein is expected to be overexpressed due to this translocation?
Your Answer: c-myc
Correct Answer: bcl-2
Explanation:Follicular lymphoma is a prevalent type of non-Hodgkin’s lymphoma that results from a chromosomal translocation between chromosome 14 and chromosome 18. This translocation causes the bcl-2 protein, which is anti-apoptotic, to be moved to the IgH promoter region, leading to overproduction of bcl-2. Consequently, abnormal B cells undergo clonal proliferation and are protected from apoptosis. Follicular lymphoma affects both genders equally, and its incidence increases with age. The disease typically presents with painless adenopathy that progresses over time. Systemic symptoms, such as fevers, night sweats, and weight loss, may occur later in the disease progression and can be associated with anaemia, thrombocytopenia, and lymphocytosis. Diagnosis requires a lymph node biopsy to demonstrate the expansion of follicles filling the node and chromosomal analysis of cells from bone marrow aspiration to detect t(14:18). Chemotherapy is the primary treatment, and rituximab, a monoclonal antibody against the CD20 protein found on B cells, is often used in combination with other agents. In Burkitt’s lymphoma, c-myc overexpression is caused by a translocation between chromosomes 8 and 14, while chronic myeloid leukaemia results from a translocation between chromosomes 9 and 22, forming the Philadelphia chromosome. Ewing’s sarcoma is caused by a translocation between chromosomes 11 and 12, leading to the formation of the fusion protein Ewsr1-fli1, which causes aberrant transcription of genes regulating cell growth and development. In a small subset of follicular lymphoma patients, translocations involving the bcl-6 gene and protein are found, which may increase the risk of transformation to a more aggressive form.
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This question is part of the following fields:
- Haematology
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Question 30
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A patient who is seen in the Renal Outpatient Department for glomerulonephritis presents to the Emergency Department with a swollen, erythematosus right leg with a 4-cm difference in circumference between the right and left leg. Routine blood tests show:
Investigation Result Normal value
Sodium (Na+) 143 mmol 135–145 mmol/l
Potassium (K+) 4.2 mmol 3.5–5.0 mmol/l
Urea 10.1 mmol 2.5–6.5 mmol/l
Creatinine 120 μmol 50–120 µmol/l
eGFR 60ml/min/1.73m2
Corrected calcium (Ca2+) 2.25 mmol 2.20–2.60 mmol/l
Bilirubin 7 μmol 2–17 µmol/l
Albumin 32 g/l 35–55 g/l
Alkaline phosphatase 32 IU/l 30–130 IU/l
Aspartate transaminase (AST) 15 IU/l 10–40 IU/l
Gamma-Glutamyl transferase (γGT) 32 IU/l 5–30 IU/l
C-reactive protein (CRP) 15 mg/l 0–10 mg/l
Haemoglobin 78 g/l
Males: 135–175 g/l
Females: 115–155 g/l
Mean corpuscular volume (MCV) 92 fl 76–98 fl
Platelets 302 x 109/l 150–400 × 109/l
White cell count (WCC) 8.5 x 109/l 4–11 × 109/l
Which of the following should be commenced after confirmation of the diagnosis?Your Answer: Apixaban
Explanation:According to NICE guidance, the first-line treatment for a confirmed proximal deep vein thrombosis is a direct oral anticoagulant such as apixaban or rivaroxaban. When warfarin is used, an initial pro-coagulant state occurs, so heparin is needed for cover until the INR reaches the target therapeutic range and until day 5. Low-molecular-weight heparin is typically used with warfarin in the initial anticoagulation phase, but it can accumulate in patients with renal dysfunction. Unfractionated heparin infusion is used in these cases. For patients with normal or slightly deranged renal function, low-molecular-weight heparin can be given once per day as a subcutaneous preparation. However, warfarin is not the first-line treatment according to NICE guidance.
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This question is part of the following fields:
- Haematology
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