Asplenic Patients and the Importance of Vaccination

Asplenic patients are individuals who have had their spleen removed, leaving them at risk of overwhelming bacterial infections, particularly from pneumococcus and meningococcus. To prevent such infections, it is recommended that these patients receive the Pneumovax vaccine two weeks before surgery or immediately after emergency surgery. This vaccine should be repeated every five years. Additionally, influenzae vaccination is also recommended to prevent super added bacterial infections.

While oral penicillin is recommended for children, its long-term use in adults is a topic of debate. However, current guidance suggests that splenectomized patients should receive both antibiotic prophylaxis and appropriate immunization. It is crucial to take these preventative measures to protect asplenic patients from potentially life-threatening infections.

Blood Film Abnormalities and Their Significance

Blood film abnormalities can provide important diagnostic information about a patient’s health. One such abnormality is Howell-Jolly bodies, which are nuclear remnants found in red blood cells and indicate hyposplenism. Other abnormalities seen in hyposplenism include target cells, Pappenheimer cells, increased red cell anisocytosis and poikilocytosis, and spherocytes. Patients with hyposplenism are at increased risk of bacterial infections and should be vaccinated accordingly.

Rouleaux formation, on the other hand, is a stack of red blood cells that stick together, forming a rouleau. This occurs in conditions where plasma protein is high, such as multiple myeloma, some infections, Waldenström’s macroglobulinemia, and some cancers.

Schistocytes are irregular and jagged fragments of red blood cells that occur due to mechanical destruction of red blood cells in conditions such as hemolytic anemia. They are not typically seen in hyposplenism.

Tear drop cells, which are seen in conditions where there is abnormality of bone marrow function, such as myelofibrosis, are also not seen in hyposplenism.

Finally, toxic granulation occurs during inflammatory processes such as bacterial infection or sepsis and refers to neutrophils which contain dark, coarse granules. It is not present in hyposplenism.

In summary, understanding blood film abnormalities and their significance can aid in the diagnosis and management of various medical conditions.

Blood Donation Guidelines

Blood donation guidelines set by the National Blood Service UK state that individuals who have a family member (parent or sibling) with Creutzfeldt-Jakob disease cannot donate blood. This is due to the risk of transmitting the disease through blood transfusion.

Other factors that may exclude individuals from donating blood include hepatitis or jaundice within the last 12 months, acupuncture within the last four months (unless performed by a registered professional), body piercing or tattoos within the last six months, any infection within the preceding two weeks, or a course of antibiotics within the last seven days.

It is important to follow these guidelines to ensure the safety of both the donor and the recipient. By excluding individuals who may have a higher risk of transmitting diseases or infections, the blood supply can remain safe and effective for those in need of transfusions.

Megaloblastic Anemia and Pernicious Anemia

This patient is suffering from a macrocytic anemia, specifically a megaloblastic anemia, which is characterized by multilobed nuclei. The most probable cause of this condition is a deficiency in vitamin B12, which is commonly associated with pernicious anemia. Pernicious anemia is part of the autoimmune polyendocrine syndrome, which is linked to other autoimmune disorders such as Addison’s disease, type 1 diabetes, Sjögren’s disease, and vitiligo. Although there are other potential causes of macrocytosis, none of them are evident in this patient. Hypothyroidism, for example, does not cause megaloblastic anemia, only macrocytosis.

Important Points to Remember about Fetomaternal Haemorrhage

Following the delivery of a baby, it is crucial to determine the degree of fetomaternal haemorrhage (FMH) in a D negative mother. This is done by analyzing a blood sample to adjust the dose of anti-D in the mother if she has delivered a D positive child. It is important to note that D positive and D negative women have the same likelihood of developing antibodies to other red cell antigens. Therefore, all pregnant women should undergo a blood group and antibody screen in their first trimester or at the time of presentation, whichever comes first. The fetal Rh type is determined by the Rh typing of both the mother and father. Additionally, maternal antibody titres are indicative of the degree of haemolytic disease of the newborn (HDN). For more information on the management of women with red cell antibodies during pregnancy, refer to the Royal College of Obstetricians and Gynaecologists (RCOG) Green-top Guideline No. 65.

Probable Diagnosis of Acute Lymphoblastic Leukaemia in a Child

This child is likely to have acute lymphoblastic leukaemia (ALL) based on the presence of immature white cells on their full blood count (FBC). Hodgkin’s disease is unlikely as the patient is too young and typically presents with lymphadenopathy. HIV is also not a probable cause of the immature cells on the FBC.

Acute lymphoblastic leukaemia is a type of cancer that affects the white blood cells, specifically the lymphocytes. It is most commonly diagnosed in children and young adults. Symptoms may include fatigue, fever, and easy bruising or bleeding. Treatment typically involves chemotherapy and may also include radiation therapy or stem cell transplantation. Early diagnosis and treatment are important for improving outcomes in patients with ALL.

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.

Different Types of Blood Cells and their Progenitor Cells

Blood cells are formed from different types of progenitor cells. The common myeloid progenitor cell gives rise to myeloblasts, pro-erythroblasts, megakaryoblasts, and monoblasts. Myeloblasts produce granulocytes, while pro-erythroblasts produce red blood cells. Megakaryoblasts give rise to megakaryocytes and platelets, and monoblasts produce monocytes that can become tissue-specific macrophages. B cells, NK cells, and T cells are derivatives of the common lymphoid progenitor cell. Plasma cells, which are antibody-secreting cells, are derived from B cells. Understanding the different types of blood cells and their progenitor cells is important for studying blood disorders and developing treatments.

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.

Understanding Abnormalities in Red Blood Cells: Hereditary Spherocytosis and Other Conditions

Hereditary spherocytosis is an inherited condition that causes red blood cells to take on a sphere shape instead of their normal biconcave disc shape. This abnormality leads to increased rupture of red blood cells in capillaries and increased degradation by the spleen, resulting in hypersplenism, splenomegaly, and haemolytic anaemia. Patients with hereditary spherocytosis often present with jaundice, splenomegaly, anaemia, and fatigue.

Schistocytes, irregular and jagged fragments of red blood cells, are not typically seen in hereditary spherocytosis. They are the result of mechanical destruction of red blood cells in conditions such as haemolytic anaemia.

Acanthocytes or spur cells, which have a spiked, irregular surface due to deposition of lipids and/or proteins on the membrane, are not typically seen in hereditary spherocytosis. They are seen in several conditions, including cirrhosis, anorexia nervosa, and pancreatitis.

Microcytic red blood cells, which are smaller than normal red blood cells but have a normal shape, are typically seen in iron deficiency anaemia, thalassaemia, and anaemia of chronic disease.

Teardrop-shaped red blood cells are seen in conditions where there is an abnormality of bone marrow function, such as myelofibrosis. This is different from hereditary spherocytosis, which is a primary disorder of abnormal red blood cell shape.

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.

Interpreting Blood Results for Anaemia: Understanding the Relationship between Haemoglobin and MCV

When interpreting blood results for anaemia, it is important to understand the relationship between haemoglobin and mean corpuscular volume (MCV). A low haemoglobin and reduced MCV may indicate iron deficiency anaemia secondary to menorrhagia, which is a common cause of microcytosis. Treatment for this would involve managing the underlying menorrhagia and supplementing with iron. On the other hand, a low haemoglobin and raised MCV may indicate macrocytic anaemia, commonly associated with vitamin B12 or folate deficiency. It is important to note that a normal haemoglobin with a reduced MCV or a normal haemoglobin and MCV is unlikely in cases of significant symptoms and abnormal bleeding. Understanding these relationships can aid in the diagnosis and management of anaemia.

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.

Infective Risks of Blood Transfusion

Blood transfusions carry the risk of transmitting viral infections such as hepatitis B, hepatitis C, and HIV. The likelihood of infection varies depending on the source of the donation and the type of testing used. In the UK, the risk of contracting hepatitis B from a blood transfusion is approximately 1 in 1.3 million donations. The risks for HIV and hepatitis C are even lower, at 1 in 6.5 million and 1 in 28 million donations, respectively. It is important for healthcare professionals to have a comprehensive of these risks when obtaining consent from patients for blood transfusions. Adequate knowledge and communication can help patients make informed decisions about their healthcare.

The diagnosis in this scenario is von Willebrand’s disease, which is the most common hereditary bleeding disorder caused by a defective von Willebrand factor. This protein plays a crucial role in haemostasis by assisting in platelet adhesion and stabilising coagulation factor VIII. A deficiency in von Willebrand factor prolongs bleeding time and APTT, but does not affect platelet counts or PT. It is more pronounced in women and may present with menorrhagia. Treatment involves administration of recombinant von Willebrand factor. Haemophilia A, Bernard-Soulier syndrome, Glanzmann’s thrombasthenia, and vitamin K deficiency are other bleeding disorders with different causes and blood test results.

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.

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.

Prognostic Factors in Acute Lymphoblastic Leukaemia

Acute lymphoblastic leukaemia (ALL) is a type of cancer that affects the blood and bone marrow. There are several factors that can affect the prognosis of a patient with ALL. Good prognostic factors include having the FAB L1 type, common ALL, a pre-B phenotype, and a low initial white blood cell count. On the other hand, poor prognostic factors include having the FAB L3 type, B or T cell type, the Philadelphia translocation (t(9;22)), increasing age at diagnosis, male sex, CNS involvement, and a high initial white blood cell count (e.g. > 100).

It is important for healthcare professionals to consider these prognostic factors when diagnosing and treating patients with ALL. By identifying these factors, they can better predict the outcome of the disease and tailor treatment plans accordingly. Patients with good prognostic factors may have a better chance of survival and may require less aggressive treatment, while those with poor prognostic factors may need more intensive therapy. Overall, the prognostic factors in ALL can help healthcare professionals provide the best possible care for their patients.

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.

Treatment Options for Bleeding Disorders: Haemophilia A and Von Willebrand Disease

Haemophilia A, a genetic bleeding disorder affecting men, is characterized by a propensity to bleed with minor injuries. Diagnosis is made through a prolonged APTT on a background of normal PT and bleeding time. Treatment for minor bleeds includes desmopressin and tranexamic acid, while major bleeds require infusion with recombinant factor 8. Fresh-frozen plasma and platelets are used in major trauma as replacement therapy, while heparin is an anticoagulant and should be avoided. Von Willebrand factor is given once the diagnosis of Von Willebrand disease is confirmed. Children with severe haemophilia A should receive prophylactic infusion of factor 8 at least once a week until physical maturity, and those undergoing elective surgery or pregnant women will require prophylactic treatment.