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Questions and Answers
Which of the following conditions is NOT a cause of secondary polycythemia?
Which of the following conditions is NOT a cause of secondary polycythemia?
- Chronic obstructive pulmonary disease (COPD)
- Renal artery stenosis
- Polycythemia vera (correct)
- High altitude
In primary polycythemia, erythropoietin levels are typically increased.
In primary polycythemia, erythropoietin levels are typically increased.
False (B)
What is the recommended daily hydration amount for patients with no symptoms of myeloproliferative neoplasms?
What is the recommended daily hydration amount for patients with no symptoms of myeloproliferative neoplasms?
3-4 liters
In secondary polycythemia due to renal artery stenosis, erythropoietin levels are typically __________.
In secondary polycythemia due to renal artery stenosis, erythropoietin levels are typically __________.
Match the conditions to their associated characteristics:
Match the conditions to their associated characteristics:
What is a key characteristic of Childhood Hemolytic Uremic Syndrome (HUS)?
What is a key characteristic of Childhood Hemolytic Uremic Syndrome (HUS)?
All cases of Hemolytic Uremic Syndrome are related to infections.
All cases of Hemolytic Uremic Syndrome are related to infections.
What is the primary organ affected by Hemolytic Uremic Syndrome?
What is the primary organ affected by Hemolytic Uremic Syndrome?
HUS often results in a deficiency of __________ due to the destruction of red blood cells.
HUS often results in a deficiency of __________ due to the destruction of red blood cells.
Match the following characteristics with the correct type of HUS:
Match the following characteristics with the correct type of HUS:
Which of the following is an example of an enzyme deficiency associated with inherited hemolytic anemia?
Which of the following is an example of an enzyme deficiency associated with inherited hemolytic anemia?
Hereditary spherocytosis is an autosomal recessive condition.
Hereditary spherocytosis is an autosomal recessive condition.
What is the most abundant protein on the red blood cell (RBC) membrane that Plasmodium falciparum binds to?
What is the most abundant protein on the red blood cell (RBC) membrane that Plasmodium falciparum binds to?
The most common enzyme deficiency seen in inherited hemolytic anemia is __________.
The most common enzyme deficiency seen in inherited hemolytic anemia is __________.
Match the following diseases with their associated characteristics:
Match the following diseases with their associated characteristics:
What is the primary cause of Thrombotic Thrombocytopenic Purpura (TTP) in most cases?
What is the primary cause of Thrombotic Thrombocytopenic Purpura (TTP) in most cases?
The platelet count in microangiopathic hemolytic anemia (MAHA) is usually normal.
The platelet count in microangiopathic hemolytic anemia (MAHA) is usually normal.
What is the main therapeutic approach for managing Thrombotic Thrombocytopenic Purpura (TTP)?
What is the main therapeutic approach for managing Thrombotic Thrombocytopenic Purpura (TTP)?
In TTP, autoantibodies against ADAMTS-13 lead to the accumulation of _____ in circulation.
In TTP, autoantibodies against ADAMTS-13 lead to the accumulation of _____ in circulation.
Match the following causes of hemolysis with their associated platelet counts:
Match the following causes of hemolysis with their associated platelet counts:
What is a common treatment for moderate hemolytic anemia in adults?
What is a common treatment for moderate hemolytic anemia in adults?
Splenectomy is indicated for severe disease in children as early as 3-4 years.
Splenectomy is indicated for severe disease in children as early as 3-4 years.
What is the main enzyme deficient in G6PD deficiency?
What is the main enzyme deficient in G6PD deficiency?
One of the precipitating factors for G6PD deficiency is __________.
One of the precipitating factors for G6PD deficiency is __________.
Match the precipitating drugs with their potential effects on G6PD deficiency:
Match the precipitating drugs with their potential effects on G6PD deficiency:
Which mutation is most commonly associated with Polycythemia rubra vera (PCRV)?
Which mutation is most commonly associated with Polycythemia rubra vera (PCRV)?
Primary myelofibrosis is classified under myeloproliferative neoplasms.
Primary myelofibrosis is classified under myeloproliferative neoplasms.
Name one terminal myeloid cell type.
Name one terminal myeloid cell type.
Chronic myeloid leukemia (CML) is characterized by the __________ mutation.
Chronic myeloid leukemia (CML) is characterized by the __________ mutation.
Match the following myeloproliferative neoplasms with their associated mutations:
Match the following myeloproliferative neoplasms with their associated mutations:
What is the significance of Howell-Jolly bodies in a peripheral smear?
What is the significance of Howell-Jolly bodies in a peripheral smear?
Bite cells are formed due to the degradation of hemoglobin in red blood cells.
Bite cells are formed due to the degradation of hemoglobin in red blood cells.
What are the three stages of blood loss in relation to anemia?
What are the three stages of blood loss in relation to anemia?
The three components of the triad associated with severe anemia due to blood loss are __________, hypothermia, and DIC.
The three components of the triad associated with severe anemia due to blood loss are __________, hypothermia, and DIC.
Match the following types of red blood cell inclusions with their description:
Match the following types of red blood cell inclusions with their description:
What is the most common mutation found in patients with Primary Polycythemia?
What is the most common mutation found in patients with Primary Polycythemia?
Moderate splenomegaly is a clinical feature of Primary Polycythemia.
Moderate splenomegaly is a clinical feature of Primary Polycythemia.
What is a common symptom experienced due to hyperviscosity in Primary Polycythemia?
What is a common symptom experienced due to hyperviscosity in Primary Polycythemia?
In Primary Polycythemia, patients often experience __________, which is burning pain of the hands and feet.
In Primary Polycythemia, patients often experience __________, which is burning pain of the hands and feet.
Match the clinical features with their descriptions related to Primary Polycythemia:
Match the clinical features with their descriptions related to Primary Polycythemia:
What is the main consequence of the defect in the lipid bilayer of red blood cells in this condition?
What is the main consequence of the defect in the lipid bilayer of red blood cells in this condition?
Anemia is a clinical feature associated with this condition.
Anemia is a clinical feature associated with this condition.
What is the most preferred method for evaluating red blood cell deformability?
What is the most preferred method for evaluating red blood cell deformability?
RBCs typically have a diameter of ________ µm.
RBCs typically have a diameter of ________ µm.
Match the investigations with their key features:
Match the investigations with their key features:
Which of the following conditions is associated with moderate extramedullary hematopoiesis?
Which of the following conditions is associated with moderate extramedullary hematopoiesis?
Hyperviscosity symptoms include headache, vertigo, and tinnitus.
Hyperviscosity symptoms include headache, vertigo, and tinnitus.
What is the primary cause of pancytopenia in Myelodysplastic Syndrome (MDS)?
What is the primary cause of pancytopenia in Myelodysplastic Syndrome (MDS)?
Dehydration leads to __________ relative polycythemia.
Dehydration leads to __________ relative polycythemia.
Match the types of polycythemia with their descriptions:
Match the types of polycythemia with their descriptions:
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Study Notes
Myeloproliferative Neoplasms (MPNs)
- MPNs are characterized by slow proliferation of mature myeloid cells from multiple lineages, without presence of immature cells.
- The WHO 5th edition classifies eight MPNs based on phenotypic heterogeneity and mutations.
- Polycythemia rubra vera (PCRV), the most common MPN, is characterized by JAK2 mutation, primarily seen in exon 14 as V617F mutation.
- Other MPNs include primary myelofibrosis (PMF), essential thrombocytosis/thrombocythemia (ET), chronic myeloid leukemia (CML), chronic neutrophilic leukemia (CNL), chronic eosinophilic leukemia (CEL), juvenile myelomonocytic leukemia (JMML) and MPN, not otherwise specified.
Secondary Polycythemia
- Secondary polycythemia develops due to increased EPO production due to underlying conditions like hypoxia, renal artery stenosis, and paraneoplastic syndromes.
- Hypoxia can be caused by various conditions including COPD, OSAS, high altitude, CO intoxication, and hepatopulmonary syndrome.
- Paraneoplastic syndromes include renal carcinoma, cerebellar hemangioblastoma associated with von Hippel-Lindau syndrome, meningioma, pheochromocytoma, uterine fibroids, and hepatoma.
Hemolytic Uremic Syndrome (HUS)
- HUS is characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury.
- It can be classified into childhood and adult types.
- The most common cause of adult HUS is IgG autoantibodies against ADAMTS13, a protein responsible for cleaving von Willebrand factor (vWF).
- HUS is often a life-threatening condition, requiring prompt diagnosis and treatment, including plasmapheresis, steroids, and rituximab.
Hemolytic Anemia: Miscellaneous
- Inherited hemolytic anemias can be categorized into hemoglobinopathies, membrane cytoskeleton disorders, and enzymopathies.
- Hemoglobinopathies include sickle cell anemia and thalassemia.
- Membrane cytoskeleton disorders include hereditary spherocytosis, a condition with autosomal dominant inheritance pattern caused by defects in ankyrin, Band 3, or B-spectrin.
- Enzymopathies involve deficiencies in G6PD, pyruvate kinase, and S' nucleotidase.
- S' nucleotidase deficiency is characterized by basophil stippling and markers of cholestasis.
Hereditary (Congenital) Spherocytosis (HS)
- HS is a chronic compensated extravascular hemolytic anemia caused by a defect in RBC membrane lipid bilayer, affecting vertical and tangential associations.
- This leads to microvesical formation, detachment, and spherocytosis, causing increased osmotic fragility of RBCs.
Thrombotic Thrombocytopenic Purpura (TTP)
- TTP is characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and thrombosis in small blood vessels.
- It's primarily caused by autoantibodies against ADAMTS-13, leading to accumulation of uncleaved vWF, causing platelet aggregation and microthrombi formation.
Blood Loss
- Blood loss is a common cause of anemia, triggering different stages of physiological response.
- Initial stages involve hypovolemia, followed by volume shift from extravascular to intravascular space.
- This process, involving EPO and reticulocyte production, can potentially lead to a triad of acidosis, hypothermia, and DIC (Disseminated Intravascular Coagulation).
Polycythemia
- Polycythemia refers to an increase in red blood cell count, categorized as relative or absolute.
- Relative polycythemia, also termed Gaisbock's syndrome, occurs due to decreased plasma volume, often associated with dehydration or post-viral infection like Dengue.
- Absolute polycythemia is further classified into primary (PCRV) and secondary.
- Primary polycythemia (PCRV), also known as Polycythemia vera, is a myeloproliferative neoplasm caused by JAK2 mutation and characterized by increased RBC mass, granulocytosis, thrombocytosis, and splenomegaly.
G6PD Deficiency
- G6PD deficiency is an X-linked intermediate deficiency that causes an inability to produce adequate NADPH, crucial for reducing oxidative stress in RBCs.
- It leads to acute intravascular hemolysis and chronic extravascular hemolysis.
- Precipitating factors include certain drugs, infections, and consumption of fava beans.
- Heterozygous carriers of G6PD deficiency exhibit resistance to malaria.
Treatment
- Treatment for G6PD deficiency primarily involves avoiding precipitating drugs, infections, and fava beans.
- For severe cases, supportive care, splenectomy, and management of complications such as jaundice and gallstones may be needed.
Blood Transfusion
- Blood transfusions are often used to address anemia caused by various conditions including blood loss, hemolytic anemia, and myelodysplastic syndromes.
Splenectomy
- Splenectomy is a surgical procedure involving the removal of the spleen.
- In cases of hereditary spherocytosis, splenectomy is often performed to reduce the rate of red blood cell destruction.
- Splenectomy is also sometimes performed for other conditions like thalassemia, sickle cell anemia, and certain types of lymphoma.
Investigative Procedures
- Peripheral smear analysis is a key tool for diagnosing anemias and other blood disorders.
- It allows for visual examination of erythrocytes, leukocytes, and platelets, revealing abnormalities like Howell-Jolly bodies, Heinz bodies, spherocytes, target cells, and other morphological changes.
- Various other test methods are used to investigate specific types of anemia, including osmotic fragility test, Ektacytometry, SDS page electrophoresis, EMA test, and quantitative assays.
- Fluorescent spot test is a screening technique used to detect G6PD deficiency.
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