Hematology — MCQs

Hematology Indian Medical PG Practice Questions and MCQs

Question 971: A patient presents with pallor, glossitis, and jaundice. Labs show high MCV and hypersegmented neutrophils. What is the most likely cause?

A. Iron deficiency
B. Vitamin B12 deficiency (Correct Answer)
C. Folate deficiency
D. Hemolysis

Explanation: ***Vitamin B12 deficiency*** - The combination of **pallor**, **glossitis**, **jaundice**, **high MCV (macrocytic anemia)**, and **hypersegmented neutrophils** is classic for megaloblastic anemia due to Vitamin B12 deficiency [1]. - **Jaundice** occurs due to ineffective erythropoiesis leading to intramedullary hemolysis and release of unconjugated bilirubin. *Iron deficiency* - This typically causes **microcytic, hypochromic anemia (low MCV)**, not high MCV [2]. - While it can cause pallor and glossitis, it does not cause **hypersegmented neutrophils** or jaundice. *Folate deficiency* - Folate deficiency also causes **macrocytic anemia** and **hypersegmented neutrophils**, along with pallor and glossitis [1]. - However, **jaundice** is more characteristic of Vitamin B12 deficiency due to the more pronounced ineffective erythropoiesis and associated hemolysis. *Hemolysis* - Hemolysis can cause **jaundice** and **pallor** due to the accelerated destruction of red blood cells. - However, acute or isolated hemolysis does not typically lead to **high MCV** or **hypersegmented neutrophils**, which are hallmarks of megaloblastic anemias.

Question 972: Which of the following statements about sickle cell disease is true?

A. Sickled cells are flexible
B. Vaso-occlusion is rare
C. Chronic hemolysis does not occur
D. Autosomal recessive (Correct Answer)

Explanation: ### Autosomal recessive - Sickle cell disease is inherited in an **autosomal recessive pattern**, meaning an individual must inherit two copies of the abnormal gene (HbS) to develop the disease. - This mode of inheritance is crucial for genetic counseling and understanding family risk. [1] ### Sickled cells are flexible - **Sickled cells are rigid** and inflexible, unlike normal red blood cells, which contributes to their inability to easily pass through small blood vessels. [1] - Their stiffness is a key factor in the pathology of vaso-occlusion, as they tend to clog capillaries. ### Vaso-occlusion is rare - **Vaso-occlusion is a hallmark** and frequent complication of sickle cell disease, leading to painful crises and organ damage. [1] - The rigid, sickled red blood cells block blood flow in small vessels, causing ischemia and infarction in various tissues. ### Chronic hemolysis does not occur - **Chronic hemolysis is a prominent feature** of sickle cell disease, as the sickled red blood cells are fragile and have a shortened lifespan, leading to constant destruction. [1] - This ongoing destruction of red blood cells causes **anemia**, jaundice, and can contribute to gallstone formation. [1]

Question 973: A 45-year-old male presents with fatigue, pale skin, and jaundice. Labs reveal hemolysis and a positive direct Coombs test. What is the most likely diagnosis?

A. Hereditary spherocytosis
B. Autoimmune hemolytic anemia (Correct Answer)
C. Sickle cell anemia
D. G6PD deficiency

Explanation: ***Autoimmune hemolytic anemia*** - The combination of **hemolysis** (fatigue, pale skin, jaundice) and a **positive direct Coombs test** is highly indicative of autoimmune hemolytic anemia [1]. - A positive direct Coombs test identifies **antibodies or complement components** bound to the surface of red blood cells, leading to their destruction [1]. *Hereditary spherocytosis* - This condition is characterized by **spherocytes** on a peripheral blood smear and an **increased osmotic fragility test** [3]. - While it causes hemolysis, a **direct Coombs test** would typically be negative as it's an intrinsic red blood cell defect, not antibody-mediated [1]. *Sickle cell anemia* - Characterized by **sickle-shaped red blood cells** and is diagnosed with **hemoglobin electrophoresis**. - While it causes hemolytic anemia, the direct Coombs test is typically **negative**, and symptoms often include **vaso-occlusive crises**. *G6PD deficiency* - This is an **X-linked recessive enzymopathy** leading to bite cells and **Heinz bodies** during oxidative stress [2]. - The direct Coombs test would be **negative**, as the hemolysis is due to enzyme deficiency, not antibodies [2].

Question 974: Which of the following is used in the treatment of Hemophilia B?

A. Factor IX (Correct Answer)
B. Cryoprecipitate
C. Fresh frozen plasma
D. Factor VIII

Explanation: ***Factor IX*** - Hemophilia B is a genetic bleeding disorder caused by a deficiency of **Factor IX** (FIX), a coagulation factor produced in the liver [2]. - Treatment involves intravenous administration of **recombinant Factor IX** or Factor IX concentrates to replace the missing clotting factor and achieve hemostasis [1]. *Cryoprecipitate* - Cryoprecipitate is a blood product rich in **Factor VIII**, von Willebrand factor, fibrinogen, and Factor XIII. - It is primarily used in the management of **Hemophilia A** (Factor VIII deficiency) or von Willebrand disease, not Hemophilia B. *Fresh frozen plasma* - Fresh frozen plasma (FFP) contains **all coagulation factors**, including Factor IX, but in lower concentrations compared to specific factor concentrates. - While FFP can be used in some clotting factor deficiencies, **Factor IX concentrate** is preferred for Hemophilia B due to a higher concentration of Factor IX with a smaller fluid volume, reducing the risk of fluid overload [1]. *Factor VIII* - **Factor VIII** is the deficient clotting factor in **Hemophilia A** [2]. - Administration of Factor VIII is the primary treatment for Hemophilia A, and it is not used in the management of Hemophilia B [1].

Question 975: In folate deficiency, which of the following statements is true?

A. B12 supplementation is recommended along with folate
B. Purine and pyrimidine synthesis are affected
C. Hemolytic anemia is not a feature
D. Elevated homocysteine & normal methylmalonic acid (Correct Answer)

Explanation: ***Elevated homocysteine & normal methylmalonic acid*** - In **folate deficiency**, the conversion of homocysteine to methionine is impaired, leading to **elevated homocysteine** levels. - Unlike vitamin B12 deficiency, **methylmalonic acid (MMA)** levels remain normal in folate deficiency because folate is not involved in its metabolism. *B12 supplementation is recommended along with folate* - Supplementation with B12 alongside folate is crucial when **macrocytic anemia** is diagnosed, as it can mask a coexisting **B12 deficiency**, potentially worsening neurological symptoms if only folate is given. - However, in confirmed isolated folate deficiency, B12 supplementation is not strictly necessary unless there is suspicion or diagnosis of co-existing B12 deficiency. *Purine and pyrimidine synthesis are affected* - While folate is essential for **DNA synthesis**, indirectly affecting purine and pyrimidine production, this statement is a consequence rather than the primary diagnostic or distinguishing feature of folate deficiency. - **Folate** acts as a coenzyme in transferring one-carbon units, vital for the synthesis of **thymidylate** (a pyrimidine base) and **purine precursors**. *Hemolytic anemia is not a feature* - **Hemolytic anemia** is not typically a feature of folate deficiency; instead, it is characterized by **macrocytic, megaloblastic anemia**. - Conditions like **glucose-6-phosphate dehydrogenase (G6PD) deficiency** or **autoimmune disorders** are commonly associated with hemolytic anemia.

Question 976: Which of the following is a typical cause of unconjugated hyperbilirubinemia?

A. Gilbert syndrome (Correct Answer)
B. Hemolytic anemia
C. Crigler-Najjar syndrome
D. Dubin-Johnson syndrome

Explanation: Gilbert syndrome - This is a common, benign genetic condition characterized by a reduced activity of UDP-glucuronosyltransferase (UGT1A1), the enzyme responsible for conjugating bilirubin [1]. - The reduced enzyme activity leads to intermittent, mild unconjugated hyperbilirubinemia, typically exacerbated by stress, fasting, or illness, without significant liver disease [1]. *Hemolytic anemia* - Hemolytic anemia is characterized by an increased breakdown of red blood cells, leading to overproduction of unconjugated bilirubin [2, 3]. - While it causes unconjugated hyperbilirubinemia, it's due to excess bilirubin production overwhelming the liver's conjugating capacity, rather than a primary defect in conjugation like Gilbert syndrome [2]. *Crigler-Najjar syndrome* - This is a rare, severe genetic disorder involving a more profound deficiency or absence of the UGT1A1 enzyme, leading to significant and often life-threatening unconjugated hyperbilirubinemia [1]. - Unlike Gilbert syndrome, Crigler-Najjar syndrome typically presents with severe jaundice in infancy and can lead to kernicterus [1]. *Dubin-Johnson syndrome* - This is a rare, autosomal recessive disorder characterized by a defect in the MRP2 transporter protein, which is responsible for the excretion of conjugated bilirubin into the bile. - This condition primarily causes conjugated hyperbilirubinemia and is associated with a distinctive darkly pigmented liver on biopsy [2].

Question 977: Which of the following statements is MOST accurate regarding cyanosis in methemoglobinemia?

A. Always associated with tachycardia
B. Caused by increased carboxyhemoglobin levels
C. Does not improve with supplemental oxygen (Correct Answer)
D. Can occur in both congenital and acquired forms of the disease.

Explanation: ***Does not improve with supplemental oxygen*** - In **methemoglobinemia**, the iron in hemoglobin is oxidized from the ferrous (Fe2+) to the ferric (Fe3+) state, making it unable to bind oxygen. [1] - Therefore, despite supplemental oxygen, the **abnormal methemoglobin** cannot carry more oxygen, leading to persistent cyanosis. *Always associated with tachycardia* - While methemoglobinemia can cause **hypoxia** and compensatory **tachycardia**, it is not *always* present, especially in mild cases or if other coexisting conditions blunt the response. - The primary mechanism of cyanosis is the presence of **deoxygenated methemoglobin**, not solely the body's compensatory responses. [1] *Caused by increased carboxyhemoglobin levels* - **Carboxyhemoglobinemia** is caused by carbon monoxide poisoning, where carbon monoxide binds to hemoglobin with high affinity, preventing oxygen transport. - Methemoglobinemia is a distinct condition involving the **oxidation of iron** in hemoglobin to the ferric state. *Can occur in both congenital and acquired forms of the disease.* - This statement is generally true about methemoglobinemia itself (it can be congenital or acquired), but it doesn't directly explain the **characteristic cyanosis** and its resistance to oxygen. [1] - The question asks what is *most accurate regarding cyanosis* in the context of methemoglobinemia, focusing on the physiological manifestation rather than the disease etiology.

Question 978: A 22-year-old male with a known history of sickle cell disease presents with sudden onset chest pain, dyspnea, and cyanosis. What is the most likely diagnosis and the immediate treatment?

A. Diagnosis: Acute chest syndrome; Treatment: Provide oxygen and hydration (Correct Answer)
B. Diagnosis: Pulmonary embolism; Treatment: Start anticoagulation
C. Diagnosis: Asthma exacerbation; Treatment: Use bronchodilators
D. Diagnosis: Pneumonia; Treatment: Administer antibiotics

Explanation: Everything in the prompt remains as is, but with citations added: ***Diagnosis: Acute chest syndrome; Treatment: Provide oxygen and hydration*** - The sudden onset of chest pain, dyspnea, and cyanosis in a patient with **sickle cell disease** is highly indicative of **acute chest syndrome (ACS)**, a common and serious complication [1]. - Immediate management involves supportive care including **oxygen supplementation** to combat hypoxemia and **adequate hydration** to prevent further sickling and improve blood flow [1]. *Diagnosis: Pulmonary embolism; Treatment: Start anticoagulation* - While pulmonary embolism can cause similar symptoms, it is less likely to present with **cyanosis** as a primary feature in sickle cell disease compared to ACS. - Although anticoagulation is the treatment for PE, it is not the immediate priority for a patient with suspected ACS, where **oxygen** and **hydration** are crucial [1]. *Diagnosis: Asthma exacerbation; Treatment: Use bronchodilators* - Asthma exacerbation would be characterized by **wheezing** and a history of asthma, which are not mentioned in the patient's presentation. - While bronchodilators are the treatment for asthma, they would not address the underlying **sickling crisis** or its pulmonary manifestations [1]. *Diagnosis: Pneumonia; Treatment: Administer antibiotics* - Pneumonia can cause chest pain and dyspnea, but **cyanosis** suggests a more acute and severe oxygenation issue beyond typical pneumonia at onset. - Although antibiotics are the treatment for bacterial pneumonia, they are not the immediate first-line intervention if ACS is suspected, especially given the prompt needs for **oxygen** and **hydration** [1].

Question 979: A 60-year-old man presents with fatigue, dark urine, and jaundice. He has a history of rheumatoid arthritis and is on methotrexate. His laboratory tests show hemolytic anemia. What is the most likely cause?

A. Methotrexate-induced hemolysis
B. Autoimmune hemolytic anemia due to rheumatoid arthritis (Correct Answer)
C. G6PD deficiency after oxidative stress
D. Paroxysmal nocturnal hemoglobinuria with hemoglobinuria

Explanation: ***Autoimmune hemolytic anemia due to rheumatoid arthritis*** - **Rheumatoid arthritis** is an autoimmune disease, and patients with autoimmune conditions are at increased risk for other autoimmune phenomena, including **autoimmune hemolytic anemia (AIHA)** [1]. - The symptoms of **fatigue**, **dark urine (hemoglobinuria)**, and **jaundice** are classic presentations of hemolytic anemia, indicating increased red blood cell destruction. *Methotrexate-induced hemolysis* - While methotrexate is a known immunosuppressant used in rheumatoid arthritis, **drug-induced hemolytic anemia** is a rare side effect, and it often presents with different mechanisms than the autoimmune type. - There is no direct evidence here to suggest a drug-specific reaction over a more common autoimmune association with the underlying disease. *G6PD deficiency after oxidative stress* - **G6PD deficiency** typically causes acute hemolytic episodes triggered by specific **oxidative stressors** like certain drugs (e.g., antimalarials, sulfonamides), infections, or fava beans [2]. - Although methotrexate can cause some oxidative stress, **G6PD deficiency** is usually identified earlier in life or through specific triggers not clearly mentioned here [2]. *Paroxysmal nocturnal hemoglobinuria with hemoglobinuria* - **Paroxysmal nocturnal hemoglobinuria (PNH)** is a rare, acquired clonal disorder resulting in complement-mediated hemolysis, characterized by recurrent episodes of **dark urine, especially in the morning**. - While presenting with dark urine, PNH is typically associated with **thrombosis** and **bone marrow failure**, and is not directly linked to rheumatoid arthritis in this manner.

Question 980: A 50-year-old woman presents with fatigue and generalized weakness. Laboratory tests show a hemoglobin level of 9 g/dL and an MCV of 70 fL. What is the most likely cause of her anemia?

A. Iron deficiency anemia (Correct Answer)
B. Vitamin B12 deficiency
C. Hemolytic anemia
D. Aplastic anemia

Explanation: ***Iron deficiency anemia*** - A **hemoglobin level of 9 g/dL** indicates anemia, and an **MCV of 70 fL** signifies **microcytic** anemia, which is characteristically seen in iron deficiency [1], [2]. - **Fatigue and generalized weakness** are common symptoms of anemia due to reduced oxygen-carrying capacity [2]. *Vitamin B12 deficiency* - This typically causes **macrocytic anemia**, characterized by an **elevated MCV** (e.g., >100 fL), which contradicts the patient's MCV of 70 fL [1]. - Other symptoms often include neurological manifestations such as **paresthesias** and **ataxia**, not primarily fatigue and weakness alone. *Hemolytic anemia* - Although it can cause fatigue and weakness, **hemolytic anemia** is characterized by signs of **red blood cell destruction**, such as **jaundice**, **splenomegaly**, and elevated **reticulocyte count**, none of which are mentioned. - The MCV can be normal or slightly elevated, but not typically as low as 70 fL. *Aplastic anemia* - **Aplastic anemia** is characterized by **pancytopenia** (low levels of all blood cell types: red, white, and platelets) due to bone marrow failure. - While it causes fatigue, the **MCV is usually normal**, and other cytopenias (leukopenia, thrombocytopenia) would also be present, which are not described.

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Anemia Evaluation and Management

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Hemoglobinopathies

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Myeloproliferative Neoplasms

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Hematology — MCQs

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