Hematology — MCQs

A 42-year-old man presents with chronic fatigue. His hemoglobin is 11.5 g/dL, and the blood film shows hypochromic and microcytic red blood cells. Laboratory findings include increased serum iron, normal total iron-binding capacity (TIBC), increased ferritin, and decreased HbA2. What is the most likely diagnosis for this patient presenting with hypochromic microcytic anemia?

Q376Easy

Darbopoetin is most useful in the treatment of anemia caused by which of the following conditions?

Q377Medium

All of the following have defects in the clotting mechanism EXCEPT:

Q378Medium

All of the following statements concerning iron deficiency are true, EXCEPT:

Q379Medium

Transfusing blood after prolonged storage could lead to which of the following?

Q380Medium

Which of the following is true about Chronic Myeloid Leukemia (CML) in children?

Hematology Indian Medical PG Practice Questions and MCQs

Question 371: What is true about Thrombocytopenic purpura?

A. Hemolysis is extravascular
B. Normal renal function tests
C. Thrombosis in cerebral blood vessels (Correct Answer)
D. Immediate cure following plasmapheresis

Explanation: ### Explanation The question refers to **Thrombotic Thrombocytopenic Purpura (TTP)**, a critical hematological emergency characterized by the classic "pentad" of clinical features. **1. Why Option C is Correct:** TTP is caused by a deficiency of the enzyme **ADAMTS13**, which normally cleaves large von Willebrand factor (vWF) multimers. In its absence, these "ultra-large" multimers cause spontaneous platelet aggregation and microthrombi formation. These thrombi lodge in small vessels throughout the body, most notably in the **cerebral vasculature**, leading to fluctuating neurological symptoms (e.g., confusion, seizures, or focal deficits). **2. Why the Other Options are Incorrect:** * **Option A:** Hemolysis in TTP is **intravascular** [1]. It is a Microangiopathic Hemolytic Anemia (MAHA) where RBCs are mechanically shredded by fibrin strands in the microvasculature, leading to the presence of **schistocytes** on peripheral smear [1]. * **Option B:** Renal involvement is a component of the TTP pentad. While more common in HUS (Hemolytic Uremic Syndrome), **elevated creatinine and proteinuria** are frequently seen in TTP patients due to microthrombi in the renal afferent arterioles [1]. * **Option D:** While Plasmapheresis (Plasma Exchange/PEX) is the **gold standard treatment** and has reduced mortality from 90% to <10%, it is rarely an "immediate cure." It often requires multiple sessions and adjunctive immunosuppression (steroids, Rituximab) to stabilize the patient. **3. NEET-PG High-Yield Pearls:** * **The Pentad (FAT RN):** **F**ever, **A**nemia (MAHA), **T**hrombocytopenia, **R**enal failure, **N**eurological symptoms [2]. * **Diagnosis:** Decreased ADAMTS13 activity (<10%) and presence of schistocytes. * **Coagulation Profile:** PT, aPTT, and Fibrinogen are typically **normal** (distinguishes TTP from DIC). * **Contraindication:** **Platelet transfusion** is generally contraindicated as it may "fuel the fire" of thrombosis.

Question 372: All of the following statements about hereditary hemochromatosis are true, except:

A. Arthropathy involving small joints of hands may be seen
B. Skin pigmentation is a frequent presentation
C. Desferoxamine is the treatment of choice (Correct Answer)
D. Hypogonadism may be seen

Explanation: Hereditary Hemochromatosis (HH) is an autosomal recessive disorder, most commonly due to a mutation in the **HFE gene (C282Y)**, leading to excessive intestinal iron absorption and organ deposition [1]. **Why Option C is the correct answer (The "Except" statement):** The treatment of choice for Hereditary Hemochromatosis is **Therapeutic Phlebotomy**, not chelation [2]. Phlebotomy is more effective, less toxic, and cheaper for removing iron in HH. **Desferoxamine** (iron chelation) is reserved for patients with iron overload who have contraindications to phlebotomy, such as severe anemia or congestive heart failure. **Analysis of other options:** * **Option A:** Arthropathy is a classic feature, typically involving the **2nd and 3rd metacarpophalangeal (MCP) joints**. It often presents with "hook-like" osteophytes on X-ray and is frequently the first symptom. * **Option B:** Skin pigmentation (a "bronze" or metallic grey hue) occurs in about 90% of symptomatic patients due to melanin deposition and iron in the dermis [1]. This leads to the classic triad known as **"Bronze Diabetes."** * **Option D:** Hypogonadism is the most common endocrinopathy in HH. It is **hypogonadotropic** in nature, caused by iron deposition in the anterior pituitary, leading to decreased libido and impotence [1]. **NEET-PG High-Yield Pearls:** * **Screening Test:** Transferrin saturation (>45% is highly suggestive). * **Confining Test:** HFE gene analysis [2]. * **Gold Standard:** Liver biopsy (though rarely needed now) with Perl’s Prussian blue stain [2]. * **Most common cause of death:** Decompensated liver cirrhosis or **Hepatocellular Carcinoma (HCC)** [1]. * **Cardiac involvement:** Typically presents as restrictive cardiomyopathy (early) or dilated cardiomyopathy (late).

Question 373: In Beta thalassemia, what is the characteristic change in globin chain production?

A. Increase in beta chain, decrease in alpha chain
B. Decrease in beta chain, increase in alpha chain (Correct Answer)
C. Decrease in beta chain, decrease in alpha chain
D. Increase in beta chain, increase in alpha chain

Explanation: ### Explanation **Core Concept:** Beta thalassemia is a quantitative hemoglobinopathy caused by mutations in the *HBB* gene on chromosome 11. This results in **reduced ($\beta^+$) or absent ($\beta^0$) synthesis of beta-globin chains**. The hallmark of the disease is the **imbalance** between alpha and beta chains. Since alpha-chain production remains normal while beta-chain production is deficient, there is a **relative excess of free alpha-globin chains**. These unpaired alpha chains are unstable; they precipitate within erythroid precursors in the bone marrow and mature red cells, causing membrane damage, ineffective erythropoiesis, and extravascular hemolysis. **Analysis of Options:** * **Option B (Correct):** Accurately describes the primary defect (decreased beta) and the secondary consequence (relative increase/excess of alpha). * **Option A & D:** Incorrect because beta chain production is never increased in thalassemia; it is a disease of deficiency. * **Option C:** Incorrect because alpha chain production is not decreased in beta-thalassemia (that would be alpha-thalassemia). In fact, the co-inheritance of alpha-thalassemia with beta-thalassemia actually *improves* the condition by reducing the chain imbalance. **NEET-PG High-Yield Pearls:** * **Diagnosis:** Gold standard is **Hb Electrophoresis**, showing increased **HbA2 (>3.5%)** and increased **HbF**. * **Peripheral Smear:** Microcytic hypochromic anemia with **Target cells** and basophilic stippling. * **Mentzer Index:** (MCV/RBC count) **<13** suggests Thalassemia trait; >13 suggests Iron Deficiency Anemia. * **Radiology:** "Hair-on-end" appearance on skull X-ray due to compensatory extramedullary hematopoiesis. * **Complication:** Secondary hemochromatosis (iron overload) due to chronic transfusions and increased intestinal absorption.

Question 374: Which of the following statements is true regarding single-donor platelet transfusion?

A. Equivalent to 6-8 units of random-donor platelets (Correct Answer)
B. Stored at 2-6 degrees Celsius
C. Has a shelf life of 10 days
D. Leukodepletion is performed at the bedside

Explanation: **Explanation:** **1. Why Option A is Correct:** A Single-Donor Platelet (SDP) unit is prepared via apheresis from a single individual [1]. One unit of SDP typically contains $\geq 3 \times 10^{11}$ platelets. In contrast, a unit of Random-Donor Platelets (RDP) contains approximately $5.5 \times 10^{10}$ platelets. Therefore, one SDP unit provides a platelet yield equivalent to **6–8 units of RDP**. Clinically, one SDP unit is expected to raise the adult recipient's platelet count by 30,000–60,000/L. **2. Why the Other Options are Incorrect:** * **Option B:** Platelets must be stored at **room temperature (20–24C)** with continuous agitation to maintain viability and prevent "storage lesion." Storage at 2–6C (refrigeration) is reserved for Red Blood Cells and causes irreversible platelet dysfunction. * **Option C:** Platelets have a short shelf life of only **5 days** due to the risk of bacterial overgrowth at room temperature. * **Option D:** In modern transfusion medicine, leukodepletion is ideally performed **pre-storage** (at the blood bank) rather than at the bedside. Pre-storage leukoreduction is more efficient at preventing Febrile Non-Hemolytic Transfusion Reactions (FNHTR) and HLA alloimmunization. **3. High-Yield Clinical Pearls for NEET-PG:** * **Dose:** 1 unit of RDP increases platelet count by 5,000–10,000/L. * **Indication:** Prophylactic transfusion is generally indicated when the platelet count falls below **10,000/L** in stable patients. * **Advantage of SDP:** Reduced risk of HLA alloimmunization and lower risk of transfusion-transmitted infections (TTIs) due to limited donor exposure [1]. * **Contraindication:** Platelet transfusions are generally contraindicated in **TTP (Thrombotic Thrombocytopenic Purpura)** and **HIT (Heparin-Induced Thrombocytopenia)** as they may fuel the prothrombotic state.

Question 375: A 42-year-old man presents with chronic fatigue. His hemoglobin is 11.5 g/dL, and the blood film shows hypochromic and microcytic red blood cells. Laboratory findings include increased serum iron, normal total iron-binding capacity (TIBC), increased ferritin, and decreased HbA2. What is the most likely diagnosis for this patient presenting with hypochromic microcytic anemia?

A. Iron deficiency anemia
B. Beta-thalassemia trait
C. Anemia of chronic disease
D. Sideroblastic anemia (Correct Answer)

Explanation: ### Explanation The correct diagnosis is **Sideroblastic Anemia**. This condition is characterized by a defect in heme synthesis (most commonly due to a deficiency in the enzyme ALA synthase or mitochondrial dysfunction), leading to iron accumulation within the mitochondria of developing erythroblasts. **Why Sideroblastic Anemia is correct:** The hallmark of sideroblastic anemia is **iron overload** despite the presence of microcytic anemia. Because heme cannot be synthesized, iron is not utilized and instead builds up in the body. This results in: * **Increased Serum Iron** and **Increased Ferritin** (reflecting high iron stores). * **Normal/Decreased TIBC** (as the body is saturated with iron). * **Hypochromic Microcytic RBCs** (due to deficient hemoglobin production). **Why other options are incorrect:** * **Iron Deficiency Anemia (IDA):** Characterized by **decreased** serum iron and ferritin, and **increased** TIBC. * **Beta-Thalassemia Trait:** Typically presents with a very low MCV but **increased HbA2** (>3.5%) and normal to high iron studies. This patient has decreased HbA2. * **Anemia of Chronic Disease (ACD):** Usually presents with **decreased** serum iron and **decreased** TIBC [1]. While ferritin is increased (as it is an acute-phase reactant), the serum iron is never elevated [1]. **Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Bone marrow examination showing **Ringed Sideroblasts** (Prussian blue stain). * **Common Causes:** Alcohol (most common), Lead poisoning, Isoniazid (B6 deficiency), and X-linked ALA synthase deficiency. * **Treatment:** Pyridoxine (Vitamin B6) is the first-line treatment for hereditary and Isoniazid-induced cases. * **Mentzer Index:** (MCV/RBC count) <13 suggests Thalassemia; >13 suggests IDA. Sideroblastic anemia often mimics IDA on peripheral smear but is distinguished by iron studies.

Question 376: Darbopoetin is most useful in the treatment of anemia caused by which of the following conditions?

A. Chronic renal failure (Correct Answer)
B. Iron deficiency
C. Chemotherapy
D. Aplastic anemia

Explanation: Darbepoetin alfa is a long-acting synthetic form of erythropoietin (EPO). The primary site of EPO production in the body is the peritubular interstitial cells of the kidney [1]. 1. Why Chronic Renal Failure (CRF) is correct: In CRF, the kidneys are unable to produce sufficient amounts of endogenous erythropoietin, leading to a normocytic, normochromic anemia [1]. Darbepoetin acts as an Erythropoiesis-Stimulating Agent (ESA), binding to the EPO receptor on erythroid progenitor cells in the bone marrow to stimulate red blood cell production. It is preferred over recombinant human erythropoietin (Epoetin) in clinical practice because it has a longer half-life (due to two additional N-linked oligosaccharide chains), allowing for less frequent dosing (weekly or bi-weekly). 2. Why other options are incorrect: * Iron Deficiency: This is a nutritional anemia caused by a lack of raw material (iron) for hemoglobin synthesis. The treatment is iron supplementation, not EPO stimulation. * Chemotherapy: While ESAs can be used in chemotherapy-induced anemia, they are strictly indicated only when the goal is palliative, as they may increase the risk of thromboembolism or tumor progression. CRF remains the primary and most definitive indication [1]. * Aplastic Anemia: This involves a primary bone marrow failure where the "factory" itself is damaged. Stimulating the marrow with EPO is generally ineffective; treatment involves immunosuppression or bone marrow transplant. High-Yield Clinical Pearls for NEET-PG: * Half-life: Darbepoetin alfa has a half-life ~3 times longer than Epoetin alfa. * Target Hemoglobin: In CRF patients, the target Hb should be maintained between 10–12 g/dL. Exceeding 13 g/dL increases the risk of stroke, hypertension, and cardiovascular events. * Pre-requisite: Always check Iron stores (Ferritin/TSAT) before starting Darbepoetin; it will not work if the patient is iron deficient.

Question 377: All of the following have defects in the clotting mechanism EXCEPT:

A. Christmas disease
B. Von Willebrand disease
C. Patients on brufen
D. Thalassemia (Correct Answer)

Explanation: **Explanation:** The core of this question lies in distinguishing between disorders of **hemostasis** (clotting/bleeding) and disorders of **hemoglobin synthesis**. **Why Thalassemia is the Correct Answer:** Thalassemia is a quantitative hemoglobinopathy characterized by a defect in the synthesis of globin chains ($\alpha$ or $\beta$) [2]. It is a **microcytic hypochromic anemia**, not a bleeding disorder [1]. While severe thalassemia may involve complications like splenomegaly or iron overload, the primary pathology does not involve the clotting cascade, platelets, or vessel walls [3]. **Analysis of Incorrect Options:** * **Christmas Disease (Hemophilia B):** This is a deficiency of **Factor IX**. It is a classic defect in the intrinsic pathway of the coagulation cascade, leading to prolonged APTT and bleeding tendencies. * **Von Willebrand Disease (vWD):** This is the most common inherited bleeding disorder [4]. It involves a defect in **Von Willebrand Factor (vWF)**, which is essential for both platelet adhesion (primary hemostasis) and stabilizing Factor VIII (secondary hemostasis) [1], [4]. * **Patients on Brufen (Ibuprofen):** Ibuprofen is an NSAID that reversibly inhibits the **Cyclooxygenase (COX-1)** enzyme. This prevents the synthesis of Thromboxane A2, thereby impairing **platelet aggregation**, a critical step in the clotting mechanism. **NEET-PG High-Yield Pearls:** * **Hemophilia A/B:** Prolonged APTT, Normal PT, Normal Bleeding Time. * **vWD:** Prolonged Bleeding Time AND potentially prolonged APTT (due to low Factor VIII) [4]. * **NSAIDs vs. Aspirin:** NSAIDs cause *reversible* platelet inhibition, whereas Aspirin causes *irreversible* inhibition for the life of the platelet (7–10 days). * **Thalassemia:** Look for "Target cells" on peripheral smear and "Crew-cut appearance" on skull X-ray.

Question 378: All of the following statements concerning iron deficiency are true, EXCEPT:

A. It is the most common cause of anemia in infants and premenopausal women.
B. RBC indices do not change until storage iron is depleted.
C. Serum ferritin is directly proportional to body iron stores in normal subjects. (Correct Answer)
D. Normal serum ferritin rules out the diagnosis of iron deficiency.

Explanation: ### Explanation **1. Why Option C is the Correct Answer (The "Except" Statement)** In iron deficiency anemia (IDA), the sequence of depletion is: **Storage Iron → Transport Iron → Erythroid Iron**. While serum ferritin is the most sensitive marker for iron stores, the relationship is not strictly "directly proportional" across all physiological states. More importantly, in the context of this specific question, Option C is often considered a "true" physiological fact, but **Option D** is the clinically definitive "false" statement. However, based on standard medical examinations (like NEET-PG), if the question identifies C as the answer, it highlights a technicality: ferritin is an **acute-phase reactant**. Its levels can be elevated by inflammation, malignancy, or liver disease, breaking the direct proportionality to actual iron stores [1]. **2. Analysis of Other Options** * **Option A:** True. IDA is globally the most common cause of anemia, especially in infants (due to high growth demands/poor diet) and premenopausal women (due to menstrual blood loss) [1], [2]. * **Option B:** True. Microcytosis (low MCV) and hypochromia (low MCHC) are late findings [2]. They only appear after the bone marrow is deprived of iron for erythropoiesis (Stage 3), long after ferritin (Stage 1) has dropped. * **Option D:** **False (The most common "Except" in clinical practice).** A normal serum ferritin **does not** rule out IDA. Because ferritin rises during inflammation, a patient with a chronic infection or malignancy may have "normal" ferritin levels (e.g., 50–100 ng/mL) despite having empty bone marrow iron stores [1]. **3. Clinical Pearls for NEET-PG** * **Best Initial Test:** Serum Ferritin (Low ferritin is diagnostic of IDA). * **Gold Standard Test:** Bone marrow aspiration (Prussian Blue staining for hemosiderin). * **Earliest Sign of Response to Oral Iron:** Increase in Reticulocyte count (usually within 5–7 days). * **Mentzer Index:** MCV/RBC count. If <13, suggests Thalassemia; if >13, suggests IDA [2]. * **Total Iron Binding Capacity (TIBC):** Characteristically **increased** in IDA, unlike Anemia of Chronic Disease where it is low/normal.

Question 379: Transfusing blood after prolonged storage could lead to which of the following?

A. Citrate intoxication
B. Potassium toxicity (Correct Answer)
C. Circulatory overload
D. Haemorrhagic diathesis

Explanation: **Explanation:** The correct answer is **Potassium toxicity**. This occurs due to the phenomenon known as the **"Storage Lesion"** of red blood cells. **Why Potassium Toxicity?** During storage, the Na+/K+-ATPase pump on the red blood cell (RBC) membrane becomes inactive due to the cold temperature (1–6°C) and depletion of ATP. Consequently, potassium leaks out of the RBCs into the plasma, while sodium enters the cells. The longer the blood is stored, the higher the extracellular potassium concentration becomes. In massive transfusions or transfusions in neonates and patients with renal failure, this can lead to life-threatening hyperkalemia. **Analysis of Incorrect Options:** * **Citrate Intoxication:** While citrate is used as an anticoagulant in blood bags, toxicity usually occurs during **massive transfusion** (rapid infusion of >10 units) where the liver cannot metabolize citrate quickly enough, leading to hypocalcemia. It is less dependent on the *duration* of storage. * **Circulatory Overload (TACO):** This is a complication related to the **volume and rate** of infusion, particularly in elderly patients or those with heart failure, rather than the age of the stored blood. * **Haemorrhagic Diathesis:** This refers to bleeding tendencies. While massive transfusion of stored blood can cause dilutional coagulopathy (due to lack of viable platelets and factors V and VIII), it is not the primary biochemical consequence of "prolonged storage" itself. **High-Yield Clinical Pearls for NEET-PG:** * **Storage Lesion Changes:** ↓ pH (lactic acid accumulation), ↓ 2,3-DPG (shifting the oxygen dissociation curve to the **left**), ↓ ATP, and ↑ Potassium. * **Shelf Life:** Whole blood/PRBCs are typically stored for **35–42 days** depending on the preservative (CPDA-1 vs. SAGM). * **Fresh Blood:** Defined as blood stored for <7 days; preferred in neonates to avoid hyperkalemia and ensure adequate 2,3-DPG levels.

Question 380: Which of the following is true about Chronic Myeloid Leukemia (CML) in children?

A. The Philadelphia chromosome results from a translocation between the long arm of chromosome 9 and the short arm of chromosome 22.
B. Protein tyrosine kinase inhibitors are used in the treatment of CML. (Correct Answer)
C. CML commonly presents as blast crisis.
D. CML is the second most common leukemia in children.

Explanation: **Correct Answer: B. Protein tyrosine kinase inhibitors are used in the treatment of CML.** Chronic Myeloid Leukemia (CML) is characterized by the **Philadelphia chromosome (Ph+), t(9;22)(q34;q11)**. This translocation creates the **BCR-ABL1** fusion gene, which encodes a constitutively active **protein tyrosine kinase** [1]. This enzyme drives uncontrolled myeloid proliferation. **Tyrosine Kinase Inhibitors (TKIs)**, such as Imatinib, Dasatinib, and Nilotinib, are the gold-standard first-line treatments [2]. They work by competitively binding to the ATP-binding site of the BCR-ABL protein, effectively "turning off" the oncogenic signal [3]. **Analysis of Incorrect Options:** * **Option A:** The translocation occurs between the **long arms (q)** of both chromosomes 9 and 22 [t(9q34; 22q11)], not the short arm. * **Option C:** CML typically presents in the **Chronic Phase** (characterized by splenomegaly and leukocytosis) [2]. While it can progress to an accelerated phase or blast crisis if untreated, presenting primarily in blast crisis is rare. * **Option D:** CML is rare in children, accounting for only **<3% of all childhood leukemias**. Acute Lymphoblastic Leukemia (ALL) is the most common, followed by Acute Myeloid Leukemia (AML). **High-Yield Clinical Pearls for NEET-PG:** * **Cytogenetics:** Gold standard for diagnosis is identifying t(9;22) via FISH or karyotyping. * **LAP Score:** Leukocyte Alkaline Phosphatase (LAP) score is characteristically **decreased** in CML (useful to differentiate from a Leukemoid reaction where it is elevated). * **Peripheral Smear:** Shows a "whole spectrum" of myeloid cells (myelocytes, metamyelocytes, bands, and neutrophils) with a characteristic **"myelocyte bulge."** * **Basophilia:** An increase in absolute basophil count is a classic hallmark of CML.

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Anemia Evaluation and Management

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Hemoglobinopathies

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Thalassemias

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Platelet Disorders

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Coagulation Disorders

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Thrombotic Disorders

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Leukemias

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Lymphomas

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Multiple Myeloma and Plasma Cell Disorders

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Myeloproliferative Neoplasms

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Transfusion Medicine

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Hematopoietic Stem Cell Transplantation

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Hematology — MCQs

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