Hematology Practice Questions

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

Desferoxamine is the treatment of choice. 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).

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

Decrease in beta chain, increase in alpha chain. ### 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 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.

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

Equivalent to 6-8 units of random-donor platelets. **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.

Q: 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?

Sideroblastic anemia. ### 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 IDA. Sideroblastic anemia often mimics IDA on peripheral smear but is distinguished by iron studies.

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

Chronic renal failure. 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.

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

Thalassemia. **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.

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

Potassium toxicity. **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 1

What is true about Thrombocytopenic purpura?

Accepted Answer

Thrombosis in cerebral blood vessels

Answer

Hemolysis is extravascular

Answer

Normal renal function tests

Answer

Immediate cure following plasmapheresis

Question 2

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

Accepted Answer

Desferoxamine is the treatment of choice

Answer

Arthropathy involving small joints of hands may be seen

Answer

Skin pigmentation is a frequent presentation

Answer

Hypogonadism may be seen

Question 3

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

Accepted Answer

Decrease in beta chain, increase in alpha chain

Answer

Increase in beta chain, decrease in alpha chain

Answer

Decrease in beta chain, decrease in alpha chain

Answer

Increase in beta chain, increase in alpha chain

Question 4

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

Accepted Answer

Equivalent to 6-8 units of random-donor platelets

Answer

Stored at 2-6 degrees Celsius

Answer

Has a shelf life of 10 days

Answer

Leukodepletion is performed at the bedside

Question 5

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?

Accepted Answer

Sideroblastic anemia

Answer

Iron deficiency anemia

Answer

Beta-thalassemia trait

Answer

Anemia of chronic disease

Question 6

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

Accepted Answer

Chronic renal failure

Answer

Iron deficiency

Answer

Chemotherapy

Answer

Aplastic anemia

Question 7

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

Accepted Answer

Thalassemia

Answer

Christmas disease

Answer

Von Willebrand disease

Answer

Patients on brufen

Question 8

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

Accepted Answer

Serum ferritin is directly proportional to body iron stores in normal subjects.

Answer

It is the most common cause of anemia in infants and premenopausal women.

Answer

RBC indices do not change until storage iron is depleted.

Answer

Normal serum ferritin rules out the diagnosis of iron deficiency.

Question 9

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

Accepted Answer

Potassium toxicity

Answer

Citrate intoxication

Answer

Circulatory overload

Answer

Haemorrhagic diathesis

Question 10

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

Accepted Answer

Protein tyrosine kinase inhibitors are used in the treatment of CML.

Answer

The Philadelphia chromosome results from a translocation between the long arm of chromosome 9 and the short arm of chromosome 22.

Answer

CML commonly presents as blast crisis.

Answer

CML is the second most common leukemia in children.

Hematology — MCQs

Hematology — MCQs

On this page

Practice by Chapter

Want unlimited practice?