Hematology Practice Questions

Q: An 18-year-old woman presents with a low hemoglobin value of 10.5 g/dL on routine CBC testing. The red cells are microcytic, but WBC and platelet counts are normal. Her iron intake is adequate, menstrual flow is normal, and there is no other history of blood loss. Physical examination is normal, and iron studies reveal a normal ferritin level. She reports that other family members have also been diagnosed with anemia and alpha-thalassemia. Which of the following hemoglobins is increased in alpha-thalassemia?

Hemoglobin H. The clinical presentation of microcytic anemia with normal ferritin levels and a positive family history strongly suggests a thalassemia syndrome. In **Alpha-Thalassemia**, there is a deficiency in the production of alpha-globin chains. Since alpha chains are essential components of all normal adult hemoglobins (HbA, HbA2, and HbF), a deficiency leads to an excess of the remaining non-alpha chains. 1. **Why Hemoglobin H is correct:** In alpha-thalassemia (specifically the 3-gene deletion or HbH disease), the relative excess of beta-globin chains leads them to aggregate into tetramers ($\beta_4$). These tetramers are known as **Hemoglobin H**. In neonates with alpha-thalassemia, excess gamma chains form tetramers ($\gamma_4$) known as **Hb Barts**. 2. **Why other options are incorrect:** * **HbA ($\alpha_2\beta_2$):** This is the major adult hemoglobin. In alpha-thalassemia, its production is decreased, not increased. * **HbF ($\alpha_2\gamma_2$) and HbA2 ($\alpha_2\delta_2$):** Both require alpha chains. Unlike Beta-thalassemia (where HbA2 and HbF increase to compensate for the lack of beta chains), in Alpha-thalassemia, these levels are typically **normal or decreased** because the alpha-chain substrate is the limiting factor. **High-Yield Clinical Pearls for NEET-PG:** * **HbH Disease:** Characterized by a "3-gene deletion" (- - / - $\alpha$). * **Golf Ball Appearance:** On supra-vital staining (Brilliant Cresyl Blue), HbH precipitates as multiple small inclusions, giving RBCs a "golf ball" appearance. * **Mentzer Index:** (MCV/RBC count) 13 suggests Iron Deficiency Anemia. * **Diagnosis:** Hb Electrophoresis is the gold standard, but in alpha-thalassemia trait (1 or 2 gene deletion), electrophoresis may be normal; definitive diagnosis requires genetic testing.

Q: Macrocytosis is seen in all of the following disorders, except?

Thalassemia major. Macrocytosis refers to an increased Mean Corpuscular Volume (MCV > 100 fL). To solve this question, one must distinguish between causes of macrocytic and microcytic anemia. **Why Thalassemia major is the correct answer:** Thalassemia major is a quantitative defect in globin chain synthesis, leading to ineffective erythropoiesis [3]. It is a classic cause of **microcytic hypochromic anemia** (MCV < 80 fL), not macrocytosis. In Thalassemia, the lack of hemoglobin leads to smaller, paler red blood cells. **Analysis of Incorrect Options:** * **Folic acid and Vitamin B12 deficiency:** These are the most common causes of **Megaloblastic Macrocytic Anemia** [1], [2]. Deficiency leads to impaired DNA synthesis while RNA synthesis remains intact, resulting in a "nuclear-cytoplasmic asynchrony" where the nucleus matures slower than the cytoplasm, creating large cells (macro-ovalocytes). * **Hypothyroidism:** This is a common cause of **Non-megaloblastic Macrocytic Anemia**. The exact mechanism is multifactorial, involving a decrease in erythropoietin levels and changes in the RBC lipid membrane. **NEET-PG High-Yield Pearls:** 1. **Megaloblastic vs. Non-megaloblastic:** Megaloblastic causes (B12/Folate deficiency, drugs like Methotrexate) show **hypersegmented neutrophils** (>5 lobes), whereas non-megaloblastic causes (Alcohol, Hypothyroidism, Liver disease) do not. 2. **Mentzer Index:** Used to differentiate Iron Deficiency Anemia (IDA) from Thalassemia. * Index (MCV/RBC count) ** 13** suggests IDA. 3. **Round vs. Oval:** Macrocytes in B12/Folate deficiency are typically **oval** (macro-ovalocytes), while those in liver disease or hypothyroidism are usually **round**.

Q: All of the following are conditions that precipitate hemolysis in G6PD deficiency, except?

Intake of acetaminophen and aspirin. **Explanation:** Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is an X-linked recessive disorder where the RBCs are unable to regenerate reduced glutathione, making them vulnerable to oxidative stress [3][4]. When exposed to oxidizing agents, hemoglobin denatures into **Heinz bodies**, leading to episodic hemolysis [1][3]. **Why Option D is Correct:** Acetaminophen and Aspirin (at standard therapeutic doses) are considered **safe** in G6PD deficiency. While high-dose aspirin was historically debated, current hematological guidelines list these as non-oxidizing agents that do not precipitate clinically significant hemolysis. **Why Other Options are Incorrect:** * **A. Ingestion of fava beans:** This causes "Favism." Fava beans contain vicine and covicine, which produce free radicals that overwhelm the limited antioxidant capacity of G6PD-deficient cells [1]. * **B. Viral hepatitis:** Infection is the **most common trigger** for hemolysis in G6PD deficiency [1]. Inflammatory responses generate reactive oxygen species (ROS) from polymorphonuclear leukocytes, causing oxidative damage to RBCs. * **C. Antimalarials:** Primaquine is the classic "textbook" trigger [1]. It undergoes redox cycling, generating superoxide anions that cause rapid hemolysis in deficient individuals [2]. **NEET-PG High-Yield Pearls:** * **Inheritance:** X-linked Recessive (Common in Mediterranean and African populations) [1][3]. * **Peripheral Smear:** Look for **Heinz Bodies** (denatured Hb) and **Bite Cells/Degmacytes** (formed when splenic macrophages pluck out Heinz bodies) [3]. * **Diagnosis:** Be careful! G6PD levels may be **falsely normal** during an acute hemolytic episode because the most deficient (older) cells have already lysed [1]. Re-testing should be done 6–8 weeks later. * **Key Contraindicated Drugs:** Primaquine, Dapsone, Nitrofurantoin, Rasburicase, and Sulfonamides [1].

Q: What type of red blood cells (RBCs) are typically seen in chronic renal failure?

Normocytic. ### Explanation **Correct Option: C. Normocytic** The anemia associated with **Chronic Renal Failure (CRF)** is typically **Normocytic Normochromic Anemia**. **Why it is correct:** The primary pathophysiology in CRF is the **deficiency of Erythropoietin (EPO)** [1]. EPO is a glycoprotein hormone produced by the peritubular interstitial cells of the kidney in response to hypoxia [1], [2]. In chronic kidney disease, the functional renal mass decreases, leading to inadequate EPO production [1]. Since the bone marrow is healthy but lacks the hormonal signal to produce more cells, the RBCs produced are normal in size (normocytic) and color (normochromic), but their absolute number is decreased. Other contributing factors include the accumulation of uremic toxins (which shorten RBC lifespan) and chronic inflammation (Anemia of Chronic Disease). **Why other options are incorrect:** * **A. Microcytic:** This is characteristic of Iron Deficiency Anemia (IDA) or Thalassemia. While CRF patients can develop IDA due to hemodialysis blood loss or poor absorption, the *primary* anemia of renal failure itself is normocytic. * **B. Macrocytic:** This is seen in Vitamin B12 or Folic acid deficiency. While some drugs used in renal patients might interfere with folate metabolism, it is not the classic presentation of renal anemia. **High-Yield Clinical Pearls for NEET-PG:** * **Target Hemoglobin:** In patients on Erythropoiesis-Stimulating Agents (ESAs), the target Hb is usually **10–11.5 g/dL**. Aiming for normal levels (>13 g/dL) increases the risk of stroke and cardiovascular events (CHOIR and CREATE trials). * **Burr Cells (Echinocytes):** These are small, spiked RBCs often seen on the peripheral smear of patients with uremia/CRF. * **First step in management:** Before starting EPO therapy, always ensure **iron stores are adequate** (Transferrin saturation >20% and Ferritin >100 ng/mL).

Q: An abnormal Ham test is most likely associated with which of the following?

Defective GPI anchor. **Explanation:** The **Ham test** (Acidified Serum Lysis test) is a classic diagnostic test for **Paroxysmal Nocturnal Hemoglobinuria (PNH)**. **1. Why the Correct Answer is Right:** PNH is an acquired clonal hematopoietic stem cell disorder caused by a somatic mutation in the **PIGA gene**. This mutation leads to a deficiency of **Glycosylphosphatidylinositol (GPI) anchors**. These anchors are essential for attaching protective proteins, such as **CD55** (Decay Accelerating Factor) and **CD59** (Membrane Inhibitor of Reactive Lysis), to the red blood cell membrane. Without these GPI-anchored proteins, RBCs become hypersensitive to complement-mediated lysis. The Ham test works by acidifying serum to activate the alternative complement pathway; PNH cells, lacking GPI-anchored protectors, will undergo lysis. **2. Why Other Options are Incorrect:** * **Option A (Defect in spectrin):** This is the hallmark of **Hereditary Spherocytosis**. It leads to osmotic fragility, not complement-mediated lysis. * **Option C (Defect in complement):** In PNH, the complement system itself is normal; the defect lies in the RBC's inability to *regulate* complement on its surface. * **Option D (Mannose-binding residue defect):** This refers to Mannose-Binding Lectin (MBL) deficiency, which is associated with increased susceptibility to infections, not PNH. **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** While the Ham test is historically significant, **Flow Cytometry** (detecting absence of CD55/CD59) is now the gold standard. * **FLAER (Fluorescently Labeled Aerolysin):** A highly sensitive flow cytometry-based test used to detect the absence of GPI anchors. * **Triad of PNH:** Hemolytic anemia, Pancytopenia, and Venous Thrombosis (often in unusual sites like the Budd-Chiari syndrome). * **Treatment:** **Eculizumab**, a monoclonal antibody against C5, is the drug of choice.

Q: Hunter's glossitis is a feature of which condition?

Pernicious anemia. **Explanation:** **Hunter’s glossitis** (also known as Moeller’s glossitis) is a classic clinical sign of **Pernicious anemia**, which results from Vitamin B12 deficiency due to a lack of intrinsic factor. 1. **Why Pernicious Anemia is correct:** Vitamin B12 is essential for DNA synthesis and the rapid turnover of mucosal cells [3]. In its absence, the lingual papillae undergo atrophy. This results in a characteristic **"beefy red," smooth, and shiny appearance** of the tongue, often accompanied by a burning sensation or soreness [1]. 2. **Why other options are incorrect:** * **Leukemia:** Typically presents with gingival hypertrophy (especially in AML-M5) [1], oral candidiasis, or petechiae due to thrombocytopenia, rather than isolated atrophic glossitis. * **Sickle cell anemia:** Oral manifestations are usually related to bone changes (e.g., "crew-cut" appearance on X-ray) or mucosal pallor due to chronic hemolysis [4], not specific glossitis. * **HIV infection:** Common oral findings include Hairy Leukoplakia (caused by EBV), Kaposi sarcoma, or Oral Candidiasis, but not Hunter’s glossitis. **High-Yield Clinical Pearls for NEET-PG:** * **Triad of Vitamin B12 Deficiency:** Megaloblastic anemia, Hunter’s glossitis, and Neurological symptoms (Subacute Combined Degeneration of the Spinal Cord). * **Peripheral Smear:** Look for **Hypersegmented neutrophils** (earliest sign) and Macro-ovalocytes. * **Schilling Test:** Historically used to differentiate B12 malabsorption causes (now largely replaced by antibody testing for Anti-Intrinsic Factor and Anti-Parietal Cell antibodies) [2]. * **Other causes of Atrophic Glossitis:** Iron deficiency anemia (Plummer-Vinson Syndrome), sprue, and other B-complex deficiencies [1].

Question 1

An 18-year-old woman presents with a low hemoglobin value of 10.5 g/dL on routine CBC testing. The red cells are microcytic, but WBC and platelet counts are normal. Her iron intake is adequate, menstrual flow is normal, and there is no other history of blood loss. Physical examination is normal, and iron studies reveal a normal ferritin level. She reports that other family members have also been diagnosed with anemia and alpha-thalassemia. Which of the following hemoglobins is increased in alpha-thalassemia?

Accepted Answer

Hemoglobin H

Answer

Hemoglobin A

Answer

Hemoglobin F

Answer

Hemoglobin A2

Question 2

Schilling test is performed to assess for what type of deficiency?

Accepted Answer

Vitamin B12 malabsorption

Answer

Folic acid level

Answer

Pancreatic enzyme deficiency

Answer

Coronary artery disease

Question 3

A 40-year-old chronic alcoholic is investigated for anemia. Tests reveal increased serum iron and increased transferrin saturation. What is the probable diagnosis?

Accepted Answer

Hemosiderosis

Answer

Iron deficiency anemia

Answer

Sideroblastic anemia

Answer

Megaloblastic anemia

Question 4

Which of the following is not a side effect of blood transfusion?

Accepted Answer

Hypokalemia

Answer

Hypomagnesemia

Answer

Hypocalcemia

Answer

Iron overload

Question 5

Macrocytosis is seen in all of the following disorders, except?

Accepted Answer

Thalassemia major

Answer

Hypothyroidism

Answer

Folic acid deficiency

Answer

Vitamin B12 deficiency

Question 6

All of the following are conditions that precipitate hemolysis in G6PD deficiency, except?

Accepted Answer

Intake of acetaminophen and aspirin

Answer

Ingestion of fava beans

Answer

Viral hepatitis

Answer

Antimalarials such as primaquine and chloroproguanil

Question 7

What is the positive finding in a patient with anemia due to chronic inflammation?

Accepted Answer

Serum iron is increased

Answer

Serum ferritin is decreased

Answer

Total iron-binding capacity (TIBC) is decreased

Answer

Presence of normal iron in blasts

Question 8

What type of red blood cells (RBCs) are typically seen in chronic renal failure?

Accepted Answer

Normocytic

Answer

Microcytic

Answer

Macrocytic

Answer

None of the above

Question 9

An abnormal Ham test is most likely associated with which of the following?

Accepted Answer

Defective GPI anchor

Answer

Defect in spectrin

Answer

Defect in complement

Answer

Mannose-binding residue defect

Question 10

Hunter's glossitis is a feature of which condition?

Accepted Answer

Pernicious anemia

Answer

Leukemia

Answer

Sickle cell anemia

Answer

HIV infection

Hematology — MCQs

Hematology — MCQs

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