Hematopathology Practice Questions

Q: Iron overload occurs in all of the following conditions except:

Polycythemia vera. **Explanation:** Iron overload (hemosiderosis) typically occurs due to two main mechanisms: repeated blood transfusions or ineffective erythropoiesis leading to increased intestinal iron absorption. **Why Polycythemia Vera is the correct answer:** Polycythemia Vera (PV) is a myeloproliferative neoplasm characterized by the autonomous overproduction of red blood cells. Because the body is rapidly utilizing iron to synthesize hemoglobin for the massive excess of erythrocytes, patients with PV are actually prone to **iron deficiency**, not overload. Therapeutic phlebotomy (the standard treatment for PV) further depletes iron stores. **Analysis of Incorrect Options:** * **Thalassemia:** Patients develop iron overload due to both chronic blood transfusions and increased dietary iron absorption triggered by "ineffective erythropoiesis" (erythroid hyperplasia). * **Myelodysplastic Syndrome (MDS):** These patients often suffer from refractory anemia requiring lifelong blood transfusions. Each unit of packed RBCs contains ~200–250 mg of iron, which the body cannot actively excrete. * **Sideroblastic Anemia:** This condition involves a defect in porphyrin synthesis, preventing iron from being incorporated into hemoglobin. Iron accumulates in the mitochondria of erythroblasts (forming ringed sideroblasts), leading to systemic overload. **High-Yield Clinical Pearls for NEET-PG:** * **Prussian Blue Stain:** The gold standard histological stain to visualize iron (hemosiderin) in tissues or bone marrow. * **Hereditary Hemochromatosis:** An autosomal recessive disorder (HFE gene mutation) causing primary iron overload. * **Classic Triad:** Bronze skin, Cirrhosis, and Diabetes mellitus ("Bronze Diabetes"). * **Hepcidin:** The key regulatory hormone produced by the liver that inhibits iron absorption; it is suppressed in states of ineffective erythropoiesis.

Q: A 3-year-old child presented with progressive anemia, jaundice, and failure to thrive. On examination, pallor and splenomegaly are seen. Peripheral smear showed normoblasts and small round intensely stained red cells. What is the likely diagnosis?

Hereditary spherocytosis. ### Explanation **1. Why Hereditary Spherocytosis (HS) is Correct:** The clinical triad of **anemia, jaundice, and splenomegaly** in a young child strongly suggests a chronic hemolytic process. The definitive clue lies in the peripheral smear: **"small round intensely stained red cells"** are **Spherocytes**. These cells lack central pallor because they have lost their biconcave shape due to defects in RBC membrane proteins (most commonly **Ankyrin**, followed by Spectrin). The presence of **normoblasts** (nucleated RBCs) indicates a robust compensatory bone marrow response to hemolysis. **2. Why Other Options are Incorrect:** * **Thalassemia:** While it presents with anemia and splenomegaly, the peripheral smear typically shows **microcytic hypochromic** cells, target cells, and basophilic stippling, not spherocytes. * **Sickle Cell Anemia:** Characterized by **sickle-shaped cells** and Howell-Jolly bodies. Splenomegaly is usually seen only in early childhood; later, "autosplenectomy" occurs due to repeated infarcts. * **Vitamin B12 Deficiency:** This is a **megaloblastic anemia** (macrocytic). The smear would show macro-ovalocytes and hypersegmented neutrophils, not spherocytes or jaundice from hemolysis. **3. High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Most commonly Autosomal Dominant. * **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test (Flow cytometry). * **Screening Test:** Osmotic Fragility Test (increased fragility). * **MCHC:** Characteristically **elevated** (>36 g/dL) due to relative dehydration of the cell. * **Complications:** Pigmented gallstones (calcium bilirubinate) and Aplastic crisis (associated with **Parvovirus B19**). * **Treatment of Choice:** Splenectomy (usually deferred until after age 5–6 to reduce sepsis risk).

Question 1

Thymoma is associated with which of the following conditions?

Accepted Answer

Myasthenia gravis

Answer

Scleroderma

Answer

Oesophageal atresia

Answer

Hypergammaglobulinemia

Question 2

All of the following are T cell lymphomas, EXCEPT:

Accepted Answer

Lymphoplasmacytic lymphoma

Answer

Mycosis fungoides

Answer

Anaplastic large cell lymphoma

Answer

Aggressive NK cell leukemia

Question 3

A patient presented with splenomegaly, anemia, and reticulocytosis with increased bone marrow cellularity. What is the most likely diagnosis?

Accepted Answer

Hemolytic anemia

Answer

Pernicious anemia

Answer

Myelofibrosis

Answer

Hairy cell leukemia

Question 4

Iron overload occurs in all of the following conditions except:

Accepted Answer

Polycythemia vera

Answer

Thalassemia

Answer

Myelodysplastic syndrome

Answer

Sideroblastic anemia

Question 5

Bence Jones proteins are associated with which of the following conditions?

Accepted Answer

Multiple myeloma

Answer

Hodgkin's lymphoma

Answer

Burkitt's Lymphoma

Answer

Infectious mononucleosis

Question 6

Dohle bodies are seen in which of the following conditions?

Accepted Answer

Burns

Answer

Multiple myeloma

Answer

Waldenstrom macroglobulinemia

Answer

Lymphoma

Question 7

Splenic infarction is associated with which of the following conditions?

Accepted Answer

Infective endocarditis

Answer

Typhoid fever

Answer

Chronic myeloid leukemia

Answer

Paroxysmal nocturnal hemoglobinuria

Question 8

A 3-year-old child presented with progressive anemia, jaundice, and failure to thrive. On examination, pallor and splenomegaly are seen. Peripheral smear showed normoblasts and small round intensely stained red cells. What is the likely diagnosis?

Accepted Answer

Hereditary spherocytosis

Answer

Thalassemia

Answer

Sickle cell anemia

Answer

Vitamin B12 deficiency anemia

Question 9

Reed Sternberg cells are characteristic of which type of cell?

Accepted Answer

B cells

Answer

T cells

Answer

Natural killer cells

Answer

All of the above

Question 10

All are true about iron deficiency anemia EXCEPT?

Accepted Answer

Presence of sideroblasts

Answer

Hypochromic microcytic red blood cells

Answer

Serum ferritin as a marker

Answer

Anisocytosis and poikilocytosis

Hematopathology — MCQs

Hematopathology — MCQs

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