Hematopathology Practice Questions

Q: HbA2 levels are increased in which of the following conditions?

Beta-thalassemia. **Explanation:** The correct answer is **C. Beta-thalassemia.** **Mechanism of Increased HbA2:** Normal adult hemoglobin consists primarily of **HbA (̢2̢2)**, with small amounts of **HbA2 (̢2̤2)** and **HbF (̢2̣2)** [3]. In Beta-thalassemia, there is a reduced or absent production of ̢-globin chains [1]. To compensate for this deficiency, the body increases the production of alternative non-̢ chains (delta and gamma) [4]. This leads to an increased proportion of HbA2 (typically >3.5%). This elevation is a hallmark diagnostic feature used to identify **Beta-thalassemia trait**. **Analysis of Incorrect Options:** * **Alpha-thalassemia:** Since HbA2 (̢2̤2) requires alpha chains, a deficiency in alpha-globin leads to **decreased or normal** HbA2 levels. * **Iron deficiency anemia (IDA):** IDA is the most common cause of a **falsely decreased** HbA2 level. Iron is a necessary cofactor for globin chain synthesis; its absence disproportionately affects delta chain production. * **Sickle cell trait (HbAS):** Patients with sickle cell trait typically have **normal** HbA2 levels. Their electrophoresis shows HbA and HbS [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Diagnostic Cut-off:** HbA2 >3.5% is diagnostic for Beta-thalassemia minor. * **The "Masking" Effect:** Co-existing Iron Deficiency Anemia can lower HbA2 levels into the normal range in a patient who actually has Beta-thalassemia trait. Therefore, iron stores should be replenished before testing for thalassemia. * **HbF levels:** While HbA2 is the primary marker for Beta-thalassemia minor, HbF is significantly elevated in Beta-thalassemia major. * **Mentzer Index:** (MCV/RBC count) 13 suggests IDA [4].

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.

Question 1

In sickle cell disease, which of the following does not influence the degree of sickling of red cells?

Accepted Answer

Intake of primaquine

Answer

Hereditary persistence of fetal hemoglobin

Answer

Intracellular pH

Answer

Co-existing a-thalassemia

Question 2

Disseminated intravascular coagulation (DIC) is seen in:

Accepted Answer

Acute promyelocytic leukemia

Answer

Acute myelomonocytic leukemia

Answer

Chronic myelogenous leukemia (CML)

Answer

Autoimmune hemolytic anemia

Question 3

If both parents are carriers of the beta-thalassemia gene, what is the chance of having a child with thalassemia major in each pregnancy?

Accepted Answer

25%

Answer

50%

Answer

75%

Answer

100%

Question 4

HbA2 levels are increased in which of the following conditions?

Accepted Answer

Beta-thalassemia

Answer

Alpha-thalassemia

Answer

Iron deficiency anemia

Answer

Sickle cell trait

Question 5

Which of the following statements about blood transfusion are true?

Accepted Answer

Anti-D is a naturally occurring antibody.

Answer

Antigen D determines Rh positivity.

Answer

Febrile reaction is due to HLA antibodies.

Answer

Cryoprecipitate contains all coagulation factors.

Question 6

Thymoma is associated with which of the following conditions?

Accepted Answer

Myasthenia gravis

Answer

Scleroderma

Answer

Oesophageal atresia

Answer

Hypergammaglobulinemia

Question 7

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 8

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 9

Iron overload occurs in all of the following conditions except:

Accepted Answer

Polycythemia vera

Answer

Thalassemia

Answer

Myelodysplastic syndrome

Answer

Sideroblastic anemia

Question 10

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

Hematopathology — MCQs

Hematopathology — MCQs

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