Hematopathology Practice Questions

Q: Which of the following conditions exhibits X-linked recessive inheritance?

G6PD deficiency. **Explanation:** **1. Correct Option: G6PD Deficiency** Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is an **X-linked recessive** enzymopathy. The G6PD gene is located on the long arm of the X chromosome (Xq28). Because of this inheritance pattern, the condition primarily affects males, while females are typically asymptomatic carriers [1]. However, females can manifest the disease due to **unfavorable lyonization** (random X-inactivation). The deficiency leads to inadequate NADPH production, making RBCs susceptible to oxidative stress, resulting in hemolysis and the formation of **Heinz bodies** and **Bite cells**. **2. Incorrect Options:** * **Sickle Cell Anaemia:** This is an **Autosomal Recessive** disorder caused by a point mutation (Glu → Val) in the β-globin chain. * **Thalassemia:** Both Alpha and Beta thalassemias are **Autosomal Recessive** conditions involving quantitative defects in globin chain synthesis. * **Hereditary Spherocytosis:** This is most commonly inherited in an **Autosomal Dominant** pattern (approx. 75% of cases), involving defects in RBC membrane proteins like Ankyrin or Spectrin. **3. NEET-PG High-Yield Pearls:** * **Common Triggers:** Fava beans, infections, and drugs (Primaquine, Sulphonamides, Nitrofurantoin). * **Morphology:** Heinz bodies (denatured hemoglobin) are visualized with **Supravital stains** (Crystal violet/Methylene blue). Bite cells (Degmacytes) are formed in the splenic sinusoids. * **Protection:** G6PD deficiency provides a selective advantage against *Plasmodium falciparum* malaria. * **Timing of Test:** Do not perform the G6PD enzyme assay during an acute hemolytic episode, as young reticulocytes have normal enzyme levels, leading to a **false-negative** result. Wait 6–8 weeks. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, p. 151.

Q: What is true about nodular lymphocytic predominant Hodgkin's lymphoma?

Has a good prognosis. **Nodular Lymphocyte Predominant Hodgkin Lymphoma (NLPHL)** is a distinct clinical and biological entity that differs significantly from Classical Hodgkin Lymphoma (CHL) [1]. ### **Explanation of the Correct Answer** **Option D is correct.** NLPHL is characterized by an indolent clinical course and an excellent long-term prognosis [1]. Most patients present with localized (Stage I or II) peripheral lymphadenopathy, typically involving cervical, axillary, or inguinal nodes [1]. It has a high cure rate, although it is prone to late relapses and carries a small risk (approx. 3-5%) of transformation into Diffuse Large B-Cell Lymphoma (DLBCL) [1]. ### **Why Other Options are Incorrect** * **Option A:** Instead of classical Reed-Sternberg (RS) cells, NLPHL features **"Popcorn cells"** (L&H cells—Lymphocytic and Histiocytic variants) [1]. These cells have multi-lobed, delicate nuclei resembling popped corn [1]. * **Option B:** Unlike CHL, Popcorn cells are **CD15 negative and CD30 negative**. They represent a B-cell lineage and are characteristically **CD20 positive** and **BCL6 positive**. * **Option C:** While the background contains many reactive T cells, the neoplastic cells (Popcorn cells) are of **B-lymphocyte origin** [1]. ### **High-Yield Clinical Pearls for NEET-PG** * **Immunophenotype:** CD20+, CD45+, CD75+, BCL6+, EMA (often positive); CD15-, CD30-. * **Gender/Age:** More common in males, typically in the 30–50 age group [1]. * **Microscopic Pattern:** Shows a nodular growth pattern of small B-lymphocytes, follicular dendritic cell meshworks, and scattered L&H cells [1]. * **Treatment:** Because it is CD20+, **Rituximab** is an effective targeted therapy, unlike in Classical Hodgkin Lymphoma. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 614-618.

Question 1

Which of the following is NOT a characteristic feature of Chronic Lymphocytic Leukemia (CLL)?

Accepted Answer

Onset typically in individuals over 50 years of age, with a slight predilection for males

Answer

Presence of small lymphocytes in peripheral smear

Answer

Hepatosplenomegaly

Answer

Expression of ZAP-70 is a marker

Question 2

A patient presents with a platelet count of 700 x 10^9/L with abnormalities in size, shape, and granularity of platelets. The WBC count is 12 x 10^9/L, hemoglobin is 11 g/dL, and the Philadelphia chromosome is absent. What is the most likely diagnosis?

Accepted Answer

Essential thrombocythemia

Answer

Polycythemia vera

Answer

Chronic myeloid leukemia

Answer

Leukemoid reaction

Question 3

What are the diagnostic criteria for Chronic Myeloid Leukemia (CML)?

Accepted Answer

Philadelphia chromosome (t(9;22))

Answer

Auer rods

Answer

Basophilia

Answer

Leukocyte Alkaline Phosphatase (LAP) score

Question 4

Giant platelets are seen in all the following conditions except:

Accepted Answer

Glanzmann thrombasthenia

Answer

Bernard-Soulier syndrome

Answer

May-Hegglin anomaly

Answer

Gray platelet syndrome

Question 5

All of the following can cause aplastic anemia except?

Accepted Answer

Wiskott-Aldrich syndrome

Answer

Fanconi's anemia

Answer

Dyskeratosis congenital

Answer

Reticular dysgenesis

Question 6

Which of the following conditions exhibits X-linked recessive inheritance?

Accepted Answer

G6PD deficiency

Answer

Sickle cell anaemia

Answer

Thalassemia

Answer

Hereditary spherocytosis

Question 7

Purpura fulminans is seen in which of the following conditions?

Accepted Answer

Severe DIC

Answer

Leukocytoclastic vasculitis

Answer

Viral hemorrhagic fever

Answer

Tularemia

Question 8

What is true about nodular lymphocytic predominant Hodgkin's lymphoma?

Accepted Answer

Has a good prognosis

Answer

Consists predominantly of classical Reed-Sternberg cells

Answer

CD15 and CD30 positive

Answer

Composed of T lymphocytes

Question 9

Glucose added to stored blood is beneficial for which of the following reasons?

Accepted Answer

To prevent hemolysis

Answer

To provide nutrition for one week

Answer

To increase blood acidosis

Answer

To prevent hyperkalemia

Question 10

In Non-Hodgkin's lymphoma, early involvement of the bone marrow is typical of which variety?

Accepted Answer

Nodular

Answer

Diffuse

Answer

Lymphocytic well differentiated

Answer

Lymphocytic poorly differentiated

Hematopathology — MCQs

Hematopathology — MCQs

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