Hematopathology Practice Questions

Q: The Fleischer assay is used for which of the following conditions?

Paroxysmal nocturnal hemoglobinuria. **Explanation:** **Paroxysmal Nocturnal Hemoglobinuria (PNH)** is an acquired clonal hematopoietic stem cell disorder characterized by a mutation in the **PIGA gene**. This mutation leads to a deficiency of Glycosylphosphatidylinositol (GPI) anchors, which are necessary to attach protective proteins like **CD55 (DAF)** and **CD59 (MIRL)** to the red blood cell membrane [1]. Without these proteins, RBCs are highly susceptible to complement-mediated lysis. The **Fleischer Assay** (also known as the **Proaerolysin variant assay**) is a specialized diagnostic test for PNH. It utilizes a fluorescently labeled, inactive variant of **Aerolysin** (a bacterial toxin from *Aeromonas hydrophila*). Unlike the traditional Ham’s test or Sucrose Hemolysis test, which are now largely obsolete, the Fleischer assay (specifically **FLAER** - Fluorescent Proaerolysin) binds directly to the GPI anchors. In PNH, the absence of these anchors results in a lack of binding, which is then detected via flow cytometry [1]. This is currently the "Gold Standard" for diagnosis due to its high sensitivity and specificity. **Why other options are incorrect:** * **Hereditary Spherocytosis:** Diagnosed via the Osmotic Fragility Test or the **EMA (Eosin-5-maleimide) binding test**. * **Thalassemia:** Diagnosed via **Hb Electrophoresis** or HPLC to identify abnormal hemoglobin chains. **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard for PNH:** Flow cytometry using **FLAER**. * **Classic Triad:** Hemolytic anemia, pancytopenia, and venous thrombosis (often in unusual sites like the Budd-Chiari syndrome) [1]. * **Key Markers:** Deficiency of CD55 and CD59. * **Treatment:** Eculizumab (a monoclonal antibody against C5 complement). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 650-651.

Q: What is the most common inherited thrombotic disorder?

Factor V Leiden mutation. **Explanation:** **Factor V Leiden mutation** is the most common inherited cause of hypercoagulability (thrombophilia) in Caucasian populations, occurring in approximately 2–15% of the general population [1]. **Why it is correct:** The condition results from a specific point mutation in the Factor V gene (G1691A), where Glutamine replaces Arginine at position 506 [1]. This site is the normal cleavage point for **Activated Protein C (APC)**. Because the mutated Factor V is resistant to degradation by APC, it remains active in the coagulation cascade for longer, leading to a prothrombotic state. This phenomenon is known as **APC Resistance** [1]. **Why other options are incorrect:** * **Protein C and S deficiencies:** While these are well-known inherited thrombophilic conditions, they are significantly rarer than Factor V Leiden, occurring in less than 1% of the population. * **tPA (Tissue Plasminogen Activator) deficiency:** This is an extremely rare cause of thrombosis. Most clinical thrombotic disorders involve the coagulation cascade or platelet activation rather than primary fibrinolytic defects. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Autosomal Dominant. * **Clinical Presentation:** Most commonly presents as recurrent Deep Vein Thrombosis (DVT) or Pulmonary Embolism (PE) [1]. * **Prothrombin G20210A mutation:** This is the *second* most common inherited thrombotic disorder [1]. * **Screening:** The initial screening test is the **Functional APC Resistance Assay**; confirmation is done via **Genetic testing (PCR)** for the F5 gene. * **Risk Factor:** Heterozygotes have a 5–10 fold increased risk of thrombosis, while homozygotes have an 80-fold increased risk. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 133-134.

Question 1

Which of the following is not a type of peripheral T-cell neoplasm according to the 2017 WHO classification?

Accepted Answer

Lymphoplasmacytic lymphoma

Answer

Mycosis fungoides

Answer

Large granular lymphocytic leukemia

Answer

Anaplastic large cell lymphoma

Question 2

The Fleischer assay is used for which of the following conditions?

Accepted Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Hereditary spherocytosis

Answer

Thalassemia

Answer

All of the above

Question 3

What is the most common inherited thrombotic disorder?

Accepted Answer

Factor V Leiden mutation

Answer

Protein C deficiency

Answer

Protein S deficiency

Answer

tPA deficiency

Question 4

What is the typical TIBC (Total Iron-Binding Capacity) in Anemia of chronic disease compared to Iron deficiency anemia?

Accepted Answer

Decreased in Anemia of chronic disease and increased in Iron deficiency anemia

Answer

Increased in both

Answer

Decreased in both

Answer

Increased in Anemia of chronic disease and decreased in Iron deficiency anemia

Question 5

Hemoglobin F is raised in which of the following conditions?

Accepted Answer

Juvenile chronic myeloid leukemia

Answer

Hereditary spherocytosis

Answer

Congenital red cell aplasia

Answer

Myasthenia gravis

Question 6

Deficiency or inhibition by blocking antibodies of ADAMTS-13 (a metalloprotein) is found in which of the following conditions?

Accepted Answer

Thrombotic Thrombocytopenic Purpura

Answer

Idiopathic Thrombocytopenic Purpura

Answer

Henoch Schonlein Purpura

Answer

Hemophilia

Question 7

An 18-year-old patient's hemogram shows Hb 12 g/dL, RBC count of 6 million/µL, decreased MCV of 56 fL, decreased MCH of 29 pg, and RDW of 14%. What is the most probable diagnosis?

Accepted Answer

Beta-thalassemia trait

Answer

Iron deficiency

Answer

Folate deficiency

Answer

Normal laboratory parameters

Question 8

Hypopigmentation, gray streaks of hair, degranulation defect of neutrophils, and neuropathy are seen in which of the following conditions?

Accepted Answer

Chediak-Higashi syndrome

Answer

Aleukemic Leukemia

Answer

Chronic granulocytic leukemia

Answer

Lazy leukocyte syndrome

Question 9

What is the most common site of lymph node enlargement in Hodgkin's lymphoma?

Accepted Answer

Mediastinal

Answer

Axillary

Answer

Cervical

Answer

Abdominal

Question 10

Which is the most common subtype of acute myeloid leukemia according to the FAB classification?

Accepted Answer

M2

Answer

M3

Answer

M4

Answer

M5

Hematopathology — MCQs

Hematopathology — MCQs

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