Hematopathology Practice Questions

Q: Cryoprecipitate is rich in which of the following factors?

Factor VIII. **Explanation:** Cryoprecipitate is the cold-insoluble fraction of plasma obtained by thawing fresh frozen plasma (FFP) at 1–6°C. It is a concentrated source of specific coagulation proteins. **Why Factor VIII is correct:** Cryoprecipitate is specifically enriched with five key components: **Factor VIII** (anti-hemophilic factor), **Von Willebrand Factor (vWF)**, **Fibrinogen** (Factor I), **Factor XIII**, and **Fibronectin**. It contains approximately 80–120 units of Factor VIII per bag, making it a traditional treatment for Hemophilia A and Von Willebrand disease (though recombinant factors are now preferred). **Why other options are incorrect:** * **Factor II (Prothrombin), Factor VII, and Factor IX:** These are Vitamin K-dependent factors. They remain in the supernatant (cryo-poor plasma) during the thawing process and are not concentrated in the cryoprecipitate. These factors are best replaced using **Prothrombin Complex Concentrate (PCC)** or **FFP**. * **Factor V:** This is a labile factor found in FFP but is not significantly concentrated in cryoprecipitate. **High-Yield Clinical Pearls for NEET-PG:** * **Primary Indication:** Currently, the most common clinical use of cryoprecipitate is **hypofibrinogenemia** (e.g., in DIC or massive hemorrhage) because it provides a high concentration of Fibrinogen in a small volume. * **Storage:** It is stored at **-18°C or colder** and has a shelf life of 1 year. Once thawed, it must be transfused within 6 hours (or 4 hours if pooled). * **Dosage:** One unit of cryoprecipitate typically raises the fibrinogen level by 5–10 mg/dL in an average adult. * **Mnemonic:** Remember **"8-1-13-vWF"** (Factors 8, 1, 13, and vWF).

Q: ADAMTS13 deficiency is associated with which disease?

Thrombotic Thrombocytopenic Purpura (TTP). **Explanation:** **Thrombotic Thrombocytopenic Purpura (TTP)** is caused by a deficiency of **ADAMTS13**, a metalloproteinase responsible for cleaving large von Willebrand Factor (vWF) multimers [1]. When ADAMTS13 is deficient (due to autoantibodies or genetic mutations), "ultra-large" vWF multimers persist in the circulation [1]. These multimers cause spontaneous platelet aggregation and microthrombi formation, leading to microangiopathic hemolytic anemia (MAHA) and consumptive thrombocytopenia. **Analysis of Incorrect Options:** * **Immune Thrombocytopenic Purpura (ITP):** Caused by anti-platelet antibodies (usually against GpIIb/IIIa) leading to splenic destruction of platelets. ADAMTS13 levels are normal. * **Hemolytic Uremic Syndrome (HUS):** While clinically similar to TTP, classic HUS is caused by **Shiga toxin** (from *E. coli* O157:H7), which damages endothelial cells [1]. ADAMTS13 levels are typically normal [1]. * **Paroxysmal Nocturnal Hemoglobinuria (PNH):** A stem cell disorder caused by a mutation in the **PIGA gene**, leading to a deficiency of GPI-anchored proteins (CD55/CD59) and complement-mediated hemolysis. **High-Yield Clinical Pearls for NEET-PG:** * **The TTP Pentad:** Fever, Anemia (MAHA), Thrombocytopenia, Neurological symptoms, and Renal failure (Mnemonic: **FAT RN**) [1]. * **Diagnosis:** Schistocytes (fragmented RBCs) on peripheral smear and reduced ADAMTS13 activity (<10%). * **Treatment:** **Plasmapheresis (Plasma Exchange)** is the gold standard as it removes antibodies and replenishes ADAMTS13. * **Note:** PT and APTT are usually **normal** in TTP/HUS, distinguishing them from DIC. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 947-948.

Question 1

Cold haemagglutinin is associated with which type of antibody?

Accepted Answer

Donath-Landsteiner antibody

Answer

Anti-IgM

Answer

Anti-IgG

Answer

Anti-IgA

Question 2

What is the normal platelet count?

Accepted Answer

Shaken baby syndrome

Answer

D1C

Answer

Microangiopathic hemolytic anaemia

Answer

Splenomegaly

Question 3

Cryoprecipitate is rich in which of the following factors?

Accepted Answer

Factor VIII

Answer

Factor II

Answer

Factor V

Answer

Factor VII

Question 4

All of the following are true of beta-thalassemia major, except?

Accepted Answer

Increased osmotic fragility

Answer

Splenomegaly

Answer

Target cells on peripheral smear

Answer

Microcytic hypochromic anemia

Question 5

Spherocytosis in a peripheral blood smear is seen in which of the following conditions?

Accepted Answer

All of the above

Answer

Post-transfusion reactions

Answer

Hereditary spherocytosis

Answer

Hemolytic disease of the newborn

Question 6

Necrotizing ragged ulceration with no apparent inflammatory response is indicative of which of the following conditions?

Accepted Answer

Agranulocytosis

Answer

Leukocytosis

Answer

Polycythemia vera

Answer

Sickle cell anemia

Question 7

ADAMTS13 deficiency is associated with which disease?

Accepted Answer

Thrombotic Thrombocytopenic Purpura (TTP)

Answer

Immune Thrombocytopenic Purpura (ITP)

Answer

Hemolytic Uremic Syndrome (HUS)

Answer

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Question 8

Which of the following is NOT included in the myeloproliferative neoplasms?

Accepted Answer

Erythroleukemia

Answer

Angiogenic myeloid metaplasia

Answer

Thrombocythemia

Answer

Megaloblastic hyperplasia

Question 9

A 17-year-old woman's urine becomes red after being given sulfonamides for a urinary tract infection. Both urine and serum test positive for free hemoglobin, and the urine red cell count is 1.2 million/mm3. A peripheral blood smear shows normocytic and normochromic red cells and a few "bite cells." Deficiency of which of the following substances is most likely responsible for these symptoms?

Accepted Answer

Glucose-6-phosphate dehydrogenase

Answer

Alpha-chain of hemoglobin

Answer

Beta-chain of hemoglobin

Answer

Glycoprotein IIb/IIIa

Question 10

The autohemolysis test is positive in which of the following conditions?

Accepted Answer

Hereditary spherocytosis

Answer

Beta thalassemia

Answer

Vitamin E deficiency

Answer

Sickle cell disease

Hematopathology — MCQs

Hematopathology — MCQs

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