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: Which of the following is NOT a component of cryoprecipitate?

Factor V. 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 clotting factors, and remembering its components is high-yield for NEET-PG. [2, 4] **Why Factor V is the correct answer:** Factor V is a **labile clotting factor** found in Fresh Frozen Plasma (FFP) but is **not** concentrated in cryoprecipitate. Cryoprecipitate specifically contains five main components: Factor VIII, Von Willebrand Factor (vWF), Fibrinogen (Factor I), Factor XIII, and Fibronectin. **Analysis of Incorrect Options:** * **Factor I (Fibrinogen):** This is the most abundant component of cryoprecipitate (approx. 150–250 mg per unit). It is the primary reason cryoprecipitate is used in clinical practice (e.g., in DIC or massive hemorrhage). [2] * **Factor VIII:** Cryoprecipitate contains significant amounts of Factor VIII (anti-hemophilic factor), making it a historical treatment for Hemophilia A. * **Von Willebrand Factor (vWF):** Cryoprecipitate is rich in vWF, which is why it is used to treat von Willebrand disease when specific concentrates are unavailable. **High-Yield Clinical Pearls for NEET-PG:** 1. **Indications:** The most common indication for cryoprecipitate today is **hypofibrinogenemia** (target fibrinogen level >100 mg/dL). [2] 2. **Storage:** It is stored at **-18°C or colder** and has a shelf life of 1 year. Once thawed, it must be used within 4–6 hours. 3. **Dosage:** One unit of cryoprecipitate typically raises the fibrinogen level by 5–10 mg/dL in an average adult. 4. **Mnemonic:** To remember the contents, use **"1, 8, 13, vWF, and Fibronectin."** (Note: Factor V is notably absent).

Q: Prothrombin level estimation is useful in the following clotting factor deficiencies, except?

Factor IX. Explanation: The **Prothrombin Time (PT)** test measures the integrity of the **Extrinsic** and **Common pathways** of the coagulation cascade. To understand why Factor IX is the correct answer, one must look at the specific factors involved in these pathways: 1. **Extrinsic Pathway:** Factor VII. 2. **Common Pathway:** Factors X, V, II (Prothrombin), and I (Fibrinogen). **Factor IX** is a component of the **Intrinsic pathway** (along with Factors XII, XI, and VIII). Deficiencies in the intrinsic pathway are monitored using the **Activated Partial Thromboplastin Time (aPTT)**, not PT. Therefore, PT levels remain normal in Factor IX deficiency (Hemophilia B), making it the correct "except" choice. **Analysis of Incorrect Options:** * **Factor II (Prothrombin):** As a key component of the common pathway, its deficiency significantly prolongs PT. * **Factor V:** This is a cofactor in the common pathway (prothrombinase complex); its deficiency leads to an abnormal PT. * **Factor VII:** This is the primary factor of the extrinsic pathway. PT is specifically designed to be highly sensitive to Factor VII levels. **NEET-PG High-Yield Pearls:** * **PT/INR** is the most sensitive marker for **liver synthetic function** because Factor VII has the shortest half-life (approx. 6 hours). * **Vitamin K deficiency** affects Factors II, VII, IX, and X. While both PT and aPTT can be prolonged, **PT is affected first** due to the rapid decline of Factor VII. * **Warfarin** monitoring is done via PT/INR (Extrinsic), while **Heparin** monitoring is done via aPTT (Intrinsic).

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

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 4

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 5

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

Accepted Answer

G6PD deficiency

Answer

Sickle cell anaemia

Answer

Thalassemia

Answer

Hereditary spherocytosis

Question 6

Purpura fulminans is seen in which of the following conditions?

Accepted Answer

Severe DIC

Answer

Leukocytoclastic vasculitis

Answer

Viral hemorrhagic fever

Answer

Tularemia

Question 7

Which of the following is NOT a component of cryoprecipitate?

Accepted Answer

Factor V

Answer

Von Willebrand factor

Answer

Factor I

Answer

Factor VIII

Question 8

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 9

Prothrombin level estimation is useful in the following clotting factor deficiencies, except?

Accepted Answer

Factor IX

Answer

Factor II

Answer

Factor V

Answer

Factor VII

Question 10

Which of the following is NOT typically seen in sickle cell disease?

Accepted Answer

Moderate to massive splenomegaly

Answer

Gamma Gandy bodies

Answer

Fish mouth vertebra

Answer

Decreased ESR

Hematopathology — MCQs

Hematopathology — MCQs

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