Hematopathology Practice Questions

Q: Primary granules in neutrophils contain all except which of the following?

Lactoferrin. **Explanation:** The correct answer is **Lactoferrin**. Neutrophils contain two main types of granules: **Primary (Azurophilic)** and **Secondary (Specific)**. Understanding their distinct compositions is high-yield for NEET-PG. **1. Why Lactoferrin is the correct answer:** Lactoferrin is a major constituent of **Secondary (Specific) granules**, not primary granules. It functions as an iron-binding protein that inhibits bacterial growth by sequestering iron. Other components of secondary granules include Vitamin B12 binding protein, Lysozyme, and Collagenase. **2. Analysis of Incorrect Options (Components of Primary Granules):** Primary granules are essentially modified lysosomes that appear at the promyelocyte stage. They contain: * **Myeloperoxidase (MPO):** The most characteristic enzyme of primary granules, essential for the respiratory burst (H2O2-MPO-Halide system) [1]. * **Defensins:** Cationic proteins that create pores in bacterial membranes [1]. * **Acid Hydrolases:** Enzymes that digest phagocytosed debris [1]. * **Other components:** Neutral proteases (Elastase, Cathepsin G) and Lysozyme (found in both primary and secondary granules) [1]. **Clinical Pearls for NEET-PG:** * **Granule Sequence:** Primary granules appear first (Promyelocyte stage); Secondary granules appear later (Myelocyte stage). * **MPO Stain:** Used in hematopathology to differentiate Acute Myeloid Leukemia (AML) from Acute Lymphoblastic Leukemia (ALL). * **Specific Granule Deficiency:** A rare condition where neutrophils lack secondary granules, leading to bilobed nuclei (pseudo-Pelger-Huët anomaly) and recurrent infections. * **Alkaline Phosphatase (LAP):** Found in tertiary granules/secretory vesicles; LAP score is decreased in Chronic Myeloid Leukemia (CML). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Inflammation and Repair, pp. 91-92.

Q: In Burkitt's lymphoma, which chromosomal translocation is typically seen?

t(8;14). **Explanation:** Burkitt’s Lymphoma is a highly aggressive B-cell non-Hodgkin lymphoma characterized by the overexpression of the **c-MYC proto-oncogene** [1]. The hallmark genetic feature is the translocation of the c-MYC gene from **chromosome 8** to the **Immunoglobulin Heavy chain (IgH) locus** on **chromosome 14** [1]. This **t(8;14)** translocation places c-MYC under the control of the constitutive IgH promoter, leading to continuous cell proliferation [1]. **Analysis of Options:** * **t(8;14):** The most common translocation (approx. 80% of cases) in Burkitt’s Lymphoma [1]. Variants include t(2;8) involving the kappa light chain and t(8;22) involving the lambda light chain [1]. * **t(12;14):** Not a classic translocation for common lymphomas; however, t(11;14) is the hallmark of Mantle Cell Lymphoma (Cyclin D1). * **t(12;18) & t(4;8):** These are not standard diagnostic translocations for major hematological malignancies. Note that t(14;18) is the characteristic translocation for Follicular Lymphoma (BCL-2) [2]. **NEET-PG High-Yield Pearls:** 1. **Morphology:** "Starry-sky appearance" (tingible body macrophages acting as "stars" against a "sky" of dark neoplastic B-cells). 2. **Association:** Strongly linked with **Epstein-Barr Virus (EBV)**, especially the endemic (African) variety involving the jaw. 3. **Immunophenotype:** CD19+, CD20+, CD10+, and BCL-6+. Crucially, it is **BCL-2 negative**. 4. **Proliferation:** Extremely high Ki-67 index (nearly 100%). 5. **Cytology:** Medium-sized cells with multiple "punched-out" cytoplasmic vacuoles. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Neoplasia, pp. 324-325. [2] 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. 602-604.

Q: Classical hemophilia is due to the absence of which of the following?

Large part of factor VIII. **Explanation:** **Classical Hemophilia (Hemophilia A)** is an X-linked recessive bleeding disorder caused by a deficiency or dysfunction of **Clotting Factor VIII** [1]. **Why Option B is Correct:** Factor VIII is a complex molecule consisting of two distinct components [1]: 1. **Factor VIII-C (Procoagulant component):** This is the **large part** of the molecule synthesized primarily in the liver. It is the functional part required for the intrinsic pathway of coagulation. 2. **vWF (von Willebrand Factor):** This is the smaller carrier protein synthesized by endothelial cells and megakaryocytes. In Classical Hemophilia, there is a deficiency of the **large procoagulant component (Factor VIII-C)**, while the vWF levels remain normal [1]. Therefore, the absence of the "large part" of the Factor VIII complex is the hallmark of the disease. **Why Other Options are Incorrect:** * **Option A:** The "small part" refers to von Willebrand Factor (vWF). Its deficiency leads to von Willebrand Disease, not Hemophilia A [1]. * **Options C & D:** Deficiency of Factor IX (Christmas factor) leads to **Hemophilia B** (Christmas Disease), not Classical Hemophilia. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** X-linked recessive (affects males; females are typically asymptomatic carriers) [1]. * **Clinical Presentation:** Characterized by deep tissue bleeding, **hemarthrosis** (bleeding into joints), and delayed post-surgical bleeding. * **Coagulation Profile:** * **Prolonged aPTT** (Intrinsic pathway defect). * **Normal PT, Normal Bleeding Time (BT), and Normal Platelet Count.** * **Mixing Study:** aPTT corrects when patient plasma is mixed with normal plasma (distinguishes deficiency from inhibitors). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 669-671.

Q: Decrease in Alkaline phosphatase is seen in:

CML. **Explanation:** The question refers to the **Leukocyte Alkaline Phosphatase (LAP) score**, also known as the Neutrophil Alkaline Phosphatase (NAP) score. This enzyme is found within the secondary granules of mature neutrophils. **Why CML is the correct answer:** In **Chronic Myeloid Leukemia (CML)**, there is a characteristic **decrease** in the LAP score (often zero or near-zero) [1]. This occurs because the neoplastic neutrophils produced from the malignant clone are biochemically defective and lack the alkaline phosphatase enzyme. A low LAP score is a classic diagnostic hallmark used to differentiate CML from other causes of neutrophilia [1]. **Analysis of Incorrect Options:** * **B. Leukemoid Reaction:** This is an exaggerated white blood cell response to infection or inflammation. Unlike CML, the neutrophils here are mature and functionally normal, leading to a **high** LAP score [1]. * **C. Eosinophilia:** LAP is specifically found in neutrophils, not eosinophils. However, reactive states causing eosinophilia generally do not result in a decreased LAP score in the co-existing neutrophil population. * **D. Malaria:** As an infectious/inflammatory state, malaria typically triggers a stress response that **increases** the LAP score. **High-Yield Clinical Pearls for NEET-PG:** * **LAP Score Calculation:** It is calculated by grading 100 neutrophils from 0 to 4+ based on staining intensity (Normal range: 40–140). * **Decreased LAP Score:** Seen in CML, Paroxysmal Nocturnal Hemoglobinuria (PNH), and Hypophosphatasia. * **Increased LAP Score:** Seen in Leukemoid reaction, Polycythemia Vera (PV), Pregnancy, and Down Syndrome [1]. * **Key Distinction:** CML (Low LAP) vs. Leukemoid Reaction (High LAP) is a frequent "must-know" exam topic [1]. **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. 624-625.

Q: Rosenthal's syndrome is seen in deficiency of which factor?

Factor XI. **Explanation:** **Rosenthal’s syndrome**, also known as **Hemophilia C**, is caused by a deficiency of **Factor XI** (Plasma Thromboplastin Antecedent). Unlike Hemophilia A and B, which are X-linked recessive, Rosenthal’s syndrome is inherited in an **autosomal recessive** pattern and is particularly prevalent in the Ashkenazi Jewish population. **Analysis of Options:** * **Factor XI (Correct):** Deficiency leads to Rosenthal's syndrome. Clinically, it is characterized by mild bleeding tendencies, often only occurring after major trauma or surgery. Unlike other hemophilias, there is a poor correlation between factor levels and bleeding severity, and spontaneous joint bleeds (hemarthrosis) are rare. * **Factor II (Prothrombin):** Deficiency is extremely rare and leads to Hypoprothrombinemia, not Rosenthal's syndrome. * **Factor V:** Deficiency causes **Parahemophilia** (Owren’s disease), characterized by epistaxis, easy bruising, and menorrhagia. * **Factor IX:** Deficiency causes **Hemophilia B** (Christmas disease), which is X-linked recessive and clinically indistinguishable from Hemophilia A [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Hemophilia A (VIII) and B (IX) are **X-linked** [1]; Hemophilia C (XI) is **Autosomal Recessive**. * **Lab Findings:** All hemophilias (A, B, and C) show an **isolated prolonged aPTT** with a normal PT and normal bleeding time. * **Treatment:** Factor XI deficiency is typically managed with Fresh Frozen Plasma (FFP) or Factor XI concentrates, as it is the only hemophilia where FFP is the primary treatment modality due to the lack of cryoprecipitate containing Factor XI. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 622-623.

Question 1

HbA2 levels are increased in all of the following conditions EXCEPT:

Accepted Answer

Alpha Thalassemia

Answer

Beta Thalassemia

Answer

Sickle cell anemia

Answer

Megaloblastic Anemia

Question 2

Primary granules in neutrophils contain all except which of the following?

Accepted Answer

Lactoferrin

Answer

Myeloperoxidase

Answer

Defensin

Answer

Acid hydrolases

Question 3

What is the commonest mode of inheritance of Von Willebrand's disease?

Accepted Answer

Autosomal dominant

Answer

Codominant

Answer

Autosomal recessive

Answer

X-linked recessive

Question 4

Which of the following conditions can cause intravascular hemolysis?

Accepted Answer

Acute G6PD deficiency

Answer

Hereditary spherocytosis

Answer

Thalassemia

Answer

Paroxysmal nocturnal hemoglobinuria (PNH)

Question 5

Which cytogenetic abnormality is associated with a favourable prognosis in Acute Myeloid Leukemia (AML)?

Accepted Answer

t(8;21)

Answer

Deletion 5q

Answer

Preceding Myelodysplastic Syndrome (MDS)

Answer

Age less than 2 years

Question 6

In Burkitt's lymphoma, which chromosomal translocation is typically seen?

Accepted Answer

t(8;14)

Answer

t(12;14)

Answer

t(4;8)

Answer

t(12;18)

Question 7

Classical hemophilia is due to the absence of which of the following?

Accepted Answer

Large part of factor VIII

Answer

Small part of factor VIII

Answer

Small part of factor IX

Answer

Large part of factor IX

Question 8

Decrease in Alkaline phosphatase is seen in:

Accepted Answer

CML

Answer

Leukemoid reaction

Answer

Eosinophilia

Answer

Malaria

Question 9

Rosenthal's syndrome is seen in deficiency of which factor?

Accepted Answer

Factor XI

Answer

Factor II

Answer

Factor V

Answer

Factor IX

Question 10

Coagulation time is prolonged in all conditions except?

Accepted Answer

Idiopathic thrombocytopenic purpura (ITP)

Answer

Hemophilia

Answer

Von Willebrand's disease

Answer

Christmas disease

Hematopathology — MCQs

Hematopathology — MCQs

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