Hematopathology — MCQs

Hematopathology Indian Medical PG Practice Questions and MCQs

Question 851: Which of the following is not a type of peripheral T-cell neoplasm according to the 2017 WHO classification?

A. Lymphoplasmacytic lymphoma (Correct Answer)
B. Mycosis fungoides
C. Large granular lymphocytic leukemia
D. Anaplastic large cell lymphoma

Explanation: **Explanation** The 2017 WHO classification of lymphoid neoplasms categorizes tumors based on their cell of origin (B-cell, T-cell, or NK-cell) and their stage of differentiation (precursor vs. mature/peripheral) [1]. **Why Lymphoplasmacytic Lymphoma (LPL) is the correct answer:** LPL is a **mature B-cell neoplasm**, not a T-cell neoplasm. It is characterized by the proliferation of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells. It is most famously associated with **Waldenström Macroglobulinemia**, where the tumor cells secrete a monoclonal IgM paraprotein, leading to hyperviscosity syndrome. **Analysis of incorrect options (Peripheral T-cell Neoplasms):** * **Mycosis Fungoides:** This is the most common type of **Cutaneous T-cell Lymphoma (CTCL)** [4]. It is a mature (peripheral) T-cell neoplasm characterized by skin patches, plaques, and Pautrier microabscesses [2], [4]. * **Large Granular Lymphocytic (LGL) Leukemia:** This is a mature T/NK-cell neoplasm. It often presents with neutropenia and rheumatoid arthritis (similar to Felty syndrome). * **Anaplastic Large Cell Lymphoma (ALCL):** This is an aggressive peripheral T-cell lymphoma characterized by "hallmark cells" (horseshoe-shaped nuclei) and is often **ALK-positive** (t(2;5) translocation) [3], [5]. **High-Yield NEET-PG Pearls:** * **LPL/Waldenström:** Look for **IgM spikes**, hyperviscosity (visual disturbances, neurological symptoms), and absence of lytic bone lesions (unlike Multiple Myeloma). * **T-cell Markers:** Most peripheral T-cell neoplasms express CD2, CD3, CD5, and CD7. * **Sezary Syndrome:** The leukemic phase of Mycosis Fungoides, characterized by erythroderma and cerebriform nuclei in peripheral blood [2]. **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. 596-598. [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. 613-614. [3] 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. 612-613. [4] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 564-565. [5] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 565-566.

Question 852: The Fleischer assay is used for which of the following conditions?

A. Hereditary spherocytosis
B. Thalassemia
C. Paroxysmal nocturnal hemoglobinuria (Correct Answer)
D. All of the above

Explanation: **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.

Question 853: What is the most common inherited thrombotic disorder?

A. Protein C deficiency
B. Protein S deficiency
C. Factor V Leiden mutation (Correct Answer)
D. tPA deficiency

Explanation: **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 854: What is the typical TIBC (Total Iron-Binding Capacity) in Anemia of chronic disease compared to Iron deficiency anemia?

A. Increased in both
B. Decreased in both
C. Increased in Anemia of chronic disease and decreased in Iron deficiency anemia
D. Decreased in Anemia of chronic disease and increased in Iron deficiency anemia (Correct Answer)

Explanation: **Explanation:** The differentiation between **Iron Deficiency Anemia (IDA)** and **Anemia of Chronic Disease (ACD)** is a high-yield NEET-PG topic. The key lies in how the body regulates iron transport protein (Transferrin) in response to iron stores. 1. **Why Option D is correct:** * **Iron Deficiency Anemia (IDA):** When systemic iron stores are depleted, the liver compensates by increasing the production of **Transferrin** (measured as TIBC) to maximize the capture of any available iron. Thus, **TIBC is increased.** [1] * **Anemia of Chronic Disease (ACD):** This is driven by inflammation and the hormone **Hepcidin**. Hepcidin sequesters iron inside macrophages and hepatocytes. [1] Because the body "perceived" it has enough stored iron (even if it's trapped), the liver reduces Transferrin production. Therefore, **TIBC is decreased.** 2. **Why other options are incorrect:** * **Option A & B:** These are incorrect because IDA and ACD have opposing physiological responses regarding iron transport capacity. * **Option C:** This is the exact opposite of the physiological reality. High TIBC is a hallmark of "hunger" for iron (IDA), not "sequestration" of iron (ACD). **High-Yield Clinical Pearls for NEET-PG:** * **Serum Ferritin:** This is the most sensitive marker. It is **decreased in IDA** (low stores) but **increased or normal in ACD** (it acts as an acute-phase reactant). * **Transferrin Saturation:** Decreased in both, but usually much lower (<15%) in IDA. * **Soluble Transferrin Receptor (sTfR):** Increased in IDA, but **normal in ACD**. This is the best test to distinguish the two when they coexist. * **Hepcidin:** The "Master Regulator." It is high in ACD (blocking ferroportin) and low in IDA. [1] **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 658-662.

Question 855: Hemoglobin F is raised in which of the following conditions?

A. Juvenile chronic myeloid leukemia (Correct Answer)
B. Hereditary spherocytosis
C. Congenital red cell aplasia
D. Myasthenia gravis

Explanation: **Explanation:** **Correct Answer: A. Juvenile chronic myeloid leukemia (JMML)** Juvenile Myelomonocytic Leukemia (formerly known as Juvenile CML) is a rare clonal hematopoietic stem cell disorder of childhood. A hallmark diagnostic feature of JMML is a **significantly elevated Hemoglobin F (HbF)** level for the patient's age (often >10-15%). This occurs because the malignant clone reverts to a "fetal-like" erythropoiesis pattern. Unlike adult CML, JMML is Philadelphia chromosome (Ph) negative and often associated with mutations in the RAS pathway or NF1. **Analysis of Incorrect Options:** * **B. Hereditary Spherocytosis:** This is a red cell membrane defect (e.g., ankyrin or spectrin deficiency) leading to extravascular hemolysis. HbF levels remain normal as there is no reversion to fetal erythropoiesis. * **C. Congenital Red Cell Aplasia (Diamond-Blackfan Anemia):** While HbF can be elevated in DBA due to "stress erythropoiesis," it is not the classic diagnostic association compared to the profound elevation seen in JMML. However, in the context of this specific question, JMML is the more characteristic "textbook" association for raised HbF in pediatric hematologic malignancies. * **D. Myasthenia Gravis:** This is an autoimmune neuromuscular junction disorder caused by antibodies against acetylcholine receptors. It has no primary association with hemoglobin synthesis or erythropoiesis. **NEET-PG High-Yield Pearls:** * **HbF Structure:** $\alpha_2\gamma_2$. * **Other conditions with raised HbF:** $\beta$-Thalassemia major (highest levels) [1], Sickle cell anemia, Hereditary Persistence of Fetal Hemoglobin (HPFH), and Aplastic anemia (during recovery). * **JMML Triad:** Splenomegaly, absence of Philadelphia chromosome, and elevated HbF. * **Drug increasing HbF:** Hydroxyurea (used in Sickle Cell Disease to prevent crises). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, p. 648.

Question 856: Deficiency or inhibition by blocking antibodies of ADAMTS-13 (a metalloprotein) is found in which of the following conditions?

A. Idiopathic Thrombocytopenic Purpura
B. Thrombotic Thrombocytopenic Purpura (Correct Answer)
C. Henoch Schonlein Purpura
D. Hemophilia

Explanation: ### Explanation **Correct Answer: B. Thrombotic Thrombocytopenic Purpura (TTP)** The pathophysiology of TTP centers on the deficiency of **ADAMTS-13**, a plasma metalloprotease responsible for cleaving large **von Willebrand Factor (vWF) multimers** into smaller, less prothrombotic fragments. [1] * **Mechanism:** When ADAMTS-13 is deficient (due to genetic mutations) or inhibited (by acquired autoantibodies), "ultra-large" vWF multimers persist in the circulation. [1] These multimers cause spontaneous platelet aggregation and microthrombi formation in small vessels. * **Consequence:** This leads to microangiopathic hemolytic anemia (MAHA) and consumptive thrombocytopenia. [1] **Why other options are incorrect:** * **A. Idiopathic Thrombocytopenic Purpura (ITP):** Caused by anti-platelet antibodies (usually against GpIIb/IIIa) leading to splenic destruction of platelets. ADAMTS-13 levels are normal. * **C. Henoch-Schonlein Purpura (HSP):** An IgA-mediated small-vessel vasculitis. It is characterized by the deposition of IgA immune complexes in vessel walls, not a metalloproteinase deficiency. * **D. Hemophilia:** A coagulation factor deficiency (Factor VIII in Hemophilia A; Factor IX in Hemophilia B) leading to secondary hemostasis defects, not a platelet-vWF interaction issue. **High-Yield Clinical Pearls for NEET-PG:** * **The Classic Pentad of TTP:** (Mnemonic: **FAT RN**) [1] 1. **F**ever 2. **A**nemia (MAHA with Schistocytes) 3. **T**hrombocytopenia 4. **R**enal failure 5. **N**eurological symptoms * **Diagnosis:** Decreased ADAMTS-13 activity (<10%) is diagnostic. * **Treatment:** **Plasmapheresis (Plasma Exchange)** is the gold standard as it removes the autoantibodies and replenishes ADAMTS-13. * **Distinction:** Unlike DIC, PT and aPTT are usually **normal** in TTP. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Kidney, pp. 947-948.

Question 857: 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?

A. Iron deficiency
B. Folate deficiency
C. Beta-thalassemia trait (Correct Answer)
D. Normal laboratory parameters

Explanation: The correct answer is **Beta-thalassemia trait (BTT)**. The key to solving this question lies in the **Mentzer Index** and the discrepancy between the RBC count and the MCV. 1. **Why Beta-thalassemia trait is correct:** In BTT, there is a defect in globin chain synthesis, leading to microcytic hypochromic anemia [1]. However, the bone marrow compensates by producing a **high number of red cells** [2]. * **Mentzer Index (MCV/RBC count):** In this case, $56 / 6 = 9.3$. A Mentzer index **< 13** strongly suggests Thalassemia, while > 13 suggests Iron Deficiency Anemia (IDA). * **RDW (Red Cell Distribution Width):** In BTT, the RDW is typically **normal** (as seen here at 14%), reflecting a uniform population of small cells. 2. **Why other options are incorrect:** * **Iron deficiency:** While it also presents with low MCV and MCH, the **RBC count is typically low or normal**, and the **RDW is characteristically high** (>15%) due to anisocytosis [2]. * **Folate deficiency:** This is a megaloblastic anemia characterized by an **increased MCV** (>100 fL) and macrocytes on peripheral smear, which contradicts the microcytosis (56 fL) seen here. * **Normal laboratory parameters:** The MCV (normal 80–100 fL) and MCH (normal 27–32 pg) are significantly low, indicating a pathological state. **High-Yield Clinical Pearls for NEET-PG:** * **Thalassemia triad:** Low MCV + High RBC count + Normal RDW. * **Confirmatory test for BTT:** HbA2 estimation via HPLC (>3.5% is diagnostic). * **Target cells:** These are the classic peripheral smear finding in Thalassemia. * **NEET-PG Tip:** If you see a very low MCV (in the 50s) with a near-normal Hemoglobin, always think of Thalassemia trait before Iron Deficiency. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 587-588. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591.

Question 858: Hypopigmentation, gray streaks of hair, degranulation defect of neutrophils, and neuropathy are seen in which of the following conditions?

A. Aleukemic Leukemia
B. Chronic granulocytic leukemia
C. Lazy leukocyte syndrome
D. Chediak-Higashi syndrome (Correct Answer)

Explanation: **Explanation:** The correct answer is **Chediak-Higashi syndrome (CHS)**. This is a rare autosomal recessive disorder caused by a mutation in the **LYST (Lysosomal Trafficking Regulator) gene**. This mutation leads to a defect in protein trafficking, resulting in the formation of **giant lysosomal granules** that cannot be secreted or fused properly [1]. 1. **Why it is correct:** * **Degranulation defect:** Neutrophils contain massive, non-functional granules [1]. While they can ingest bacteria, they cannot release enzymes to kill them, leading to recurrent pyogenic infections. * **Hypopigmentation/Gray hair:** Melanocytes cannot distribute melanin-containing melanosomes to keratinocytes, resulting in partial oculocutaneous albinism and silvery-gray hair [1]. * **Neuropathy:** Defective axonal transport due to microtubule dysfunction leads to progressive peripheral neuropathy [1]. 2. **Why incorrect options are wrong:** * **Aleukemic Leukemia:** Refers to leukemia where abnormal cells are present in the bone marrow but absent in the peripheral blood; it does not present with albinism or specific degranulation defects. * **Chronic Granulocytic Leukemia (CML):** Characterized by the Philadelphia chromosome and massive splenomegaly, not pigmentary changes or giant granules. * **Lazy Leukocyte Syndrome:** A defect specifically in neutrophil chemotaxis and motility, but it lacks the systemic features of albinism and giant granules. **High-Yield Clinical Pearls for NEET-PG:** * **Peripheral Smear:** Look for pathognomonic **giant azurophilic granules** in neutrophils and platelets [1]. * **Associated Feature:** Patients often develop an "Accelerated Phase" (Hemophagocytic Lymphohistiocytosis - HLH), characterized by pancytopenia and hepatosplenomegaly. * **Key Triad:** Albinism + Recurrent Infections + Giant Granules. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 245-246.

Question 859: What is the most common site of lymph node enlargement in Hodgkin's lymphoma?

A. Mediastinal (Correct Answer)
B. Axillary
C. Cervical
D. Abdominal

Explanation: **Explanation:** **Hodgkin’s Lymphoma (HL)** typically presents as a painless, firm, and rubbery enlargement of lymph nodes. The disease characteristically spreads in a predictable, contiguous fashion along anatomical lymphatic chains [1]. **Why Mediastinal is the correct answer:** While cervical lymphadenopathy is a very frequent clinical finding, **mediastinal involvement** is the most common site of presentation in Hodgkin’s Lymphoma, particularly in the **Nodular Sclerosis** subtype (the most common variant) [1]. Approximately 60-80% of patients with Nodular Sclerosis HL present with a mediastinal mass, often discovered on routine chest X-ray [2]. In the context of competitive exams like NEET-PG, when asked for the "most common site," mediastinal involvement is prioritized due to its high diagnostic association with the disease's most prevalent subtype. **Analysis of Incorrect Options:** * **B. Axillary:** While axillary nodes can be involved, they are rarely the primary or most common site of initial presentation compared to the neck or chest. * **C. Cervical:** This is the most common *peripheral* site of palpable lymphadenopathy. However, statistically, mediastinal involvement (often asymptomatic initially) occurs with higher frequency across the dominant subtypes [2]. * **D. Abdominal:** Retroperitoneal or mesenteric involvement is more characteristic of Non-Hodgkin Lymphoma (NHL). HL rarely skips to the abdomen without involving the mediastinum or cervical regions first. **High-Yield Clinical Pearls for NEET-PG:** * **Bimodal Age Distribution:** Peaks at 15–35 years and again after 50 years. * **Reed-Sternberg (RS) Cells:** The diagnostic hallmark (e.g., "Owl’s eye" appearance). * **Nodular Sclerosis:** Most common subtype; characterized by lacunar cells and collagen bands; strong predilection for young females and the mediastinum [1]. * **Alcohol-induced pain:** A rare but classic symptom where lymph nodes become painful after alcohol consumption [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Diseases Of The Urinary And Male Genital Tracts, pp. 557-559. [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. 616-618.

Question 860: Which is the most common subtype of acute myeloid leukemia according to the FAB classification?

A. M2 (Correct Answer)
B. M3
C. M4
D. M5

Explanation: **Explanation:** The French-American-British (FAB) classification categorizes Acute Myeloid Leukemia (AML) into subtypes M0 through M7 based on morphology and cytochemistry. **Why M2 is correct:** **AML-M2 (Acute Myeloblastic Leukemia with maturation)** is statistically the most common subtype, accounting for approximately **25–30%** of all AML cases [1]. It is characterized by the presence of >20% blasts in the bone marrow with significant maturation (i.e., >10% of cells are promyelocytes, myelocytes, or mature neutrophils). It is frequently associated with the **t(8;21)** translocation, which generally carries a favorable prognosis [1]. **Why other options are incorrect:** * **M3 (Acute Promyelocytic Leukemia):** Characterized by t(15;17) and high risk of DIC [2]. While clinically distinct and high-yield, it accounts for only about 10–15% of cases. * **M4 (Acute Myelomonocytic Leukemia):** The second most common subtype (approx. 20%). It involves both myeloid and monocytic lineages. * **M5 (Acute Monocytic Leukemia):** Accounts for about 10% of cases [2]. It is classically associated with **gum hypertrophy** and tissue infiltration. **High-Yield Clinical Pearls for NEET-PG:** * **Most common subtype overall:** M2. * **Subtype with best prognosis:** M3 (due to targeted therapy with ATRA) and M2 with t(8;21) [1]. * **Subtype associated with DIC:** M3 (APL) [1]. * **Subtype associated with Gum Hypertrophy:** M4 and M5. * **Auer Rods:** Most commonly seen in M2 and M3; they are never seen in lymphoblasts (ALL) [1]. * **WHO Classification:** Note that the modern WHO classification has largely superseded FAB by incorporating cytogenetics, but FAB remains a favorite for "most common" type questions in exams [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, p. 620. [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. 621-622.

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Thrombotic Disorders

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