Hematology — MCQs

Hematology Indian Medical PG Practice Questions and MCQs

Question 611: All of the following are causes of pancytopenia with cellular bone marrow except?

A. Paroxysmal nocturnal hemoglobinuria
B. Dyskeratosis congenita (Correct Answer)
C. Megaloblastic anemia
D. Hairy cell leukemia

Explanation: ### Explanation The core concept in this question is distinguishing between **hypocellular** (empty) bone marrow and **cellular** (hypercellular or normocellular) bone marrow in the setting of pancytopenia. A trephine biopsy is superior for assessing marrow cellularity and infiltration by abnormal cells [2]. **Why Dyskeratosis Congenita is the correct answer:** Dyskeratosis congenita is a congenital form of **Aplastic Anemia** caused by telomere maintenance defects. By definition, aplastic anemia presents with **pancytopenia and a hypocellular bone marrow** (fatty replacement). Since the question asks for causes of pancytopenia with *cellular* marrow, Dyskeratosis congenita is the "except" because its marrow is characteristically empty. **Analysis of Incorrect Options (Causes of Cellular Marrow):** * **Megaloblastic Anemia:** Due to Vitamin B12 or Folate deficiency, there is "ineffective hematopoiesis." The marrow is **hypercellular** [1] with megaloblasts, but the cells die before entering circulation, leading to peripheral pancytopenia. * **Paroxysmal Nocturnal Hemoglobinuria (PNH):** While PNH can be associated with aplastic anemia, it often presents with a cellular marrow during hemolytic phases. It is a classic cause of pancytopenia where the marrow remains active. * **Hairy Cell Leukemia:** This is a lymphoproliferative disorder where the bone marrow is **hypercellular** (infiltrated by leukemic cells), but the peripheral blood shows pancytopenia due to splenic sequestration and marrow replacement. **NEET-PG High-Yield Pearls:** 1. **Pancytopenia with Hypocellular Marrow:** Aplastic anemia (Acquired/Idiopathic), Fanconi anemia, Dyskeratosis congenita. 2. **Pancytopenia with Cellular Marrow:** Megaloblastic anemia [1], Myelodysplastic Syndrome (MDS), Aleukemic leukemia, Hypersplenism, and PNH. 3. **Dyskeratosis Congenita Triad:** Abnormal skin pigmentation, nail dystrophy, and oral leukoplakia. 4. **Hairy Cell Leukemia:** Characterized by "Dry Tap" on marrow aspiration and TRAP (Tartrate-Resistant Acid Phosphatase) positivity.

Question 612: A 67-year-old elderly male presents with headache, recurrent infections, and multiple punched-out lytic lesions on X-ray skull and lumbago for the past 1 month. Which investigation will be most helpful in establishing a diagnosis?

A. Protein electrophoresis (Correct Answer)
B. Serum calcium
C. Alkaline phosphatase levels
D. PSA levels

Explanation: ### Explanation The clinical presentation of an elderly male with **recurrent infections**, **punched-out lytic lesions** on the skull, and **lumbago** (lower back pain) is a classic triad for **Multiple Myeloma (MM)**. [1] #### Why Protein Electrophoresis is Correct: Multiple Myeloma is a plasma cell dyscrasia characterized by the monoclonal proliferation of malignant plasma cells in the bone marrow. These cells produce excessive amounts of a single type of immunoglobulin (M-protein). **Serum Protein Electrophoresis (SPEP)** is the primary screening tool to identify this "M-spike" (usually in the gamma or beta region), which is essential for establishing the diagnosis. [1] #### Why Other Options are Incorrect: * **Serum Calcium:** While hypercalcemia is a common feature of MM (part of the **CRAB** criteria), it is non-specific and occurs in many other malignancies and metabolic disorders. It does not confirm the diagnosis. * **Alkaline Phosphatase (ALP):** In MM, lytic lesions are caused by osteoclast activation without osteoblastic activity. Therefore, **ALP levels are typically normal**, unlike in bone metastases from prostate or breast cancer where ALP is elevated. * **PSA Levels:** While prostate cancer can cause back pain and bone lesions in elderly males, it typically produces **osteoblastic (sclerotic)** lesions, not the punched-out lytic lesions described here. #### High-Yield Clinical Pearls for NEET-PG: * **CRAB Criteria for MM:** **C**alcium elevation, **R**enal insufficiency, **A**nemia, **B**one lesions. * **Bence-Jones Proteins:** These are free light chains found in urine; they precipitate at 40–60°C and redissolve at 100°C. * **Peripheral Smear:** Look for **Rouleaux formation** due to increased globulins. * **Bone Marrow:** Definitive diagnosis requires **>10% clonal plasma cells**. [1] * **Radiology:** Always perform a **Skeletal Survey** (X-rays); a Bone Scan is often falsely negative because it detects osteoblastic activity, which is absent in MM. [1]

Question 613: All of the following are causes of aplastic anemia except?

A. Paroxysmal nocturnal hemoglobinuria
B. Hepatitis
C. Pregnancy
D. Cold hemoglobinuria (Correct Answer)

Explanation: **Explanation:** Aplastic anemia is a bone marrow failure syndrome characterized by pancytopenia and a hypocellular marrow. The underlying mechanism is typically immune-mediated destruction of hematopoietic stem cells. **Why Cold Hemoglobinuria is the Correct Answer:** **Cold Hemoglobinuria** (specifically Paroxysmal Cold Hemoglobinuria or PCH) is a type of **autoimmune hemolytic anemia** caused by the Donath-Landsteiner antibody [2]. It involves the destruction of mature red blood cells in the peripheral circulation, not the failure of production in the bone marrow [1]. Therefore, it does not cause aplastic anemia. **Analysis of Incorrect Options:** * **Paroxysmal Nocturnal Hemoglobinuria (PNH):** There is a strong pathophysiological link between PNH and aplastic anemia. Many patients with aplastic anemia have a small clone of PNH cells, and aplastic anemia can evolve into PNH (and vice versa) [3]. * **Hepatitis:** Post-hepatitic aplastic anemia is a well-recognized entity. It typically occurs 2–3 months after an episode of acute hepatitis (usually non-A, non-B, non-C, non-G viruses). It is often severe and mediated by T-cell activation. * **Pregnancy:** Though rare, pregnancy is a documented association with aplastic anemia. It may be related to hormonal changes or immune alterations and sometimes resolves spontaneously after delivery. **Clinical Pearls for NEET-PG:** * **Most common cause:** Idiopathic (up to 70% of cases). * **Drugs:** Chloramphenicol, sulfonamides, and gold salts are classic triggers. * **Fanconi Anemia:** The most common inherited cause of aplastic anemia (look for short stature, thumb anomalies, and café-au-lait spots). * **Diagnosis:** Bone marrow biopsy is essential to show "fatty replacement" of marrow spaces.

Question 614: Spur cell anemia is seen in which of the following conditions?

A. Drug-induced anemia
B. Hepatocellular disease (Correct Answer)
C. Renal disease
D. Alcoholism

Explanation: **Explanation:** **Spur Cell Anemia** is a severe form of hemolytic anemia characterized by the presence of **Acanthocytes** (spur cells) on the peripheral blood smear. **1. Why Hepatocellular Disease is Correct:** The pathophysiology lies in abnormal lipid metabolism. In advanced liver disease (especially cirrhosis), there is an increase in free cholesterol in the plasma. This cholesterol deposits into the erythrocyte membrane, increasing the surface area-to-volume ratio. These cholesterol-rich cells are then remodeled by the spleen, which shears off portions of the membrane, resulting in the characteristic thorny, irregular projections known as **spurs** [2]. These rigid cells are eventually trapped and destroyed in the splenic sinusoids, leading to extravascular hemolysis [1]. **2. Analysis of Incorrect Options:** * **Drug-induced anemia:** Typically presents as immune-mediated hemolysis (Spherocytes) or oxidative stress-induced hemolysis (Heinz bodies/Bite cells, e.g., G6PD deficiency), not acanthocytosis [3]. * **Renal disease:** Characteristically associated with **Burr cells (Echinocytes)**. Unlike spur cells, Burr cells have small, uniform, symmetric projections and are seen in uremia. * **Alcoholism:** While chronic alcohol use can lead to liver disease, "Spur Cell Anemia" is specifically a complication of the resulting end-stage **hepatocellular disease** rather than a direct effect of alcohol itself (which more commonly causes macrocytosis or sideroblastic changes). **Clinical Pearls for NEET-PG:** * **Acanthocytes (Spur Cells):** Irregular projections. Seen in **Liver disease** and **Abetalipoproteinemia**. * **Echinocytes (Burr Cells):** Regular, uniform projections. Seen in **Uremia**, hypophosphatemia, and as a storage artifact. * **Target Cells (Codocytes):** Also seen in liver disease due to increased membrane cholesterol, but they are not the defining feature of "Spur Cell Anemia." * **Prognosis:** The development of spur cell anemia in a cirrhotic patient signifies a very poor prognosis, often indicating the need for a liver transplant.

Question 615: Haemophilia B is due to a mutation in the gene corresponding to which coagulation factor?

A. Factor 8
B. Factor 9 (Correct Answer)
C. Factor 7
D. Factor 11

Explanation: **Explanation:** Hemophilia is a group of hereditary genetic disorders that impair the body's ability to control blood clotting. **Hemophilia B**, also known as **Christmas Disease**, is caused by a deficiency or mutation in the gene encoding **Coagulation Factor IX (9)**. It is inherited as an **X-linked recessive** trait, primarily affecting males [1]. **Analysis of Options:** * **Factor 9 (Correct):** Deficiency leads to Hemophilia B [1]. Factor IX is a serine protease that, when activated (IXa), forms a complex with Factor VIIIa to activate Factor X in the intrinsic pathway. * **Factor 8 (Incorrect):** Deficiency of Factor VIII causes **Hemophilia A** (Classic Hemophilia) [1]. It is the most common type of hemophilia (80-85% of cases). * **Factor 11 (Incorrect):** Deficiency leads to **Hemophilia C** (Rosenthal Syndrome). Unlike A and B, it is autosomal recessive and often seen in Ashkenazi Jews. * **Factor 7 (Incorrect):** Deficiency causes a rare autosomal recessive bleeding disorder. Factor VII is part of the extrinsic pathway (measured by PT/INR). **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** Both Hemophilia A and B are **X-linked recessive** [1]. * **Lab Findings:** Characterized by a **prolonged aPTT** with a **normal PT** and **normal bleeding time** (platelet function is unaffected). * **Clinical Presentation:** Hallmark is **Hemarthrosis** (bleeding into joints, most commonly the knee) and deep muscle hematomas. * **Treatment:** Factor replacement therapy is the mainstay. For Hemophilia B, recombinant Factor IX is used. Unlike Hemophilia A, Desmopressin (dDAVP) is **not** effective for Hemophilia B.

Question 616: A 25-year-old patient presents with fatigue and abdominal pain. Examination reveals jaundice and splenomegaly. Ultrasound shows gallstones. What is the most likely diagnosis?

A. Sickle cell anemia
B. Acute pancreatitis
C. Hereditary spherocytosis (Correct Answer)
D. Cholangitis

Explanation: ### Explanation **Correct Answer: C. Hereditary spherocytosis** The clinical triad of **anemia (fatigue), jaundice, and splenomegaly** in a young patient, coupled with the presence of **gallstones**, is a classic presentation of chronic extravascular hemolysis. In **Hereditary Spherocytosis (HS)**, a defect in red blood cell (RBC) membrane proteins (most commonly **Ankyrin**, followed by Spectrin) leads to the formation of spherical, fragile RBCs. These spherocytes are trapped and destroyed in the splenic sinusoids, leading to splenomegaly. The chronic breakdown of RBCs results in unconjugated hyperbilirubinemia, which predisposes the patient to **pigment (calcium bilirubinate) gallstones**. **Why other options are incorrect:** * **A. Sickle cell anemia:** While it causes hemolysis and gallstones, the spleen in an adult sickle cell patient is usually small and fibrotic due to repeated infarctions (**autosplenectomy**), making splenomegaly unlikely at age 25. * **B. Acute pancreatitis:** Presents with severe epigastric pain radiating to the back and elevated amylase/lipase; it does not typically cause chronic jaundice or splenomegaly. * **D. Cholangitis:** Presents with Charcot’s Triad (fever, jaundice, RUQ pain). It is an acute bacterial infection of the biliary tree, not a chronic hemolytic process. **High-Yield NEET-PG Pearls:** * **Gold Standard Test:** Eosin-5-maleimide (EMA) binding test via flow cytometry. * **Screening Test:** Osmotic Fragility Test (increased fragility). * **Peripheral Smear:** Spherocytes (small, dark RBCs lacking central pallor) and reticulocytosis. * **MCHC:** Characteristically **increased** (the only anemia with high MCHC). * **Treatment of Choice:** Splenectomy (indicated in moderate to severe cases, usually after age 5 to reduce sepsis risk).

Question 617: Which of the following is a poor prognostic factor in Acute Lymphoblastic Leukemia (ALL)?

A. Hyperdiploidy
B. t (9;22); t (4; 11) (Correct Answer)
C. 2-8 years of age
D. WBC count < 50,000

Explanation: In Acute Lymphoblastic Leukemia (ALL), prognosis is determined by age, initial white blood cell (WBC) count, and specific cytogenetic abnormalities [1]. ### **Explanation of the Correct Answer** **Option B: t(9;22) and t(4;11)** are well-established **poor prognostic factors** [1]. * **t(9;22):** Known as the Philadelphia chromosome ($Ph+$), it creates the *BCR-ABL1* fusion gene [1]. It is more common in adults and is associated with high resistance to standard chemotherapy and a high relapse rate. * **t(4;11):** Involves the *KMT2A* (MLL) gene rearrangement. It is typically seen in infant ALL and is associated with very high WBC counts and CNS involvement, signifying an aggressive disease course [1]. ### **Analysis of Incorrect Options** * **A. Hyperdiploidy:** Defined as >50 chromosomes per cell, this is a **favorable** prognostic factor. These cells are highly sensitive to methotrexate and apoptosis. * **C. 2–8 years of age:** This is the "golden age" for ALL prognosis. Patients between **1 and 10 years** have the best outcomes. Age <1 year (infants) or >10 years (adolescents/adults) indicates a poorer prognosis. * **D. WBC count < 50,000:** A lower tumor burden at presentation is a **favorable** sign. A WBC count >50,000 in B-ALL (or >100,000 in T-ALL) is considered a high-risk feature [1]. ### **High-Yield Clinical Pearls for NEET-PG** * **Best Prognosis Cytogenetics:** t(12;21) involving *ETV6-RUNX1* (most common in children) and Hyperdiploidy. * **Worst Prognosis Cytogenetics:** t(9;22), t(4;11), and Hypodiploidy (<44 chromosomes) [1]. * **Immunophenotype:** Early pre-B cell (CD10/CALLA positive) has a better prognosis than mature B-cell or T-cell ALL. * **Minimal Residual Disease (MRD):** The most important predictor of relapse after starting treatment.

Question 618: A 25-year-old female presented with mild pallor and moderate hepatosplenomegaly. Her hemoglobin was 92 g/L and fetal hemoglobin level was 65%. She has not received any blood transfusions to date. She is most likely to be suffering from:

A. Thalassemia major
B. Thalassemia intermedia (Correct Answer)
C. Hereditary persistent fetal hemoglobin, homozygous state
D. Hemoglobin D, homozygous state

Explanation: ### Explanation **1. Why Thalassemia Intermedia is Correct:** The clinical hallmark of **Thalassemia Intermedia (TI)** is a phenotype that falls between the asymptomatic trait and the transfusion-dependent Thalassemia Major. * **Clinical Presentation:** The patient has moderate anemia (Hb 92 g/L) and hepatosplenomegaly but, crucially, has **not required blood transfusions** by age 25. This "transfusion-independence" is the defining clinical feature of TI. * **Laboratory Findings:** In TI, there is a marked increase in HbF (often 60–90%) as the body attempts to compensate for the lack of adult hemoglobin (HbA). **2. Why Other Options are Incorrect:** * **Thalassemia Major:** Patients typically present in infancy (6–12 months) with severe anemia (Hb <70 g/L) and require lifelong, regular blood transfusions for survival. A 25-year-old with untreated Major would have severe skeletal deformities and growth failure. * **Hereditary Persistence of Fetal Hemoglobin (HPFH), Homozygous:** While HbF is 100% in this condition, it is a **benign** condition. Patients are usually asymptomatic with near-normal hemoglobin levels and **no splenomegaly**. * **Hemoglobin D Disease:** Homozygous HbD (HbDD) usually causes very mild hemolytic anemia or is asymptomatic. It does not typically present with HbF levels as high as 65%. **3. NEET-PG High-Yield Pearls:** * **The "Transfusion Rule":** If a patient has features of Thalassemia but is surviving without regular transfusions into adulthood, always think **Thalassemia Intermedia**. * **Molecular Basis:** TI is often caused by inheritance of mild $\beta^+$ mutations or the co-inheritance of $\alpha$-thalassemia with $\beta$-thalassemia (which reduces the $\alpha/\beta$ chain imbalance). * **Complications of TI:** Even without transfusions, these patients are at risk for **iron overload** (due to increased GI absorption) and **extramedullary hematopoiesis** (causing "hair-on-end" skull appearance and masses).

Question 619: Thrombocytosis is a recognized feature of which condition?

A. Myelofibrosis (Correct Answer)
B. Systemic lupus erythematosus
C. Azidothymidine therapy
D. All of the above

Explanation: Platelet counts exceeding 450,000/µL characterize thrombocytosis [1]. This condition is a hallmark feature of Myelofibrosis (Primary Myelofibrosis), particularly in its early "pre-fibrotic" stage, where megakaryocytic proliferation is prominent [2]. As a Myeloproliferative Neoplasm (MPN), it involves the clonal proliferation of multipotent hematopoietic stem cells, leading to an overproduction of megakaryocytes and elevated platelet counts before the bone marrow eventually undergoes extensive fibrosis [2]. **Analysis of Options:** * **A. Myelofibrosis (Correct):** In the early stages, there is hypercellularity of the marrow. The platelet count in myelofibrosis may be high, normal, or low depending on the stage of the disease [2]. * **B. Systemic Lupus Erythematosus (SLE):** SLE is typically associated with **thrombocytopenia** (low platelets) due to immune-mediated destruction; SLE is specifically noted as a collagen vascular disease associated with increased platelet consumption [1]. * **C. Azidothymidine (Zidovudine/AZT) therapy:** This antiretroviral drug is notorious for causing **bone marrow suppression**. Its most common hematological side effect is macrocytic anemia and neutropenia, not thrombocytosis. **Clinical Pearls for NEET-PG:** * **MPN Differential:** Thrombocytosis is seen in all "Philadelphia chromosome-negative" MPNs. * **Reactive vs. Clonal:** Always differentiate reactive causes from clonal causes like MPNs. * **Myelofibrosis Hallmark:** Look for **teardrop cells (dacrocytes)** and a **leukoerythroblastic blood picture** on a peripheral smear, along with a "dry tap" on bone marrow aspiration [2].

Question 620: Disseminated Intravascular Coagulation (DIC) is common in which subtype of Acute Myeloid Leukemia?

A. Monocytic (M5)
B. Promyelocytic (M3) (Correct Answer)
C. Erythrocytic (M6)
D. Megakaryocytic (M7)

Explanation: **Explanation** **Acute Promyelocytic Leukemia (APL)**, formerly classified as **FAB M3**, is the subtype most strongly associated with life-threatening **Disseminated Intravascular Coagulation (DIC)**. **Why M3 is the Correct Answer:** The hallmark of APL is the translocation **t(15;17)**, which results in the accumulation of abnormal promyelocytes containing numerous **Auer rods** [1]. These cells contain high concentrations of **Tissue Factor** and **Annexin II**. When these cells undergo apoptosis or are lysed by chemotherapy, they release these procoagulant granules into the circulation. This triggers the extrinsic coagulation pathway and primary fibrinolysis, leading to a "consumptive coagulopathy" (DIC) characterized by low fibrinogen, prolonged PT/aPTT, and severe bleeding [2]. **Analysis of Incorrect Options:** * **M5 (Monocytic):** Characterized by gum hypertrophy and CNS involvement. While it can cause DIC, it is much less frequent than in M3. * **M6 (Erythrocytic):** Associated with bizarre multinucleated erythroblasts; not typically linked to acute coagulopathy. * **M7 (Megakaryocytic):** Frequently associated with **Acute Myelofibrosis** and is common in children with Down Syndrome (under age 5). **High-Yield Clinical Pearls for NEET-PG:** * **Morphology:** Look for "Faggot cells" (cells with bundles of Auer rods) in the peripheral smear. * **Treatment:** Emergency management involves **ATRA (All-Trans Retinoic Acid)** and Arsenic Trioxide, which promote the differentiation of promyelocytes into mature neutrophils, rapidly resolving the DIC. * **Differentiation Syndrome:** A common complication of ATRA treatment, presenting with fever, dyspnea, and pulmonary infiltrates.

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Hemoglobinopathies

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Thalassemias

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Platelet Disorders

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Coagulation Disorders

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Thrombotic Disorders

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Leukemias

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Lymphomas

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Multiple Myeloma and Plasma Cell Disorders

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Myeloproliferative Neoplasms

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Transfusion Medicine

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Hematopoietic Stem Cell Transplantation

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Hematology — MCQs

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