Hematology Practice Questions

Q: All of the following are causes of aplastic anemia except?

Cold hemoglobinuria. **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.

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

Factor 9. **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.

Q: 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?

Hereditary spherocytosis. ### 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).

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

t (9;22); t (4; 11). 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 10 years (adolescents/adults) indicates a poorer prognosis. * **D. 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.

Q: Thrombocytosis is a recognized feature of which condition?

Myelofibrosis. 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].

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

Promyelocytic (M3). **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.

Question 1

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

Accepted Answer

Dyskeratosis congenita

Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Megaloblastic anemia

Answer

Hairy cell leukemia

Question 2

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?

Accepted Answer

Protein electrophoresis

Answer

Serum calcium

Answer

Alkaline phosphatase levels

Answer

PSA levels

Question 3

All of the following are causes of aplastic anemia except?

Accepted Answer

Cold hemoglobinuria

Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Hepatitis

Answer

Pregnancy

Question 4

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

Accepted Answer

Hepatocellular disease

Answer

Drug-induced anemia

Answer

Renal disease

Answer

Alcoholism

Question 5

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

Accepted Answer

Factor 9

Answer

Factor 8

Answer

Factor 7

Answer

Factor 11

Question 6

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?

Accepted Answer

Hereditary spherocytosis

Answer

Sickle cell anemia

Answer

Acute pancreatitis

Answer

Cholangitis

Question 7

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

Accepted Answer

t (9;22); t (4; 11)

Answer

Hyperdiploidy

Answer

2-8 years of age

Answer

WBC count < 50,000

Question 8

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:

Accepted Answer

Thalassemia intermedia

Answer

Thalassemia major

Answer

Hereditary persistent fetal hemoglobin, homozygous state

Answer

Hemoglobin D, homozygous state

Question 9

Thrombocytosis is a recognized feature of which condition?

Accepted Answer

Myelofibrosis

Answer

Systemic lupus erythematosus

Answer

Azidothymidine therapy

Answer

All of the above

Question 10

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

Accepted Answer

Promyelocytic (M3)

Answer

Monocytic (M5)

Answer

Erythrocytic (M6)

Answer

Megakaryocytic (M7)

Hematology — MCQs

Hematology — MCQs

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