Hematology Practice Questions

Q: Which drug is associated with warm antibody hemolytic anemia?

Methyldopa. Autoimmune Hemolytic Anemia (AIHA) is classified into **Warm (IgG)** and **Cold (IgM)** types based on the thermal reactivity of the antibodies. [1] **1. Why Methyldopa is Correct:** Methyldopa is the classic prototype drug associated with **Warm AIHA**. It induces an autoimmune response by altering the antigens on the red blood cell (RBC) surface or by interfering with T-suppressor cell function, leading to the production of **true autoantibodies** against Rh antigens. Characteristically, the Direct Antiglobulin Test (Coombs test) remains positive even after the drug is discontinued, although hemolysis usually resolves. [1] **2. Why the other options are incorrect:** * **EBV Infection:** Associated with **Cold Agglutinin Disease (Cold AIHA)**. It typically triggers the production of anti-i antibodies. * **Mycoplasma pneumoniae:** Another classic cause of **Cold AIHA**, specifically associated with **anti-I antibodies** (large 'I'). [1] * **Quinine:** Causes drug-induced hemolytic anemia via the **"Innocent Bystander" mechanism** (Type II hypersensitivity). It involves IgM antibodies forming immune complexes that fix complement on the RBC surface, leading to acute intravascular hemolysis, rather than the warm IgG-mediated extravascular hemolysis seen with Methyldopa. **High-Yield Clinical Pearls for NEET-PG:** * **Warm AIHA (IgG):** "Warm Weather is Great" (IgG). Most common type. Associated with SLE, CLL, and drugs like **Methyldopa, Penicillin, and Fludarabine**. * **Cold AIHA (IgM):** "Cold Ice Cream" (IgM). Associated with **Mycoplasma, Infectious Mononucleosis (EBV)**, and Lymphoma. [1] * **Blood Film:** Warm AIHA typically shows **Spherocytes** (due to partial phagocytosis in the spleen), whereas Cold AIHA shows **RBC Clumping/Agglutination**. [1]

Q: A 46-year-old woman with prominent splenomegaly presents with a 3-month history of malaise, easy fatigability, weakness, weight loss, and anorexia. A complete blood count and differential demonstrates a white blood cell count of 250,000/mm3 with a predominance of myelocytes, metamyelocytes, band cells, and segmented neutrophils. Cytogenetic analysis is most likely to reveal which of the following translocations?

t(9;22). The clinical presentation of massive splenomegaly, constitutional symptoms (weight loss, malaise), and a markedly elevated WBC count (250,000/mm³) with a "left shift" (presence of myelocytes, metamyelocytes, and bands) is classic for **Chronic Myeloid Leukemia (CML)**. **Why t(9;22) is correct:** CML is characterized by the **Philadelphia chromosome**, which results from a reciprocal translocation between chromosomes 9 and 22, **t(9;22)(q34;q11)** [1]. This fuses the *ABL1* gene on chromosome 9 with the *BCR* gene on chromosome 22, creating the **BCR-ABL1 fusion protein** [1]. This protein is a constitutively active tyrosine kinase that drives uncontrolled proliferation of the myeloid lineage. **Analysis of Incorrect Options:** * **t(8;14):** Associated with **Burkitt Lymphoma**. It involves the translocation of the *c-MYC* proto-oncogene to the immunoglobulin heavy chain (IgH) locus. * **t(11;22):** Associated with **Ewing Sarcoma**. It results in the *EWS-FLI1* fusion gene. * **t(14;18):** Associated with **Follicular Lymphoma**. It involves the *BCL-2* proto-oncogene, leading to the inhibition of apoptosis. **High-Yield Clinical Pearls for NEET-PG:** * **Leukocyte Alkaline Phosphatase (LAP) Score:** Characteristically **low** in CML (helps differentiate it from a Leukemoid reaction, where LAP is high). * **Peripheral Smear:** Shows a "bulge" in myelocytes and neutrophils; **basophilia** is a highly specific finding for CML. * **Treatment:** The first-line treatment is **Imatinib** (a Tyrosine Kinase Inhibitor) [1]. * **Phases:** CML progresses from a Chronic phase to an Accelerated phase, and finally to a **Blast Crisis** (can be AML or ALL).

Q: Which of the following is NOT true of long-standing sickle cell anemia?

Massive splenomegaly. The hallmark of long-standing Sickle Cell Anemia (SCA) is **autosplenectomy**, which makes **Massive Splenomegaly (Option D)** the correct answer as it is characteristically absent in adults. [1] 1. **Why Option D is correct:** In early childhood, patients with SCA may have splenomegaly. However, repeated episodes of vaso-occlusive crises lead to multiple splenic infarcts. Over time, the spleen becomes fibrotic, shrunken, and calcified—a process known as **autosplenectomy** [1]. By adulthood, the spleen is usually non-functional and not palpable. Massive splenomegaly is more characteristic of conditions like Myelofibrosis, Chronic Myeloid Leukemia, or Malaria. 2. **Why other options are incorrect:** * **Option A:** SCA is a hemolytic anemia where RBC indices typically remain within the normal range, resulting in a **normocytic normochromic** pattern unless there is a coexisting nutritional deficiency (like folate). * **Option B:** Chronic hemolysis leads to increased production of unconjugated bilirubin, causing **recurrent jaundice** (icterus). * **Option C:** Persistent hyperbilirubinemia results in the formation of calcium bilirubinate stones, making **cholelithiasis** (pigment gallstones) a very common complication. **High-Yield Clinical Pearls for NEET-PG:** * **Howell-Jolly Bodies:** These nuclear remnants are seen on a peripheral smear due to functional asplenia/autosplenectomy. * **Acute Splenic Sequestration:** A life-threatening crisis in children where the spleen suddenly enlarges due to trapped blood; this is the exception to the "small spleen" rule in SCA [1]. * **Salmonella Osteomyelitis:** Patients with SCA are uniquely predisposed to this infection due to splenic dysfunction. * **H-shaped vertebrae:** Also known as "Codfish vertebrae," seen on X-ray due to end-plate infarction.

Q: Which of the following is NOT a feature of Antiphospholipid Syndrome (APS)?

Decreased PT with increased APTT. Antiphospholipid Syndrome (APS) is an autoimmune prothrombotic state characterized by arterial/venous thrombosis and pregnancy complications. [1] **Why Option D is the Correct Answer:** In APS, the presence of **Lupus Anticoagulant (LA)** interferes with phospholipid-dependent coagulation tests *in vitro*. This leads to a **prolonged (increased) APTT** that does not correct with a mixing study. However, the **Prothrombin Time (PT) is typically normal**, not decreased. [1] The hallmark of APS is this "laboratory paradox": an increased APTT (suggesting bleeding risk) despite the patient being in a hypercoagulable state (thrombosis risk). **Analysis of Incorrect Options:** * **A. Haemolytic Anemia:** Autoimmune hemolytic anemia (AIHA) and Evans Syndrome (AIHA + thrombocytopenia) are well-recognized hematologic manifestations associated with APS and SLE. * **B. False positive VDRL:** The reagin antibodies in syphilis cross-react with the cardiolipin antigen used in the VDRL/RPR tests. Since anti-cardiolipin antibodies are a staple of APS, a false positive syphilis screen is a classic diagnostic clue. * **C. Thrombocytopenia:** Mild to moderate thrombocytopenia (100,000–150,000/mm³) occurs in nearly 30% of APS patients due to increased platelet consumption and anti-platelet antibodies. **NEET-PG High-Yield Pearls:** * **Sapporo Criteria:** Diagnosis requires 1 clinical (thrombosis or pregnancy loss) + 1 lab criterion (Anti-cardiolipin, Anti-β2 glycoprotein I, or Lupus Anticoagulant) positive on two occasions 12 weeks apart. * **Mixing Study:** APTT remains prolonged in APS (presence of inhibitor), whereas it corrects in factor deficiencies (e.g., Hemophilia). [1] * **Treatment:** Lifelong anticoagulation with Warfarin (INR 2-3) is preferred; DOACs are generally avoided in triple-positive APS. [2]

Q: A 69-year-old woman presents with fatigue. Her hemoglobin is 9.0 g/dL (14 g/dL one year prior) with no other symptoms. A blood film is provided. What is the most appropriate next diagnostic step?

Colonoscopy. ***Colonoscopy*** - In a **post-menopausal** 69-year-old woman with **microcytic hypochromic anemia**, the most likely cause is **iron deficiency** from **occult GI bleeding**. - **Colonoscopy** is essential to rule out **colorectal malignancy** and identify sources of chronic blood loss in this age group. *Serum B12 level* - **B12 deficiency** typically causes **macrocytic anemia** with **megaloblastic** changes on blood film, not microcytic hypochromic RBCs. - The blood film shows **microcytic** RBCs, making B12 deficiency unlikely as the primary cause. *Hemoglobin electrophoresis* - Used to diagnose **hemoglobinopathies** like **thalassemia** or **sickle cell disease**, which are congenital conditions. - This 69-year-old woman had **normal hemoglobin** (14 g/dL) one year prior, making acquired **iron deficiency** more likely than congenital hemoglobinopathy. *Bone marrow aspiration* - Reserved for cases where the diagnosis remains unclear after initial investigations or when **hematologic malignancy** is suspected. - **Iron deficiency anemia** can usually be diagnosed and managed without bone marrow examination, especially when **GI source** is likely.

Q: Which of the following is a characteristic feature of hypoproliferative anemia?

Normocytic normochromic anemia with reticulocyte index < 2-2.5. Explanation: Hypoproliferative anemias are characterized by a failure of the bone marrow to produce an appropriate number of erythrocytes in response to a decrease in hemoglobin. This failure is objectively measured by the **Reticulocyte Index (RI)**. 1. **Why Option A is Correct:** In hypoproliferative states (such as early iron deficiency, inflammation, or renal disease), the bone marrow cannot mount an appropriate erythropoietic response [1]. Therefore, the RI remains low (**< 2–2.5**). Morphologically, these anemias are typically **normocytic and normochromic** because the RBCs that *are* produced are of normal size and hemoglobin content, but they are simply produced in insufficient quantities. 2. **Why the other options are incorrect:** * **Option B:** While the RI is low, microcytic hypochromic anemia is more characteristic of **maturation disorders** (like established iron deficiency or thalassemia) rather than pure hypoproliferative states [1]. * **Option C:** An RI **> 2.5** indicates an appropriate marrow response, typically seen in **hemolytic anemias** or acute blood loss, where the marrow is hyperproliferative. * **Option D:** Ringed sideroblasts are the hallmark of **Sideroblastic Anemia**, which is classified as a cytoplasmic maturation defect, not a primary hypoproliferative anemia. **High-Yield Clinical Pearls for NEET-PG:** * **The RI Formula:** $\text{Reticulocyte Count} \times (\text{Observed Hct} / \text{Normal Hct}) \times (1 / \text{Maturation Correction Factor})$. * **Common Causes:** The most common cause of hypoproliferative anemia is **Iron Deficiency** (early stage), followed by **Anemia of Chronic Disease/Inflammation** [1]. * **Key Differentiator:** If the MCV is abnormal (high or low) with a low RI, think of **Maturation Disorders**. If the MCV is normal with a low RI, think **Hypoproliferative Anemia**.

Question 1

Spherocytes are not seen in which of the following conditions?

Accepted Answer

Plasmodium falciparum infection

Answer

Splenectomy

Answer

Hereditary spherocytosis

Answer

Autoimmune hemolytic anemia

Question 2

A 15-year-old boy presents with acute onset of right flank pain that developed after physical exercise. Physical examination demonstrates an area of ecchymosis in the right flank that is tender to palpation. The patient has a lifelong history of easy bruising, and a sibling has the same symptoms. The serum level of clotting factor VIII is less than 2% of normal. Which of the following is the most likely underlying mechanism for the bleeding tendency in this patient?

Accepted Answer

Genetic defect involving the factor VIII gene

Answer

Deficiency of vitamin K

Answer

Decreased hepatic synthesis of multiple coagulation factors

Answer

Circulating antibodies directed against factor VIII

Question 3

A 48-year-old woman presents with a 3-week history of fatigue and yellow skin and sclerae. Physical examination reveals mild jaundice. Laboratory findings include a serum bilirubin level of 3.7 mg/dL (predominantly unconjugated), normal serum AST, ALT, and alkaline phosphatase, and a hemoglobin level of 6.0 g/dL. Jaundice resolved after administration of corticosteroids. What is the most likely cause of hyperbilirubinemia in this patient?

Accepted Answer

Autoimmune hemolytic anemia

Answer

Acute hepatitis B infection

Answer

Gallstone in the common bile duct

Answer

Primary biliary cirrhosis

Question 4

Which drug is associated with warm antibody hemolytic anemia?

Accepted Answer

Methyldopa

Answer

EBV infection

Answer

Quinine

Answer

Mycoplasma infection

Question 5

A 46-year-old woman with prominent splenomegaly presents with a 3-month history of malaise, easy fatigability, weakness, weight loss, and anorexia. A complete blood count and differential demonstrates a white blood cell count of 250,000/mm3 with a predominance of myelocytes, metamyelocytes, band cells, and segmented neutrophils. Cytogenetic analysis is most likely to reveal which of the following translocations?

Accepted Answer

t(9;22)

Answer

t(8;14)

Answer

t(11;22)

Answer

t(14;18)

Question 6

Which of the following is NOT true of long-standing sickle cell anemia?

Accepted Answer

Massive splenomegaly

Answer

Normocytic normochromic anemia

Answer

Recurrent jaundice

Answer

Cholelithiasis

Question 7

Which of the following is NOT a feature of Antiphospholipid Syndrome (APS)?

Accepted Answer

Decreased PT with increased APTT

Answer

Haemolytic anemia

Answer

False positive VDRL

Answer

Thrombocytopenia

Question 8

A 69-year-old woman presents with fatigue. Her hemoglobin is 9.0 g/dL (14 g/dL one year prior) with no other symptoms. A blood film is provided. What is the most appropriate next diagnostic step?

Accepted Answer

Colonoscopy

Answer

Serum B12 level

Answer

Hemoglobin electrophoresis

Answer

Bone marrow aspiration

Question 9

To which of the following diseases is pyruvate kinase deficiency most similar clinically?

Accepted Answer

Glucose-6-phosphate dehydrogenase deficiency

Answer

Alpha thalassemia

Answer

Beta thalassemia

Answer

Hereditary spherocytosis

Question 10

Which of the following is a characteristic feature of hypoproliferative anemia?

Accepted Answer

Normocytic normochromic anemia with reticulocyte index < 2-2.5

Answer

Microcytic hypochromic anemia with reticulocyte index < 2-2.5

Answer

Microcytic hypochromic anemia with reticulocyte index > 2-2.5

Answer

Microcytic anemia with ringed sideroblasts

Hematology — MCQs

Hematology — MCQs

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