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**.

Q: The use of desmopressin is best indicated for therapy in which of the following bleeding disorders?

Von Willebrand disease (VWD). **Explanation:** **Desmopressin (DDAVP)** is a synthetic analogue of vasopressin that stimulates the release of **von Willebrand factor (vWF)** and **Factor VIII** from endothelial storage sites (Weibel-Palade bodies) [2]. 1. **Why Option C is Correct:** In **Von Willebrand Disease (VWD)**, particularly Type 1 (the most common form), there is a quantitative deficiency of vWF [2]. DDAVP effectively increases endogenous plasma levels of vWF and Factor VIII, making it the treatment of choice for minor bleeding episodes or surgical prophylaxis in these patients [2]. 2. **Why Other Options are Incorrect:** * **Severe Hemophilia A (Option A):** DDAVP can be used in *mild* Hemophilia A. However, in *severe* cases (Factor VIII <1%), there are no endogenous stores to release; therefore, exogenous Factor VIII replacement is mandatory. [1] * **Severe Hemophilia B (Option B):** Hemophilia B is a deficiency of Factor IX. DDAVP has no effect on Factor IX levels; these patients require Factor IX concentrates. * **Glanzmann Thrombasthenia (Option D):** This is a qualitative platelet disorder (deficiency of GpIIb/IIIa) [3]. DDAVP does not correct this receptor defect; treatment usually involves platelet transfusions or recombinant Factor VIIa. **NEET-PG High-Yield Pearls:** * **Route:** DDAVP can be administered IV, SC, or via a highly concentrated nasal spray (Stimate). * **Side Effects:** The most critical side effect is **hyponatremia** (due to the antidiuretic effect), which can lead to seizures. Patients should be advised on fluid restriction. * **Tachyphylaxis:** Repeated doses lead to a diminishing response as endothelial stores become exhausted. * **Contraindication:** Avoid DDAVP in **VWD Type 2B**, as it can cause paradoxical thrombocytopenia due to the release of abnormal vWF that induces platelet aggregation.

Q: Which drug is used for the treatment of Acute Myeloid Leukemia (AML) with FLT-3 mutation?

Gilteritinib. **Explanation:** **Acute Myeloid Leukemia (AML)** management has evolved with the identification of specific molecular markers. The **FLT3 (Fms-like tyrosine kinase 3)** mutation is one of the most common genetic alterations in AML (seen in ~30% of cases) and is associated with a poor prognosis and high relapse rates. * **Why Gilteritinib is correct:** **Gilteritinib** is a highly potent, selective **second-generation FLT3 inhibitor**. It targets both FLT3-ITD (Internal Tandem Duplication) and FLT3-TKD (Tyrosine Kinase Domain) mutations. It is specifically FDA-approved for the treatment of adult patients with **relapsed or refractory AML** with a FLT3 mutation. Other FLT3 inhibitors used in AML include Midostaurin (used in induction) and Quizartinib. **Analysis of Incorrect Options:** * **A. Crizotinib:** An ALK (Anaplastic Lymphoma Kinase) and ROS1 inhibitor primarily used in **Non-Small Cell Lung Cancer (NSCLC)** and ALK-positive Anaplastic Large Cell Lymphoma. * **C. Acalabrutinib:** A second-generation **Bruton Tyrosine Kinase (BTK) inhibitor** used in Chronic Lymphocytic Leukemia (CLL) and Mantle Cell Lymphoma. * **D. Ibrutinib:** The first-generation **BTK inhibitor** used for CLL, Small Lymphocytic Lymphoma (SLL), and Waldenström Macroglobulinemia. **High-Yield Clinical Pearls for NEET-PG:** * **FLT3-ITD** mutation is a marker of **poor prognosis** in AML [1]. * **Midostaurin** is the first-generation FLT3 inhibitor added to standard "7+3" chemotherapy for newly diagnosed FLT3+ AML. * **All-trans retinoic acid (ATRA)** and **Arsenic Trioxide** remain the drugs of choice for the M3 subtype (APML) characterized by t(15,17) [1]. * **Venetoclax** (BCL-2 inhibitor) is another high-yield drug now used in AML for elderly patients unfit for intensive chemotherapy.

Question 1

Which drug is associated with warm antibody hemolytic anemia?

Accepted Answer

Methyldopa

Answer

EBV infection

Answer

Quinine

Answer

Mycoplasma infection

Question 2

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 3

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 4

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 5

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 6

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 7

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

Question 8

A 22-year-old woman from a large Italian family is screened for a familial blood disorder. Laboratory studies reveal a hemoglobin of 9.5 g/dL and a peripheral smear showing mild microcytosis, hypochromia, and a few target cells. Hemoglobin electrophoresis demonstrates a mild increase in hemoglobin A2 (7.5%). What is the most likely diagnosis?

Accepted Answer

Heterozygous beta-thalassemia

Answer

Anemia of chronic disease

Answer

G6PD deficiency

Answer

Homozygous beta-thalassemia

Question 9

The use of desmopressin is best indicated for therapy in which of the following bleeding disorders?

Accepted Answer

Von Willebrand disease (VWD)

Answer

Severe hemophilia A

Answer

Severe hemophilia B

Answer

Glanzmann thrombasthenia

Question 10

Which drug is used for the treatment of Acute Myeloid Leukemia (AML) with FLT-3 mutation?

Accepted Answer

Gilteritinib

Answer

Crizotinib

Answer

Acalabrutinib

Answer

Ibrutinib

Hematology — MCQs

Hematology — MCQs

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