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: Which of the following is not a cause of secondary polycythemia?

Myeloproliferative changes. Polycythemia is classified into **Primary** and **Secondary** types based on the serum Erythropoietin (EPO) levels and the underlying trigger for red cell production. **1. Why "Myeloproliferative changes" is the correct answer:** Myeloproliferative changes, specifically **Polycythemia Vera (PV)**, represent **Primary Polycythemia**. In PV, there is an autonomous, neoplastic proliferation of erythroid precursors in the bone marrow, usually due to a mutation in the **JAK2 gene** [1]. Because the production is independent of external stimuli, **serum EPO levels are characteristically low**. **2. Why the other options are incorrect (Causes of Secondary Polycythemia):** Secondary polycythemia occurs when red cell production is driven by **elevated EPO levels**. * **High Altitude (Option A):** Chronic hypoxia at high altitudes triggers the kidneys to release more EPO (physiologic compensation) to increase oxygen-carrying capacity [1]. * **Pheochromocytoma (Option C) & Cerebellar Hemangioblastoma (Option D):** These are classic examples of **inappropriate EPO secretion**. Certain tumors (including Renal Cell Carcinoma and Hepatocellular Carcinoma) ectopically produce EPO, leading to increased erythropoiesis. **Clinical Pearls for NEET-PG:** * **JAK2 V617F Mutation:** Present in >95% of Polycythemia Vera cases [1]. * **Diagnostic Clue:** If a patient has a high hematocrit and **low EPO**, think Primary (PV). If **high EPO**, think Secondary (Hypoxia or Tumor). * **Hyperviscosity:** Both types can present with headache, dizziness, and thrombosis, but **aquagenic pruritus** (itching after a hot bath) and **splenomegaly** are highly suggestive of Polycythemia Vera [1]. * **Rule of 4 (Tumors causing Secondary Polycythemia):** Renal Cell Carcinoma, Hepatoma, Cerebellar Hemangioblastoma, and Uterine Fibroids (and Pheochromocytoma).

Q: Von Willebrand disease is associated with which of the following laboratory findings?

Prolonged PTT; Normal PT; Prolonged BT; Prolonged CT. Von Willebrand Disease (vWD) is the most common inherited bleeding disorder, characterized by a deficiency or dysfunction of **von Willebrand Factor (vWF)**. To understand the lab findings, one must recall the dual role of vWF: 1. **Platelet Adhesion:** vWF acts as a bridge between platelets (GpIb receptor) and the subendothelial collagen [4]. A deficiency leads to impaired primary hemostasis, resulting in a **Prolonged Bleeding Time (BT)**. 2. **Stabilization of Factor VIII:** vWF serves as a carrier protein for Factor VIII, protecting it from rapid degradation. Low vWF leads to secondary Factor VIII deficiency, which impairs the intrinsic pathway, resulting in a **Prolonged PTT** and a **Prolonged Clotting Time (CT)** [2][3]. **Analysis of Options:** * **Correct Answer (D):** Reflects the dual defect. Prolonged BT (platelet dysfunction) + Prolonged PTT/CT (Factor VIII deficiency). * **Option A & B:** Incorrect because the **Prothrombin Time (PT)** measures the extrinsic pathway (Factor VII) [2]. vWD does not affect Factor VII or the common pathway, so PT remains **Normal**. * **Option C:** Incorrect because a significant deficiency in Factor VIII (secondary to low vWF) will typically prolong the **Clotting Time (CT)**, which is a measure of the time required for whole blood to clot via the intrinsic system. **NEET-PG High-Yield Pearls:** * **Inheritance:** Most types are Autosomal Dominant (Type 1 is most common). * **Screening Test of Choice:** Ristocetin Cofactor Assay (measures vWF-induced platelet agglutination) [3]. * **Clinical Presentation:** Mucocutaneous bleeding (epistaxis, menorrhagia, gingival bleeding) [3]. * **Treatment:** **Desmopressin (DDAVP)** releases stored vWF from Weibel-Palade bodies in endothelial cells [3]. For severe cases, use vWF/Factor VIII concentrates [1].

Q: In multiple myeloma, which of the following clinical manifestations are typically seen?

Increased serum calcium. Multiple myeloma is a plasma cell dyscrasia characterized by the neoplastic proliferation of a single clone of plasma cells [1]. The clinical manifestations are classically remembered by the mnemonic **CRAB**: **C**alcium elevation, **R**enal insufficiency, **A**nemia, and **B**one lesions [1]. 1. **Why Option A is correct:** Hypercalcemia occurs due to increased osteoclast activity mediated by **RANK-ligand** and various cytokines (like IL-6 and TNF-α) secreted by myeloma cells. This leads to extensive bone resorption, releasing calcium into the extracellular fluid. 2. **Why Options B and C are incorrect (in the context of this specific question):** While bone pain (due to lytic lesions/pathological fractures) and renal failure (due to Bence-Jones proteinuria/cast nephropathy) are hallmark features of multiple myeloma [1], the question asks for the clinical manifestation typically seen among the choices. In many standardized formats, if "All of the above" is present but a specific physiological marker like hypercalcemia is highlighted, it emphasizes the metabolic hallmark. However, **clinically, all three are correct.** In the context of the provided answer key, hypercalcemia is the primary metabolic derangement. **Clinical Pearls for NEET-PG:** * **Diagnosis:** The "Gold Standard" is bone marrow aspiration/biopsy showing **>10% clonal plasma cells** [1]. * **Peripheral Smear:** Characterized by **Rouleaux formation** due to high ESR and paraproteins. * **Radiology:** "Punched-out" lytic lesions on a skeletal survey [1]; **Skull X-ray** shows a "pepper pot" appearance. * **Renal Pathology:** "Myeloma Kidney" or Cast Nephropathy is caused by the precipitation of light chains (Bence-Jones proteins) in the distal tubules. * **M-Spike:** Found on Serum Protein Electrophoresis (SPEP), usually IgG (most common) or IgA.

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.

Question 1

A 65-year-old man presents with tingling in his hands and feet and increased forgetfulness. A CBC shows mild anemia. The patient reports no change in diet, other than increased consumption of red meat. What is the most appropriate treatment for this patient?

Accepted Answer

Intramuscular vitamin B12 injections

Answer

Oral vitamin B12

Answer

Oral folic acid

Answer

Oral methionine

Question 2

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 3

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 4

Which drug is associated with warm antibody hemolytic anemia?

Accepted Answer

Methyldopa

Answer

EBV infection

Answer

Quinine

Answer

Mycoplasma infection

Question 5

Which of the following is not a cause of secondary polycythemia?

Accepted Answer

Myeloproliferative changes

Answer

High Altitude

Answer

Pheochromocytoma

Answer

Cerebellar hemangioblastoma

Question 6

Von Willebrand disease is associated with which of the following laboratory findings?

Accepted Answer

Prolonged PTT; Normal PT; Prolonged BT; Prolonged CT

Answer

Prolonged PTT; Prolonged PT; Prolonged BT; Prolonged CT

Answer

Prolonged PTT; Prolonged PT; Prolonged BT; Normal CT

Answer

Prolonged PTT; Normal PT; Prolonged BT; Normal CT

Question 7

In multiple myeloma, which of the following clinical manifestations are typically seen?

Accepted Answer

Increased serum calcium

Answer

Bone pain

Answer

Renal failure

Answer

All of the above

Question 8

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 9

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 10

What is the standard treatment for Hodgkin's disease of the stomach?

Accepted Answer

Gastric resection and chemotherapy

Answer

Gastric resection

Answer

None of these

Answer

Purely medical treatment

Hematology — MCQs

Hematology — MCQs

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