Hematology Practice Questions

Q: Waldenstrom's macroglobulinemia is seen in which of the following conditions?

Plasmacytic Lymphoma. **Explanation:** **Waldenström’s Macroglobulinemia (WM)** is a distinct clinical syndrome defined by the presence of a monoclonal IgM protein (macroglobulinemia) in the setting of bone marrow infiltration by **Lymphoplasmacytic Lymphoma (LPL)** [1]. 1. **Why Option B is Correct:** According to the WHO classification, Waldenström’s Macroglobulinemia is the clinical manifestation of Lymphoplasmacytic Lymphoma (LPL). LPL is a B-cell neoplasm characterized by a mixture of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells. These cells secrete large amounts of **monoclonal IgM**, leading to the hallmark hyperviscosity syndrome [1]. 2. **Why Other Options are Incorrect:** * **Option A (Non-Hodgkin’s Lymphoma):** While LPL is technically a subtype of indolent NHL, the question asks for the specific underlying condition. "NHL" is too broad a category, whereas Plasmacytic (Lymphoplasmacytic) Lymphoma is the definitive pathological diagnosis. * **Option C (Chronic Lymphocytic Leukemia):** CLL is a malignancy of mature B-cells that typically expresses CD5 and CD23. While CLL can occasionally produce a monoclonal protein (M-spike), it is usually IgG or IgA, and it does not define Waldenström’s. **High-Yield Clinical Pearls for NEET-PG:** * **Hallmark Symptom:** Hyperviscosity syndrome (visual disturbances, mucosal bleeding, and neurological symptoms like "sausage-link" appearance of retinal veins) [1]. * **Genetic Marker:** Over 90% of patients possess the **MYD88 L265P mutation**. * **Diagnosis:** Bone marrow biopsy showing >10% infiltration by lymphoplasmacytic cells + any size IgM monoclonal spike [1]. * **Distinction from Multiple Myeloma:** Unlike Myeloma, WM rarely causes lytic bone lesions or hypercalcemia; it is primarily a disease of organomegaly (hepatosplenomegaly and lymphadenopathy).

Q: Bone marrow transplantation is used as a treatment modality in all of the following conditions except:

Hemochromatosis. **Explanation:** Bone marrow transplantation (BMT), or Hematopoietic Stem Cell Transplantation (HSCT), is used to treat conditions where the underlying pathology resides in the hematopoietic system or where donor cells can provide a missing enzyme or functional cell type [1]. **Why Hemochromatosis is the Correct Answer:** Hereditary Hemochromatosis is a disorder of **iron metabolism** caused primarily by mutations in the *HFE* gene, leading to excessive intestinal iron absorption. The defect lies in the liver (hepcidin regulation) and the gut, not the bone marrow. Therefore, BMT has no role in its management. The standard treatment remains **therapeutic phlebotomy** or iron chelation. **Analysis of Other Options:** * **Adrenoleukodystrophy (ALD):** This is a peroxisomal disorder. HSCT is the standard of care in the early stages of childhood cerebral ALD as it provides donor-derived microglial cells that can process very-long-chain fatty acids (VLCFA), halting neurodegeneration. * **Hurler’s Syndrome (MPS I):** A lysosomal storage disorder caused by alpha-L-iduronidase deficiency. BMT provides donor cells that secrete the functional enzyme, which is then taken up by the patient's cells (cross-correction), preventing progressive organ damage and cognitive decline. * **Osteopetrosis:** The "marble bone disease" is caused by **defective osteoclasts** (which are derived from the monocyte-macrophage lineage in the bone marrow). BMT provides functional donor-derived osteoclasts that can perform normal bone resorption. **NEET-PG High-Yield Pearls:** * **Osteopetrosis** is a classic "non-malignant" indication for BMT often tested in exams. * **Gaucher disease** and **Niemann-Pick** are other storage disorders where BMT may be considered. * In **Thalassemia and Sickle Cell Anemia**, BMT is currently the only curative treatment [2].

Q: An 18-year-old man of Italian descent presents with a hypochromic microcytic anemia of 10 g/dL, characterized by anisocytosis, poikilocytosis, and target cells on peripheral blood smear. His WBC count is 9500/mL, platelet count is 240,000/mL, and reticulocyte count is 7%. The spleen is palpable 5 cm below the left costal margin. What is the most likely diagnosis?

Thalassemia minor. **Explanation:** The clinical presentation points towards **Thalassemia Minor (Beta-Thalassemia Trait)**. The patient exhibits a classic triad: Mediterranean (Italian) descent, mild microcytic hypochromic anemia (Hb 10 g/dL), and significant splenomegaly. **Why Thalassemia Minor is correct:** 1. **Peripheral Smear:** The presence of **target cells**, anisocytosis, and poikilocytosis is hallmark. 2. **Reticulocytosis:** A reticulocyte count of 7% indicates a compensatory marrow response to chronic hemolysis or ineffective erythropoiesis. 3. **Splenomegaly:** Chronic extravascular hemolysis leads to palpable splenomegaly, which is common in Thalassemia but rare in simple Iron Deficiency Anemia. 4. **Mentzer Index (High-Yield):** Though not explicitly calculated here, Thalassemia typically presents with a very low MCV relative to the RBC count (Mentzer Index <13). **Why other options are incorrect:** * **Sickle cell trait:** Usually asymptomatic with a normal peripheral smear (no sickling or target cells) and no splenomegaly [1]. * **HbS-C disease:** While it shows target cells and splenomegaly, it typically presents with more significant vaso-occlusive symptoms and specific "HbC crystals" on the smear. * **Sideroblastic anemia:** Characterized by dimorphic RBC populations and ringed sideroblasts in the bone marrow; it does not specifically correlate with Italian descent or the degree of splenomegaly seen here. **NEET-PG High-Yield Pearls:** * **Confirmatory Test:** Hb Electrophoresis showing **increased HbA2 (>3.5%)** is diagnostic for Beta-Thalassemia minor. * **Target Cells (Codocytes):** Seen in "HALT" (HbC disease, Asplenia, Liver disease, Thalassemia). * **Differentiating from IDA:** Thalassemia has a **normal to high RBC count** despite low Hb; Iron Deficiency has a low RBC count.

Q: Which of the following is NOT a Vitamin K-dependent clotting factor?

Factor VIII. **Explanation:** The synthesis of certain coagulation factors in the liver requires **Vitamin K** as a cofactor for the enzyme **gamma-glutamyl carboxylase**. This enzyme adds a carboxyl group to glutamate residues on these proteins, allowing them to bind calcium ions and anchor to phospholipid surfaces, which is essential for their activation [1]. **Why Factor VIII is the correct answer:** Factor VIII (Anti-hemophilic factor) is **not** Vitamin K-dependent. Unlike most other clotting factors synthesized in the liver hepatocytes, Factor VIII is primarily produced in the **sinusoidal endothelial cells** of the liver and extrahepatic endothelial cells. It circulates in the plasma bound to von Willebrand Factor (vWF). **Analysis of incorrect options:** * **Factor II (Prothrombin):** A key Vitamin K-dependent serine protease that is converted to thrombin [1], [2]. * **Factor IX (Christmas Factor):** A Vitamin K-dependent factor involved in the intrinsic pathway [1]. * **Factor X (Stuart-Prower Factor):** A Vitamin K-dependent factor that marks the beginning of the common pathway [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic:** Remember the Vitamin K-dependent factors as **"1972"** (Factors **10, 9, 7, and 2**) plus **Protein C and Protein S** (anticoagulants). * **Warfarin Mechanism:** Warfarin inhibits **Vitamin K Epoxide Reductase (VKORC1)**, preventing the recycling of Vitamin K and thus inhibiting the synthesis of these specific factors. * **Factor VII** has the shortest half-life among these factors, which is why the Prothrombin Time (PT/INR) is the first to rise during Vitamin K deficiency or early Warfarin therapy [1]. * **Factor VIII** levels are notably **normal or elevated** in liver disease (since it is made in endothelium), helping clinicians differentiate liver failure from Vitamin K deficiency.

Q: Low serum iron and low serum ferritin is seen in which of the following conditions?

Iron deficiency anemia. The correct answer is **Iron deficiency anemia (IDA)**. This condition is characterized by a depletion of total body iron stores [2]. **1. Why Iron Deficiency Anemia is correct:** In IDA, the body’s iron stores are exhausted. **Serum Ferritin** is the most sensitive and specific initial marker for IDA as it directly reflects total body iron stores. When stores are depleted, ferritin levels drop (<15–30 ng/mL). Consequently, **Serum Iron** also decreases because there is insufficient iron to be released into the circulation for erythropoiesis. **2. Why the other options are incorrect:** * **Chronic Kidney Disease (Anemia of Chronic Disease):** Characterized by high **Hepcidin** levels which trap iron inside macrophages [4]. This leads to **low serum iron** [1] but **normal or high serum ferritin** (as it is an acute-phase reactant and stores are not actually empty). * **Sideroblastic Anemia:** This is a defect in heme synthesis leading to iron overload. It typically shows **high serum iron** and **high serum ferritin**, with characteristic ring sideroblasts in the bone marrow. * **Fanconi Anemia:** This is an inherited bone marrow failure syndrome (aplastic anemia). It involves a stem cell defect, not an iron metabolism issue. Iron studies are usually normal unless the patient has received multiple blood transfusions (leading to iron overload). **High-Yield Clinical Pearls for NEET-PG:** * **Best initial test for IDA:** Serum Ferritin (Low). * **Gold Standard test for IDA:** Bone marrow aspiration (Prussian blue staining showing absent iron stores). * **TIBC (Total Iron Binding Capacity):** This is the only parameter that **increases** in IDA; it decreases in Anemia of Chronic Disease [3]. * **Transferrin Saturation:** Decreased in both IDA and Anemia of Chronic Disease.

Q: In a patient with thrombotic thrombocytopenic purpura, all of the following are seen EXCEPT?

Coomb's positive. **Explanation:** Thrombotic Thrombocytopenic Purpura (TTP) is a microangiopathic hemolytic anemia (MAHA) characterized by the formation of small-vessel thrombi due to a deficiency in the **ADAMTS13** enzyme. This deficiency leads to uncleaved large von Willebrand factor (vWF) multimers, causing platelet aggregation. **1. Why "Coombs positive" is the correct answer (the "EXCEPT"):** The hemolysis in TTP is **mechanical**, not immune-mediated [1]. As RBCs pass through fibrin/platelet meshes in small vessels, they are physically sheared, forming **schistocytes** [1]. Since there are no antibodies directed against the RBC surface, the Direct Antiglobulin Test (Coombs test) is **negative**. A positive Coombs test would instead suggest Autoimmune Hemolytic Anemia (AIHA) or Evans Syndrome. **2. Why the other options are seen in TTP:** * **Anemia:** This is a hallmark feature. It is a microangiopathic hemolytic anemia (MAHA) characterized by low hemoglobin, high LDH, and schistocytes on peripheral smear [1]. * **Thrombocytopenia:** Extensive platelet consumption occurs during the formation of microthrombi, leading to low platelet counts (usually <50,000/µL) [2]. * **Neurologic features:** These occur due to microvascular occlusion in the CNS, manifesting as headaches, confusion, seizures, or focal deficits. **High-Yield Clinical Pearls for NEET-PG:** * **The Classic Pentad (FAT RN):** **F**ever, **A**nemia (MAHA), **T**hrombocytopenia, **R**enal failure, and **N**eurologic symptoms [1]. (Note: The full pentad is seen in <25% of patients; Anemia and Thrombocytopenia are the most consistent). * **Diagnosis:** Decreased **ADAMTS13** activity (<10%). * **Treatment:** **Plasmapheresis (Plasma Exchange)** is the gold standard. It removes the autoantibodies and replaces the ADAMTS13 enzyme. * **Contraindication:** Platelet transfusion is generally avoided as it may "fuel the fire" of thrombus formation.

Q: Splenomegaly is least likely associated with which of the following conditions?

Primary thrombocytosis. **Explanation:** The question tests the ability to differentiate between various **Myeloproliferative Neoplasms (MPNs)** based on their clinical presentation, specifically the degree of splenic involvement. **1. Why Primary Thrombocytosis (Essential Thrombocythemia) is the correct answer:** In Essential Thrombocythemia (ET), the primary pathology is the overproduction of platelets. While mild splenomegaly can occur in about 25–40% of patients, it is **least likely** to be a prominent feature compared to other MPNs. In fact, many patients with ET may eventually develop **splenic atrophy** or "autosplenectomy" due to repeated silent splenic infarctions caused by microvascular thrombosis. **2. Analysis of Incorrect Options:** * **Chronic Myeloid Leukemia (CML):** Splenomegaly is a hallmark feature, present in over 50–70% of cases. It is often massive, especially in the accelerated or blast phase. * **Polycythemia Vera (PV):** Splenomegaly is a common finding (approx. 70% of patients) due to extramedullary hematopoiesis and increased red cell mass. * **Idiopathic Myelofibrosis (Primary Myelofibrosis):** This condition is associated with the **most massive splenomegaly** among all MPNs. As the bone marrow becomes fibrotic, the spleen becomes the primary site of extramedullary hematopoiesis. **Clinical Pearls for NEET-PG:** * **Massive Splenomegaly (Spleen crossing midline/reaching iliac crest):** Remember the mnemonic **"M-C-I"** — **M**yelofibrosis, **C**ML, and **I**nfectious (Kala-azar/Malaria) or **I**nherited (Gaucher’s). * **JAK2 Mutation:** Present in >95% of PV cases and ~50% of ET and Myelofibrosis cases. * **Diagnostic Clue:** If a patient has a platelet count >600,000/µL but a **normal-sized spleen**, think of Essential Thrombocythemia over other MPNs.

Question 1

A 14-year-old presented with recurrent epistaxis along with characteristic skin and mucous membrane lesions. The lesions were seen at the dorsum of the tongue, oral cavity, buccal mucosa, lips, palate, and nasal mucosa. Lab findings revealed a normal coagulation profile. Which of the following drugs has been recently approved for this condition?

Accepted Answer

Bevacizumab

Answer

Ranibizumab

Answer

Transtuzumab

Answer

Sorafenib

Question 2

Waldenstrom's macroglobulinemia is seen in which of the following conditions?

Accepted Answer

Plasmacytic Lymphoma

Answer

Non-Hodgkin's Lymphoma (NHL)

Answer

Chronic Lymphocytic Leukemia (CLL)

Answer

None of the above

Question 3

Bone marrow transplantation is used as a treatment modality in all of the following conditions except:

Accepted Answer

Hemochromatosis

Answer

Adrenoleukodystrophy

Answer

Hurler's Syndrome

Answer

Osteopetrosis

Question 4

An 18-year-old man of Italian descent presents with a hypochromic microcytic anemia of 10 g/dL, characterized by anisocytosis, poikilocytosis, and target cells on peripheral blood smear. His WBC count is 9500/mL, platelet count is 240,000/mL, and reticulocyte count is 7%. The spleen is palpable 5 cm below the left costal margin. What is the most likely diagnosis?

Accepted Answer

Thalassemia minor

Answer

Sickle cell trait

Answer

HbS-C disease

Answer

Sideroblastic anemia

Question 5

Which of the following is NOT a Vitamin K-dependent clotting factor?

Accepted Answer

Factor VIII

Answer

Factor II

Answer

Factor IX

Answer

Factor X

Question 6

A 20-year-old female presents with a 2-year history of anemia and mild jaundice. Her peripheral smear shows spherocytes. What is the most appropriate investigation?

Accepted Answer

Coombs test

Answer

Reticulocyte count

Answer

Osmotic fragility test

Answer

Bone marrow aspiration

Question 7

A 64-year-old man developed persistent back pain. On examination, his BP is 120/80, pulse 70, respiratory rate is 15, and temp is 98.6 deg F. Physical findings include severe pallor and evidence of muscle wasting. Urinalysis shows 4+ protein, and microscopy reveals occasional broad and occasional granular casts. Lab studies include: Serum creatinine = 5.1 mg/dl, serum Na = 141 mEq/L, K = 5.6 mEq/L, chloride = 101 mEq/L, CO2 = 14 mEq/L, serum calcium = 11.7 mg/dl, and serum phosphorus = 6.0 mg/dl. What is the most likely etiology?

Accepted Answer

Multiple myeloma

Answer

Renovascular disease

Answer

Thrombotic renal disease

Answer

Systemic lupus erythematosus (SLE)

Question 8

Low serum iron and low serum ferritin is seen in which of the following conditions?

Accepted Answer

Iron deficiency anemia

Answer

Chronic kidney disease

Answer

Sideroblastic anemia

Answer

Fanconi anemia

Question 9

In a patient with thrombotic thrombocytopenic purpura, all of the following are seen EXCEPT?

Accepted Answer

Coomb's positive

Answer

Anemia

Answer

Neurologic features

Answer

Thrombocytopenia

Question 10

Splenomegaly is least likely associated with which of the following conditions?

Accepted Answer

Primary thrombocytosis

Answer

Chronic myeloid leukemia (CML)

Answer

Polycythemia ruba vera

Answer

Idiopathic myelofibrosis

Hematology — MCQs

Hematology — MCQs

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