Hematology Practice Questions

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.

Q: A 45-year-old female patient presents with symptoms of easy bruisability and frequent headaches. Physical examination shows moderate splenomegaly. Blood counts show a normal leucocyte count and a platelet count of 1000 × 10^3/cu mm. The leucocyte alkaline phosphatase score is normal. Which one of the following is the drug of choice for the treatment of this patient?

Hydroxyurea. The patient presents with a significantly elevated platelet count (1000 × 10³/cu mm), splenomegaly, and symptoms of vasomotor instability (headaches) and bleeding (bruisability). Given the normal leucocyte count and normal LAP score (which rules out CML), the most likely diagnosis is **Essential Thrombocythemia (ET)**. **1. Why Hydroxyurea is the Correct Answer:** Hydroxyurea is the **first-line myelosuppressive agent** for high-risk ET patients (defined as age >60 years or a history of thrombosis/bleeding). It effectively lowers the platelet count, reduces the risk of thrombotic events, and is generally well-tolerated. In this patient, the presence of symptoms and a very high platelet count necessitates cytoreductive therapy, and Hydroxyurea remains the gold standard. **2. Why the Other Options are Incorrect:** * **Radioactive Phosphorus (P³²):** While effective, it is associated with a high risk of transformation into Acute Myeloid Leukemia (AML) and is now rarely used. * **Anagrelide:** This is a second-line agent. While it specifically lowers platelets, it is less effective than Hydroxyurea in preventing arterial thrombosis and carries side effects like palpitations and fluid retention. * **Interferon-alpha:** Usually reserved for pregnant patients or those refractory to Hydroxyurea due to its significant side-effect profile (flu-like symptoms, depression). **Clinical Pearls for NEET-PG:** * **Diagnosis of ET:** Requires platelets >450 × 10³/cu mm and the presence of a driver mutation (**JAK2 V617F** in ~55%, **CALR**, or **MPL**). * **LAP Score:** Used to differentiate Leukemoid reaction (High) from CML (Low). A normal score here points away from CML. * **Treatment Goal:** In ET, the goal is to maintain platelets <400 × 10³/cu mm to prevent thrombosis and paradoxical bleeding (due to acquired von Willebrand syndrome at very high platelet counts).

Q: For what procedure is the instrument depicted in the diagram used?

Bone marrow biopsy. ***Bone marrow biopsy*** - The **Jamshidi needle** depicted has a distinctive **tapered bore** that is wider at the tip with a **cutting edge**, specifically designed to obtain bone marrow core samples from the **iliac crest** or sternum. - Features a **removable stylet** and sturdy construction to penetrate cortical bone and extract intact marrow specimens for histological examination. *Pleural biopsy* - Requires specialized needles like the **Abrams needle** or **Cope needle** with a **notched cutting mechanism** to sample pleural tissue. - These instruments have a **hook-like design** with a cutting chamber, completely different from the straight, tapered Jamshidi needle. *Kidney biopsy* - Utilizes **Tru-Cut needles** or **automated core biopsy guns** with spring-loaded mechanisms for precise tissue sampling. - These needles have **smaller gauge** (14-18G) and **automated firing systems**, unlike the manual, larger-bore Jamshidi needle. *Liver biopsy* - Performed with **Menghini needles** or **Vim-Silverman needles** that have **suction capability** and thinner profiles. - These instruments feature **aspiration mechanisms** and are designed for softer tissue penetration, not the bone-cutting capability of the Jamshidi needle.

Question 1

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 2

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 3

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 4

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 5

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 6

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 7

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

Question 8

A 45-year-old female patient presents with symptoms of easy bruisability and frequent headaches. Physical examination shows moderate splenomegaly. Blood counts show a normal leucocyte count and a platelet count of 1000 × 10^3/cu mm. The leucocyte alkaline phosphatase score is normal. Which one of the following is the drug of choice for the treatment of this patient?

Accepted Answer

Hydroxyurea

Answer

Radioactive phosphorus

Answer

Anagrelide

Answer

Interferon alpha

Question 9

For what procedure is the instrument depicted in the diagram used?

Accepted Answer

Bone marrow biopsy

Answer

Pleural biopsy

Answer

Kidney biopsy

Answer

Liver biopsy

Question 10

Which condition is associated with Coomb's positive hemolytic anemia?

Accepted Answer

Systemic lupus erythematosus (SLE)

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Polyarteritis nodosa (PAN)

Answer

Hemolytic uremic syndrome (HUS)

Hematology — MCQs

Hematology — MCQs

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