Hematology Practice Questions

Q: What is the most common symptom of Henoch-Schönlein purpura?

Purpura. **Explanation:** Henoch-Schönlein Purpura (HSP), now commonly referred to as **IgA Vasculitis**, is the most common systemic vasculitis in children [1]. It is a small-vessel vasculitis characterized by the deposition of IgA-dominant immune complexes [1]. **1. Why Purpura is the correct answer:** **Purpura** is the hallmark of the disease and is present in **100% of cases** [1]. It typically presents as "palpable purpura" (raised, non-blanching lesions) distributed symmetrically over gravity-dependent areas, such as the lower extremities and buttocks [1]. While other symptoms are common, purpura is the defining clinical feature required for diagnosis. **2. Why the other options are incorrect:** * **Intussusception (A):** This is the most common *serious gastrointestinal complication* of HSP (specifically ileo-ileal), but it occurs in only about 1–5% of patients. * **Edema (C):** Subcutaneous edema (angioedema) is common in younger children (scalp, hands, feet), but it is less frequent than the skin rash itself. * **Vomiting (D):** This is a non-specific gastrointestinal symptom. While GI involvement (colicky pain) occurs in ~75% of cases, vomiting is a secondary feature and not the primary diagnostic sign. **High-Yield Clinical Pearls for NEET-PG:** * **Classic Triad:** Palpable purpura, arthritis/arthralgia, and abdominal pain [1]. * **Renal Involvement:** The most important prognostic factor is the severity of renal involvement (HSP nephritis), which histologically resembles **IgA Nephropathy (Berger’s disease)**. * **Trigger:** Often follows an **Upper Respiratory Tract Infection (URTI)**, frequently involving *Streptococcus* [1]. * **Platelet Count:** Characteristically **normal** (Non-thrombocytopenic purpura), which helps differentiate it from ITP.

Q: Thrombotic thrombocytopenic purpura involves which of the following phenomena, except?

High complement level. Thrombotic Thrombocytopenic Purpura (TTP) is a life-threatening hematological emergency caused by a deficiency of the metalloproteinase **ADAMTS13**. This deficiency leads to the persistence of ultra-large von Willebrand factor (vWF) multimers, which cause spontaneous platelet aggregation and microthrombi formation. **Why Option C is the correct answer:** TTP is primarily a disorder of platelet aggregation and is **not** mediated by the complement system. Therefore, complement levels (C3, C4) remain **normal**. In contrast, low complement levels are characteristic of conditions like Systemic Lupus Erythematosus (SLE) or certain types of glomerulonephritis. Note that *Atypical Hemolytic Uremic Syndrome (aHUS)* involves complement dysregulation, but classic TTP does not. **Analysis of incorrect options:** * **A. Microangiopathy:** TTP is a classic Microangiopathic Hemolytic Anemia (MAHA) [1]. The microthrombi in small vessels shear passing RBCs, leading to schistocyte formation. * **B. Neural dysfunction:** Fluctuating neurological symptoms (confusion, seizures, focal deficits) are a hallmark of the TTP pentad, caused by microthrombi in the cerebral vasculature. * **D. Intravascular hemolysis:** The mechanical destruction of RBCs by fibrin strands/microthrombi results in non-immune, intravascular hemolysis (elevated LDH, low haptoglobin, and indirect hyperbilirubinemia) [1]. **High-Yield Clinical Pearls for NEET-PG:** * **The Classic Pentad (FAT RN):** **F**ever, **A**nemia (MAHA), **T**hrombocytopenia, **R**enal failure, and **N**eurological deficits. * **Diagnosis:** Decreased ADAMTS13 activity (<10%). * **Peripheral Smear:** Presence of **Schistocytes** (helmet cells). * **Coagulation Profile:** PT, aPTT, and Fibrinogen are typically **normal** (distinguishes TTP from DIC). * **Treatment:** Emergency **Plasmapheresis (Plasma Exchange)** is the gold standard. Never delay treatment for ADAMTS13 results.

Q: ADAMTS13 is associated with which of the following conditions?

Thrombotic thrombocytopenic purpura (TTP). **Explanation:** **Correct Answer: A. Thrombotic thrombocytopenic purpura (TTP)** ADAMTS13 is a metalloprotease enzyme responsible for cleaving large **von Willebrand Factor (vWF) multimers** into smaller, less active fragments. In TTP, there is a deficiency of ADAMTS13 (either congenital or, more commonly, acquired via autoantibodies). This leads to the persistence of "ultra-large" vWF multimers, which cause spontaneous platelet aggregation and microthrombi formation. This results in the classic clinical pentad: microangiopathic hemolytic anemia (MAHA), thrombocytopenia [1], neurological symptoms, renal failure, and fever. **Incorrect Options:** * **B. Churg-Strauss Syndrome (EGPA):** This is a small-vessel vasculitis characterized by asthma, eosinophilia, and necrotizing granulomas. It is associated with **p-ANCA** (anti-MPO), not ADAMTS13. * **C. Wegener’s Granulomatosis (GPA):** This is a granulomatous vasculitis involving the respiratory tract and kidneys. It is strongly associated with **c-ANCA** (anti-PR3). * **D. Membranous Nephropathy:** This is a common cause of nephrotic syndrome in adults. The primary biomarker associated with the idiopathic form is the **PLA2R (Phospholipase A2 receptor) antibody**. **NEET-PG High-Yield Pearls:** * **The "FAT RN" Mnemonic:** Fever, Anemia (MAHA), Thrombocytopenia, Renal failure, Neurological symptoms (the TTP pentad). * **Diagnosis:** Look for **Schistocytes** (helmet cells) on peripheral smear and a **decreased ADAMTS13 activity level (<10%)**. * **Treatment:** The treatment of choice is **Plasmapheresis (Plasma Exchange)** to remove antibodies and replenish the enzyme. Steroids and Rituximab are used as adjuncts. * **Distinction:** Unlike DIC, PT and aPTT are typically **normal** in TTP.

Q: The NESTROFT test is used in the screening of which condition?

Thalassemia. **Explanation:** **NESTROFT** (Naked Eye Single Tube Red Cell Osmotic Fragility Test) is a simple, cost-effective, and rapid screening tool used primarily for **Thalassemia Minor (Trait)**. **Why Thalassemia is Correct:** In Thalassemia, there is a defect in globin chain synthesis leading to **hypochromic microcytic** red blood cells [1]. These cells have a high surface-area-to-volume ratio, making them more resistant to hemolysis in hypotonic solutions compared to normal RBCs. In the NESTROFT test, blood is added to a 0.36% buffered saline solution. If the solution remains **turbid** (meaning the line behind the tube is not visible), the test is positive, indicating decreased osmotic fragility characteristic of Thalassemia. **Why Other Options are Incorrect:** * **Autoimmune Hemolytic Anemia:** This is characterized by antibody-mediated destruction; screening usually involves the Coombs test, not osmotic fragility. * **Spherocytosis:** Hereditary Spherocytosis is characterized by **increased** osmotic fragility (cells burst easily). While an Osmotic Fragility Test (OFT) is used for diagnosis, NESTROFT specifically screens for the *resistance* seen in Thalassemia. * **G6PD Deficiency:** This is an enzyme defect. Screening is done using the Fluorescent Spot Test or Methemoglobin Reduction Test. **Clinical Pearls for NEET-PG:** * **Sensitivity:** NESTROFT has a high sensitivity (approx. 95-98%) making it an ideal mass screening tool in resource-limited settings. * **Confirmatory Test:** A positive NESTROFT must be followed by **Hb Electrophoresis** or HPLC (showing HbA2 > 3.5%) for confirmation of Thalassemia Trait. * **Differential:** Iron Deficiency Anemia (IDA) can also show a positive NESTROFT, but the turbidity is usually less marked than in Thalassemia.

Q: Maximum ESR is seen in which of the following conditions?

Multiple myeloma. The **Erythrocyte Sedimentation Rate (ESR)** is a non-specific marker of inflammation that measures the rate at which red blood cells (RBCs) settle in a tube of anticoagulated blood. **Why Multiple Myeloma is the correct answer:** In Multiple Myeloma, there is a malignant proliferation of plasma cells [1]. There is a massive production of monoclonal immunoglobulins (paraproteins). These positively charged proteins neutralize the negative surface charge (Zeta potential) of RBCs, which normally keeps them apart. This allows RBCs to stack together like coins, a phenomenon known as **Rouleaux formation**. These heavy aggregates settle much faster than individual cells, leading to an extremely high ESR, as paraproteinaemia is a known cause of an elevated ESR above 100 mm/hr [1]. **Analysis of Incorrect Options:** * **Congestive Heart Failure (CHF):** This condition is associated with a **decreased** ESR, primarily due to hemodilution and changes in plasma proteins. * **Polycythemia Vera:** An increase in the number of RBCs increases the internal friction (viscosity) of the blood, which physically hinders the settling process, leading to a **very low or zero ESR**. * **Sickle Cell Anemia:** The abnormal, rigid shape of sickled cells prevents them from forming Rouleaux stacks. Since they cannot aggregate effectively, the **ESR is characteristically low**. **High-Yield Clinical Pearls for NEET-PG:** * **Extreme ESR (>100 mm/hr):** Think of the "Big Three": Multiple Myeloma [1], Temporal Arteritis/Polymyalgia Rheumatica, and Metastatic Malignancy (or severe infections like Tuberculosis). * **Zero ESR:** Classically seen in Polycythemia vera, Afibrinogenemia, and severe Agammaglobulinemia. * **Factors increasing ESR:** Anemia (fewer RBCs to resist settling), pregnancy, and aging. * **Factors decreasing ESR:** Spherocytosis, Acanthocytosis (abnormal shapes), and Leukocytosis.

Q: A stem cell disorder affecting all three cell lines (platelets, RBCs, and leucocytes) is:

Paroxysmal nocturnal hemoglobinuria. ### Explanation **Correct Answer: C. Paroxysmal nocturnal hemoglobinuria (PNH)** **Why it is correct:** PNH is an acquired clonal **hematopoietic stem cell disorder**. It is caused by a somatic mutation in the **PIGA gene**, which is essential for synthesizing the GPI-anchor. This anchor attaches protective proteins (CD55 and CD59) to the cell surface. Without these anchors, cells are susceptible to complement-mediated destruction. Since the mutation occurs in a multipotent stem cell, it affects all three lineages: **RBCs** (leading to intravascular hemolysis), **Leucocytes** (leading to leukopenia), and **Platelets** (leading to thrombocytopenia and dysfunctional clotting). **Why the other options are incorrect:** * **A. Hemolytic anemia:** This is a broad category of disorders (like Hereditary Spherocytosis or AIHA) that primarily affects the Red Blood Cell lineage, not the stem cell or other cell lines [1]. * **B. Paroxysmal cold hemoglobinuria (PCH):** This is a rare autoimmune hemolytic anemia caused by the **Donath-Landsteiner antibody**. It is restricted to the destruction of RBCs following cold exposure and does not involve a stem cell defect [2]. * **C. Blackfan Diamond syndrome:** This is a congenital **pure red cell aplasia**. It specifically affects the erythroid progenitor cells, leading to anemia, but typically spares the platelets and leucocytes. **High-Yield Clinical Pearls for NEET-PG:** * **Triad of PNH:** Hemolytic anemia, Pancytopenia, and Venous thrombosis (often in unusual sites like the Budd-Chiari syndrome). * **Gold Standard Investigation:** Flow cytometry (shows absence of CD55 and CD59). * **Association:** PNH is closely linked with **Aplastic Anemia**; one can evolve into the other. * **Treatment of choice:** Eculizumab (a monoclonal antibody against Complement C5).

Q: A 60-year-old male presents with generalized bone pain. On examination, there is an elevated ESR of 100 mm/hr, serum globulin of 7 g/dL, lytic lesions in the skull, serum creatinine of 3.5 mg/dL, and serum calcium of 11 mg/dL. What is the most likely diagnosis?

Multiple myeloma. The clinical presentation is a classic case of **Multiple Myeloma (MM)**, a neoplastic proliferation of plasma cells [1]. The diagnosis is confirmed by the **CRAB** criteria (Calcium elevation, Renal insufficiency, Anemia, and Bone lesions), all of which are present here: * **Bone Pain & Lytic Lesions:** Plasma cells produce OAFs (Osteoclast Activating Factors), leading to "punched-out" lytic lesions (skull) and bone pain [1]. * **Renal Insufficiency:** Serum creatinine of 3.5 mg/dL indicates "Myeloma Kidney," caused by the precipitation of Bence-Jones proteins (light chains) in the tubules. * **Hyperglobulinemia & High ESR:** The monoclonal (M) protein spike leads to a reversal of the Albumin:Globulin ratio and causes "Rouleaux formation," which significantly elevates the ESR [1]. **Why other options are incorrect:** * **Waldenstrom’s Macroglobulinemia:** Characterized by IgM paraprotein and hyperviscosity [1]. Crucially, it **lacks** lytic bone lesions and hypercalcemia. * **Hyperparathyroidism:** While it causes hypercalcemia and bone resorption (osteitis fibrosa cystica), it does not explain the massive hyperglobulinemia (7 g/dL) or the extremely high ESR. * **Osteomalacia:** This involves defective mineralization of the bone matrix, typically presenting with **low** calcium and phosphate, not lytic lesions or renal failure. **NEET-PG High-Yield Pearls:** * **Most common initial symptom:** Bone pain (back/ribs). * **Best initial test:** Serum Protein Electrophoresis (shows M-spike) [1]. * **Gold standard:** Bone marrow biopsy (>10% clonal plasma cells) [1]. * **Note:** Bone scans are often negative in MM because they detect osteoblastic activity; **Skeletal Survey (X-ray)** or MRI is preferred to find lytic lesions [1].

Q: A 45-year-old lady diagnosed to have anemia presents with Hb of 7.8 g/dL and MCV of 72 fL. Her serum ferritin is 8 ng/mL. After one month of adequate iron therapy, her Hb is still 8.0 g/dL. What is the most probable cause for treatment failure?

Non-compliance. **Explanation:** The patient presents with **Microcytic Hypochromic Anemia** (Hb 7.8 g/dL, MCV 72 fL) and a low serum ferritin (8 ng/mL), which is the most specific biochemical marker for **Iron Deficiency Anemia (IDA)**. [1] In a patient with confirmed IDA, oral iron therapy typically results in a reticulocyte count peak within 7–10 days and a rise in Hemoglobin (Hb) of approximately **1–2 g/dL every 2–3 weeks**. After one month of "adequate" therapy, this patient’s Hb has remained stagnant (7.8 to 8.0 g/dL). **1. Why Non-compliance is the Correct Answer:** Statistically, **non-compliance** is the most common cause of failure to respond to oral iron therapy. Oral iron (especially ferrous sulfate) frequently causes gastrointestinal side effects like nausea, epigastric pain, constipation, or metallic taste, leading many patients to discontinue the medication or take it irregularly. **2. Why other options are incorrect:** * **Acquired Sideroblastic Anemia:** This would typically present with *increased* serum ferritin (iron overload) and ring sideroblasts in the bone marrow, not the low ferritin seen here. * **Inadequate intake of iron:** While a cause of IDA, the question states the patient was put on "adequate iron therapy." [1] Failure to respond to prescribed treatment points toward compliance or absorption issues rather than dietary intake. * **Folate deficiency:** This causes Macrocytic anemia (high MCV), which contradicts the microcytic (low MCV) picture provided. [2] **Clinical Pearls for NEET-PG:** * **First sign of response to iron:** Increase in Reticulocyte count (5–10 days). * **Most specific test for IDA:** Serum Ferritin (<15 ng/mL). * **Gold Standard for IDA:** Bone marrow iron stores (Perl’s Prussian Blue stain)—though rarely done. * **Malabsorption:** If compliance is confirmed but Hb still doesn't rise, consider Celiac disease or *H. pylori* infection.

Question 1

What is the most common symptom of Henoch-Schönlein purpura?

Accepted Answer

Purpura

Answer

Intussusception

Answer

Edema

Answer

Vomiting

Question 2

Thrombotic thrombocytopenic purpura involves which of the following phenomena, except?

Accepted Answer

High complement level

Answer

Microangiopathy

Answer

Neural dysfunction

Answer

Intravascular hemolysis

Question 3

ADAMTS13 is associated with which of the following conditions?

Accepted Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Churg-Strauss syndrome

Answer

Wegener's granulomatosis

Answer

Membranous nephropathy

Question 4

The NESTROFT test is used in the screening of which condition?

Accepted Answer

Thalassemia

Answer

Autoimmune hemolytic anemia

Answer

Spherocytosis

Answer

G6PD deficiency

Question 5

Which of the following conditions is most likely to present with massive splenomegaly and pancytopenia?

Accepted Answer

Myelofibrosis

Answer

Chronic Lymphocytic Leukemia (CLL)

Answer

Pure red cell aplasia

Answer

Chronic Myeloid Leukemia (CML)

Question 6

Maximum ESR is seen in which of the following conditions?

Accepted Answer

Multiple myeloma

Answer

Congestive Heart Failure (CHF)

Answer

Polycythemia vera

Answer

Sickle cell anemia

Question 7

A four-year-old boy presented with a two-month history of abdominal pain and fever, a ten-day history of maculopapular rash, and three days of dry cough, dyspnea, and wheezing. On examination, the liver and spleen were enlarged, measuring 4 cm and 3 cm below the costal margin, respectively. His hemoglobin was 10.0 g/dL, platelet count was 3.7 x 10^5/µL, and total leukocyte count was 70 x 10^3/µL, with 80% eosinophils. Bone marrow examination revealed a cellular marrow with 45% blasts, 34% eosinophils, and eosinophilic precursors. The blasts stained negative for myeloperoxidase and non-specific esterase and were positive for CD19, CD10, CD22, and CD20. Which one of the following statements is not true about the disease?

Accepted Answer

Inv(16) is often detected in the blasts and the eosinophils.

Answer

Eosinophils are not part of the neoplastic clone.

Answer

t(5;14) rearrangement may be detected in the blasts.

Answer

Peripheral blood eosinophilia may normalize with chemotherapy.

Question 8

A stem cell disorder affecting all three cell lines (platelets, RBCs, and leucocytes) is:

Accepted Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Hemolytic anemia

Answer

Paroxysmal cold hemoglobinuria

Answer

Blackfan Diamond syndrome

Question 9

A 60-year-old male presents with generalized bone pain. On examination, there is an elevated ESR of 100 mm/hr, serum globulin of 7 g/dL, lytic lesions in the skull, serum creatinine of 3.5 mg/dL, and serum calcium of 11 mg/dL. What is the most likely diagnosis?

Accepted Answer

Multiple myeloma

Answer

Waldenstrom's macroglobulinemia

Answer

Hyperparathyroidism

Answer

Osteomalacia

Question 10

A 45-year-old lady diagnosed to have anemia presents with Hb of 7.8 g/dL and MCV of 72 fL. Her serum ferritin is 8 ng/mL. After one month of adequate iron therapy, her Hb is still 8.0 g/dL. What is the most probable cause for treatment failure?

Accepted Answer

Non-compliance

Answer

Acquired sideroblastic anemia

Answer

Inadequate intake of iron

Answer

Folate deficiency

Hematology — MCQs

Hematology — MCQs

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