Hematology Practice Questions

Q: An otherwise healthy 1-year-old child has a routine CBC revealing polymorphonuclear neutrophils as shown below. Which of the following is the most appropriate next step?

Begin folic acid supplementation. ***Begin folic acid supplementation*** - **Hypersegmented neutrophils** (PMNs with ≥5 lobes) are the hallmark finding of **megaloblastic anemia**, indicating folate or B12 deficiency. - In a **1-year-old infant**, **folate deficiency** is the most common cause of megaloblastic anemia, making folic acid supplementation the appropriate first-line treatment. *Bone marrow aspiration* - This invasive procedure is unnecessary when **hypersegmented neutrophils** already provide clear evidence of megaloblastic anemia. - Bone marrow aspiration would only be considered if there were concerns about **malignancy** or other hematologic disorders, which is not indicated here. *Begin oral iron therapy* - **Iron deficiency anemia** presents with **microcytic hypochromic** red blood cells, not hypersegmented neutrophils. - Iron therapy would not address the underlying **folate deficiency** causing the megaloblastic changes observed. *Hemoglobin electrophoresis* - This test is used to diagnose **hemoglobinopathies** like sickle cell disease or thalassemia, not megaloblastic anemia. - **Hypersegmented neutrophils** are not associated with hemoglobin variants and would not be corrected by identifying hemoglobinopathies.

Q: A 5-year-old child presents with a history of intermittent fever for the past 2 weeks, petechial spots all over the body, and increasing pallor for the past 1 month. Examination reveals splenomegaly 2 cm below the costal margin. What is the most likely diagnosis?

Acute leukemia. ### Explanation The clinical presentation of **fever, pallor, and petechiae** represents the classic triad of **pancytopenia** (anemia, leukopenia/neutropenia, and thrombocytopenia). In a pediatric patient, this triad combined with **organomegaly** (splenomegaly) is highly suggestive of **Acute Leukemia** (most commonly ALL in this age group). **Why Acute Leukemia is correct:** Leukemic cells infiltrate the bone marrow, leading to "marrow failure." This results in decreased production of normal RBCs (pallor), WBCs (fever/infections), and platelets (petechiae). Crucially, the presence of **splenomegaly** indicates an infiltrative process or extramedullary hematopoiesis, which distinguishes it from simple marrow failure like aplastic anemia. **Why the other options are incorrect:** * **Idiopathic Thrombocytopenic Purpura (ITP):** Typically presents with isolated thrombocytopenia (petechiae/bruising) in a "well-looking" child. It does **not** cause pallor, fever, or splenomegaly. * **Aplastic Anemia:** While it presents with pancytopenia (pallor, fever, petechiae), it is characterized by an "empty" marrow. Therefore, **splenomegaly is characteristically absent**. * **Hypersplenism:** While it can cause cytopenias due to splenic sequestration, the primary cause of the splenomegaly would usually be more prominent, and it rarely presents with such acute, severe systemic symptoms in a 5-year-old without a known prior history (like portal hypertension or thalassemia). **Clinical Pearls for NEET-PG:** * **Acute Lymphoblastic Leukemia (ALL)** is the most common childhood malignancy (peak age 2–5 years). * **Bone pain** is a frequent additional finding in pediatric leukemia due to marrow expansion. * **Key Diagnostic Step:** Bone marrow aspiration showing **>20% blasts**. * **Differentiating Point:** Pancytopenia + Splenomegaly = Think Leukemia; Pancytopenia + No Splenomegaly = Think Aplastic Anemia.

Q: What is true about Immune Thrombocytopenic purpura (ITP)?

Intracranial bleed is a rare complication.. **Explanation:** Immune Thrombocytopenic Purpura (ITP) is an acquired hemorrhagic disorder caused by the immune-mediated destruction of platelets. **1. Why Option A is correct:** Intracranial hemorrhage (ICH) is the most dreaded but **rare** complication of ITP, occurring in less than **0.1% to 0.5%** of cases. It typically occurs when the platelet count falls below 10,000/mm³. Because the primary defect is in platelet number (not function) and the remaining platelets are often young and hyperfunctional, spontaneous major bleeding is uncommon despite very low counts. **2. Why the other options are incorrect:** * **Option B:** Splenomegaly is **not** a feature of ITP. The spleen is the site of platelet destruction, but it does not typically enlarge. If significant splenomegaly is present, clinicians must investigate alternative diagnoses like leukemia or hypersplenism. * **Option C:** In children (Acute ITP), there is **no gender predilection** (Male = Female). A female preponderance is only seen in the chronic form, typically in adolescents and adults. * **Option D:** While Acute ITP is indeed self-limiting (resolving within 6 months in 80% of children), **Option A is the more definitive "classic" teaching point** regarding complications in standard pediatric textbooks (like Nelson). *Note: In many exam formats, D is also considered a true statement, but A is the high-yield clinical fact prioritized in NEET-PG.* **Clinical Pearls for NEET-PG:** * **Trigger:** Often follows a viral infection (e.g., URTI) by 1–4 weeks. * **Bone Marrow:** Shows increased or normal megakaryocytes (compensatory). * **Treatment:** Observation is preferred if bleeding is minimal. If treatment is needed (platelets 12 months.

Q: A child presents with Hb of 6.5 gm%, MCV of 65, MCH of 15, and protoporphyria with a much reduced red cell distribution width. What is the most likely diagnosis?

Iron deficiency anemia. This question tests your ability to differentiate between microcytic hypochromic anemias using red cell indices and biochemical markers. ### **Explanation of the Correct Answer** The patient presents with **Iron Deficiency Anemia (IDA)**. * **Microcytic Hypochromic Picture:** Hb 6.5 g%, MCV 65 fL (low), and MCH 15 pg (low) indicate microcytic hypochromic anemia. * **Protoporphyria:** In IDA, iron is unavailable to combine with protoporphyrin to form Heme. This leads to an accumulation of **Free Erythrocyte Protoporphyrin (FEP)**, a classic biochemical marker for IDA. * **RDW (Red Cell Distribution Width):** While the question mentions a "reduced" RDW, it is important to note that in clinical practice, IDA typically shows an **increased RDW** (anisocytosis). However, among the given options, the combination of microcytosis and elevated protoporphyrin is pathognomonic for IDA. ### **Why Other Options are Incorrect** * **Thalassemia:** While it presents with a low MCV and very low RDW (Mentzer index <13), **protoporphyrin levels are normal** because there is no defect in heme synthesis; the defect lies in globin chain production. * **Porphyria:** These are enzymatic defects in the heme synthesis pathway. While they involve protoporphyrins, they do not typically present with this specific microcytic blood picture or low iron indices. * **Megaloblastic Anemia:** This is a **macrocytic** anemia (MCV >100 fL), which contradicts the provided MCV of 65. ### **NEET-PG High-Yield Pearls** 1. **Mentzer Index:** MCV/RBC count. If ** 13**, suspect IDA. 2. **RDW:** The earliest sign of IDA is an increase in RDW. 3. **Gold Standard:** Bone marrow aspiration (Perl’s Prussian blue stain) is the gold standard for IDA, but **Serum Ferritin** is the best initial screening test. 4. **FEP:** Elevated in IDA and Lead Poisoning, but normal in Thalassemia.

Q: Autosplenectomy is seen in which of the following conditions?

Sickle cell disease. **Explanation:** **1. Why Sickle Cell Disease (SCD) is correct:** Autosplenectomy is a hallmark of **Sickle Cell Anemia (HbSS)**. It occurs due to repeated episodes of splenic sequestration and micro-infarctions. In SCD, deoxygenated hemoglobin polymerizes, causing RBCs to "sickle." These rigid cells get trapped in the narrow splenic sinusoids, leading to vaso-occlusion. Over time, recurrent ischemic injury leads to progressive fibrosis and shrinkage of the spleen. By age 5–8 years, the spleen becomes a small, shrunken, and non-functional fibrous remnant. **2. Why other options are incorrect:** * **Beta-thalassemia:** This condition typically presents with **splenomegaly** (enlarged spleen) due to extramedullary hematopoiesis and increased clearance of abnormal RBCs. * **Chronic Myeloid Leukemia (CML):** This is characterized by **massive splenomegaly** (often crossing the midline) due to the infiltration of the spleen by neoplastic myeloid cells. **3. Clinical Pearls for NEET-PG:** * **Howell-Jolly Bodies:** The presence of these nuclear remnants on a peripheral smear is a classic sign of functional asplenia/autosplenectomy. * **Infection Risk:** Patients with autosplenectomy are highly susceptible to **encapsulated organisms** (*Streptococcus pneumoniae*, *Haemophilus influenzae*, and *Neisseria meningitidis*). Prophylactic penicillin and vaccinations are mandatory. * **Radiology:** On X-ray or CT, a "calcified spleen" may be visible in late stages of SCD. * **Exception:** In **Sickle-Thalassemia (HbS-βThal)** or **HbSC disease**, splenomegaly may persist into adulthood, and autosplenectomy occurs much later or not at all.

Q: A 15-year-old boy presented with a one-day history of bleeding gums, subconjunctival bleed, and purpuric rash. Investigations revealed the following results: Hb - 6.4 gm/dL; TLC - 26,500/mm3; Platelets - 35,000/mm3; Prothrombin time - 20 sec (control 13 sec); Partial thromboplastin time - 50 sec; and Fibrinogen - 10 mg/dL. Peripheral smear was suggestive of acute myeloblastic leukemia. Which of the following is the most likely diagnosis?

Promyelocytic leukemia. **Explanation:** The clinical presentation of a young patient with sudden onset bleeding (gums, subconjunctival, purpura) and laboratory evidence of **Disseminated Intravascular Coagulation (DIC)** is the hallmark of **Acute Promyelocytic Leukemia (APL)**, classified as AML-M3. **Why Option C is correct:** The key to this diagnosis is the combination of leukocytosis and severe coagulopathy. In APL, the promyelocytes contain numerous **procoagulant-rich granules** (and multiple Auer rods). When these cells lyse, they release tissue factor-like substances and plasminogen activators, triggering a massive consumption of clotting factors. This is reflected in the patient's labs: prolonged PT/aPTT and **profoundly low Fibrinogen (10 mg/dL)**. **Why other options are incorrect:** * **Options A (M1) and B (M2):** While these are common forms of AML, they typically present with features of bone marrow failure (anemia, infections) but are rarely associated with primary DIC at presentation. * **Option D (M4):** Myelomonocytic leukemia often presents with gum hypertrophy and extramedullary involvement (skin/CNS), but DIC is not its defining initial feature. **High-Yield Clinical Pearls for NEET-PG:** * **Cytogenetics:** Associated with **t(15;17)**, involving the PML-RARA fusion gene. * **Morphology:** Presence of **"Faggot cells"** (cells with bundles of Auer rods). * **Treatment:** Medical emergency! Start **ATRA (All-Trans Retinoic Acid)** immediately to induce differentiation of promyelocytes and resolve coagulopathy. * **Complication:** Watch for **Differentiation Syndrome** (fever, respiratory distress, pulmonary infiltrates) during ATRA therapy.

Question 1

An 18-month-old boy presented with a tender, swollen, warm right knee with significant hemarthrosis after an injury. His PT is 12 seconds (normal, 13 seconds), PTT is over 100 seconds (normal, 25 seconds), platelet count is 300,000/mm
3, and bleeding time is normal. What should be the initial management?

Accepted Answer

Factor VIII concentrate

Answer

Fresh-frozen plasma

Answer

Aspiration of knee

Answer

Passive exercise

Question 2

An otherwise healthy 1-year-old child has a routine CBC revealing polymorphonuclear neutrophils as shown below. Which of the following is the most appropriate next step?

Accepted Answer

Begin folic acid supplementation

Answer

Bone marrow aspiration

Answer

Begin oral iron therapy

Answer

Hemoglobin electrophoresis

Question 3

A 5-year-old child presents with a history of intermittent fever for the past 2 weeks, petechial spots all over the body, and increasing pallor for the past 1 month. Examination reveals splenomegaly 2 cm below the costal margin. What is the most likely diagnosis?

Accepted Answer

Acute leukemia

Answer

Idiopathic thrombocytopenic purpura

Answer

Hypersplenism

Answer

Aplastic anemia

Question 4

What is true about Immune Thrombocytopenic purpura (ITP)?

Accepted Answer

Intracranial bleed is a rare complication.

Answer

Splenomegaly is prominent.

Answer

It is more common in females.

Answer

Acute ITP is a self-limiting condition.

Question 5

A child presents with Hb of 6.5 gm%, MCV of 65, MCH of 15, and protoporphyria with a much reduced red cell distribution width. What is the most likely diagnosis?

Accepted Answer

Iron deficiency anemia

Answer

Thalassemia

Answer

Porphyria

Answer

Megaloblastic anemia

Question 6

Autosplenectomy is seen in which of the following conditions?

Accepted Answer

Sickle cell disease

Answer

Beta-thalassemia

Answer

Chronic myeloid leukemia

Answer

All of the above

Question 7

Diamond Blackfan anemia is another name for which of the following conditions?

Accepted Answer

Inherited erythroid aplasia

Answer

Inherited thrombocytopenia

Answer

Inherited leucopenia

Answer

All of the above

Question 8

In a newborn with ABO incompatibility, what is the characteristic feature observed on the peripheral smear?

Accepted Answer

Microspherocytes

Answer

Fragmented RBCs

Answer

Polychromasia

Answer

Elliptocytosis

Question 9

Which finding is abnormal in the classic presentation of hemorrhagic disease of the newborn?

Accepted Answer

Thrombin time

Answer

Platelet count

Answer

Fibrinogen level

Answer

Activated Partial Thromboplastin Time (APTT)

Question 10

A 15-year-old boy presented with a one-day history of bleeding gums, subconjunctival bleed, and purpuric rash. Investigations revealed the following results: Hb - 6.4 gm/dL; TLC - 26,500/mm3; Platelets - 35,000/mm3; Prothrombin time - 20 sec (control 13 sec); Partial thromboplastin time - 50 sec; and Fibrinogen - 10 mg/dL. Peripheral smear was suggestive of acute myeloblastic leukemia. Which of the following is the most likely diagnosis?

Accepted Answer

Promyelocytic leukemia

Answer

Myeloblastic leukemia without maturation

Answer

Myeloblastic leukemia with maturation

Answer

Myelomonocytic leukemia

Hematology — MCQs

Hematology — MCQs

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