Hematology — MCQs

Hematology Indian Medical PG Practice Questions and MCQs

Question 21: A 21-year-old male presents with anemia and mild hepatosplenomegaly. His hemoglobin is 5 g/dL and he has a history of a single blood transfusion. What is the most probable diagnosis?

A. Thalassemia major
B. Thalassemia minor
C. Thalassemia intermedia
D. Autoimmune hemolytic anemia (Correct Answer)

Explanation: **Explanation:** The correct answer is **Autoimmune Hemolytic Anemia (AIHA)**. The key to this question lies in the clinical presentation versus the patient's age and transfusion history. **1. Why Autoimmune Hemolytic Anemia is correct:** The patient is 21 years old with severe anemia (Hb 5 g/dL) and hepatosplenomegaly, yet he has a history of only **one** blood transfusion. In chronic hereditary hemolytic anemias like Thalassemia Major, a patient would not survive until age 21 with such severe anemia without regular, lifelong transfusions. AIHA can present acutely at any age with severe hemolysis and compensatory organomegaly, explaining the low hemoglobin despite a lack of chronic transfusion dependence. **2. Why the other options are incorrect:** * **Thalassemia Major:** This typically presents in infancy (6–9 months). Without regular monthly transfusions, these patients develop severe skeletal deformities and growth failure, and rarely survive into their 20s with a hemoglobin of 5 g/dL. * **Thalassemia Minor:** This is usually asymptomatic or presents with mild anemia (Hb typically >10 g/dL). It does not cause severe anemia (5 g/dL) or significant hepatosplenomegaly. * **Thalassemia Intermedia:** While these patients are "transfusion-independent," they usually maintain a hemoglobin between 7–10 g/dL. A hemoglobin of 5 g/dL in an adult would be unusually low for Intermedia unless triggered by an infection or aplastic crisis. **Clinical Pearls for NEET-PG:** * **Thalassemia Major:** "Transfusion-dependent"; look for "Chipmunk facies" and "Hair-on-end" appearance on X-ray. * **Thalassemia Intermedia:** "Transfusion-independent"; presents later than Major but with similar (though milder) features. * **AIHA Diagnosis:** The gold standard test is the **Direct Coombs Test (Direct Antiglobulin Test)**. * **Rule of Thumb:** If a young adult has severe anemia but a near-absent transfusion history, think of acquired causes (AIHA) or milder genetic variants (Intermedia) rather than Thalassemia Major.

Question 22: What type of transfusion is ideally recommended for children with thalassemia?

A. Packed RBC
B. Platelet rich plasma
C. Saline washed packed RBC (Correct Answer)
D. Whole blood

Explanation: **Explanation:** The primary goal of transfusion in Thalassemia Major is to maintain hemoglobin levels (typically >9.5 g/dL) to suppress ineffective erythropoiesis and ensure normal growth. **Why Saline Washed Packed RBCs are preferred:** Thalassemia patients require lifelong, chronic transfusions. This puts them at a high risk for **Febrile Non-Hemolytic Transfusion Reactions (FNHTR)** and allergic reactions caused by donor plasma proteins and leucocytes. * **Saline washing** removes 99% of plasma proteins, antibodies, and cytokines. * It significantly reduces the risk of allergic reactions and sensitization to HLA antigens. * *Note:* While **Leucoreduced RBCs** (using 3rd generation filters) are now the gold standard globally to prevent FNHTR and CMV transmission, "Saline washed RBCs" remains a classic and correct answer in the context of preventing plasma-mediated allergic reactions in multi-transfused patients. **Analysis of Incorrect Options:** * **A. Packed RBC:** While better than whole blood due to less volume overload, standard packed RBCs still contain plasma proteins and leucocytes that can cause reactions in chronic recipients. * **B. Platelet Rich Plasma:** Thalassemia is a disorder of hemoglobin synthesis, not a platelet deficiency. This is clinically irrelevant. * **D. Whole Blood:** Contraindicated because it causes **volume overload** (congestive heart failure) and provides unnecessary plasma and electrolytes, increasing the risk of iron overload and transfusion reactions. **High-Yield Clinical Pearls for NEET-PG:** * **Iron Overload:** The most common complication of chronic transfusion. Chelation therapy (e.g., Deferasirox, Deferiprone) is usually started after 10–20 transfusions or when Serum Ferritin >1000 ng/ml. * **Target Hb:** Pre-transfusion Hb should be maintained between **9.5–10.5 g/dL**. * **Neocytes:** Transfusing younger RBCs (neocytes) can theoretically increase the interval between transfusions, though it is technically difficult.

Question 23: What is the approximate percentage of Hemoglobin F (HbF) at birth?

A. 20%
B. 50%
C. 70% (Correct Answer)
D. 90%

Explanation: **Explanation:** **Understanding the Concept:** Hemoglobin F (HbF, $\alpha_2\gamma_2$) is the predominant hemoglobin during fetal life due to its high affinity for oxygen, which facilitates oxygen transfer from maternal blood across the placenta. The switch from fetal ($\gamma$-globin) to adult ($\beta$-globin) synthesis begins around 30 weeks of gestation. At the time of birth, a full-term neonate typically has **70% to 80% HbF**, with the remaining 20–30% being adult hemoglobin (HbA). **Analysis of Options:** * **Option C (70%) is Correct:** This represents the standard physiological level of HbF in a term neonate. * **Option A (20%) & B (50%):** These levels are too low for a newborn. HbF levels drop to approximately 50% by 1 month of age and reach adult levels (<1%) by 6–12 months. * **Option D (90%):** While HbF is nearly 90–95% at 30 weeks gestation, the progressive switch to HbA reduces this to ~70% by the time the baby is born at term. **NEET-PG High-Yield Pearls:** 1. **The "Switch":** HbF levels decline rapidly after birth. By **6 months of age**, HbF usually drops to **<5%**. 2. **Clinical Significance:** Beta-hemoglobinopathies (like **Beta-Thalassemia Major** and **Sickle Cell Anemia**) do not manifest clinically until 6 months of age, as the protective effect of HbF wanes and the defective HbA production becomes dominant. 3. **Structure:** HbF consists of two alpha ($\alpha$) and two gamma ($\gamma$) chains. 4. **P50 Value:** HbF has a lower P50 (approx. 19 mmHg) compared to HbA (approx. 27 mmHg), reflecting its higher oxygen affinity.

Question 24: "Chipmunk face" is characteristically seen in?

A. Beta thalassemia major (Correct Answer)
B. Fetal alcohol syndrome
C. Cushing's syndrome
D. Acromegaly

Explanation: **Explanation:** **Beta Thalassemia Major (Correct Answer):** The "Chipmunk face" (or Thalassemic facies) is a classic manifestation of **ineffective erythropoiesis** and chronic hemolytic anemia. In Beta Thalassemia Major, the body attempts to compensate for severe anemia by expanding the bone marrow (erythroid hyperplasia). This **extramedullary hematopoiesis** causes the marrow cavities of the facial and cranial bones to expand, leading to: * Prominent cheekbones (malar prominence). * Frontal bossing. * Depressed nasal bridge. * Protrusion of the upper jaw (maxillary hypertrophy) with malocclusion of teeth. On X-ray, this marrow expansion in the skull gives the characteristic **"Hair-on-end" appearance**. **Why other options are incorrect:** * **Fetal Alcohol Syndrome:** Characterized by a smooth philtrum, thin upper lip, and short palpebral fissures, but not bony overgrowth. * **Cushing’s Syndrome:** Presents with a **"Moon face"** due to redistribution of adipose tissue (fat), not bony changes. * **Acromegaly:** Features frontal bossing and a prominent jaw (**prognathism**) due to excess Growth Hormone in adults, but it is not described as "chipmunk-like." **High-Yield Clinical Pearls for NEET-PG:** * **Management:** Regular blood transfusions are required, which can lead to **secondary hemosiderosis** (iron overload). * **Iron Chelation:** Usually started when ferritin levels exceed 1000 ng/ml or after 10–20 transfusions. * **Hb Electrophoresis:** Shows absence or marked reduction of HbA, with significantly elevated **HbF**.

Question 25: Increased fetal hemoglobin is seen in which of the following conditions?

A. Juvenile chronic myeloid leukemia (CML) (Correct Answer)
B. Congenital red cell aplasia
C. Hereditary spherocytosis
D. Acute myeloid leukemia (AML)

Explanation: **Explanation:** The correct answer is **Juvenile Chronic Myeloid Leukemia (JCML)**, now more commonly classified under Juvenile Myelomonocytic Leukemia (JMML). **1. Why JCML is correct:** JCML is a unique pediatric clonal myeloproliferative disorder. A hallmark feature of this condition is **"fetal-like erythropoiesis."** In JCML, the red blood cell precursors revert to a fetal phenotype, leading to a significant elevation of **Fetal Hemoglobin (HbF)**, often reaching 15–50% of total hemoglobin. This is a crucial diagnostic marker that helps differentiate JCML from adult-type CML occurring in children. **2. Why other options are incorrect:** * **Congenital red cell aplasia (Diamond-Blackfan Anemia):** While HbF can be elevated in this condition due to stress erythropoiesis, it is not the classic diagnostic association compared to the dramatic rise seen in JCML. * **Hereditary spherocytosis:** This is a red cell membrane defect. Hemoglobin electrophoresis is typically normal; there is no inherent mechanism for increased HbF production. * **Acute myeloid leukemia (AML):** While some cases of erythroleukemia (M6) might show minor elevations, increased HbF is not a characteristic or diagnostic feature of AML. **Clinical Pearls for NEET-PG:** * **JCML Triad:** Hepatosplenomegaly, lymphadenopathy, and skin rash (often xanthomas or café-au-lait spots). * **Diagnostic Clue:** Absence of the Philadelphia chromosome ($t[9;22]$) distinguishes JCML from adult CML. * **Other conditions with high HbF:** Beta-thalassemia major, Sickle cell anemia, and Hereditary Persistence of Fetal Hemoglobin (HPFH). * **JMML Association:** Strongly associated with **Neurofibromatosis type 1 (NF1)** and Noonan syndrome.

Question 26: Which of the following is true about acute idiopathic thrombocytopenic purpura (ITP)?

A. It is more common in adults.
B. Detection of specific anti-platelet antibodies is mandatory for diagnosis.
C. Viral infections predispose to ITP. (Correct Answer)
D. Approximately 80% of cases transform into chronic ITP.

Explanation: **Explanation:** **Acute Immune Thrombocytopenic Purpura (ITP)** is an acquired immune-mediated disorder characterized by isolated thrombocytopenia (platelet count <100,000/µL) due to the destruction of antibody-coated platelets in the reticuloendothelial system (primarily the spleen). **1. Why Option C is Correct:** In children, acute ITP is typically a post-infectious phenomenon. In approximately **70-80% of cases**, a preceding **viral illness** (such as Upper Respiratory Tract Infection, Varicella, Mumps, or EBV) occurs 1–3 weeks before the onset of bruising. It is thought to be caused by molecular mimicry, where antibodies produced against viral antigens cross-react with platelet surface glycoproteins (like GPIIb/IIIa). **2. Why Other Options are Incorrect:** * **Option A:** Acute ITP is primarily a disease of **childhood** (peak age 2–5 years). The adult form is more commonly chronic and insidious. * **Option B:** ITP is a **diagnosis of exclusion**. While anti-platelet antibodies are present, their detection is **not mandatory** for diagnosis because current assays have low sensitivity and the diagnosis is based on history, physical exam, and a peripheral smear. * **Option D:** The prognosis in children is excellent. Approximately **70–80% of cases resolve spontaneously** within 6 months. Only about 20% of childhood cases progress to chronic ITP (defined as lasting >12 months). **High-Yield Clinical Pearls for NEET-PG:** * **Classic Presentation:** A previously healthy child with sudden onset of petechiae, purpura, and epistaxis following a viral prodrome. * **Peripheral Smear:** Shows **isolated thrombocytopenia** with "Giant Platelets" (megathrombocytes). * **Bone Marrow:** Not routinely required, but if done, it shows **increased or normal megakaryocytes**. * **Treatment:** Most cases are self-limiting. If treatment is required (usually for mucosal bleeding or platelets <20,000), **IVIG** or **Corticosteroids** are first-line. Anti-D is used only in Rh-positive patients.

Question 27: A 5-year-old girl presents with a history of progressively increasing pallor for the past year and hepatosplenomegaly. Which of the following is the most relevant test for achieving a diagnosis?

A. Hb electrophoresis (Correct Answer)
B. Peripheral smear examination
C. Osmotic fragility test
D. Bone marrow examination

Explanation: **Explanation:** The clinical presentation of a 5-year-old child with **progressive pallor** (chronic anemia) and **hepatosplenomegaly** is a classic triad for **Thalassemia Major**. In the Indian context, this is the most common cause of transfusion-dependent hereditary anemia presenting in early childhood. **1. Why Hb Electrophoresis is the Correct Answer:** Hb electrophoresis (or HPLC) is the **gold standard** for diagnosing hemoglobinopathies. In Thalassemia Major, it reveals a characteristic pattern: a significant increase in **HbF (>90%)**, variable HbA2, and a near-total absence of HbA. This confirms the defect in globin chain synthesis, which is the underlying pathology. **2. Why Other Options are Incorrect:** * **Peripheral Smear:** While it shows microcytic hypochromic anemia with target cells and nucleated RBCs, these findings are non-specific and can overlap with Iron Deficiency Anemia (IDA). It suggests the diagnosis but does not confirm it. * **Osmotic Fragility Test:** This is the screening test for **Hereditary Spherocytosis**. In Thalassemia, osmotic fragility is actually *decreased* (cells are more resistant to lysis), making it an inappropriate diagnostic tool here. * **Bone Marrow Examination:** While it would show erythroid hyperplasia, it is an invasive procedure and is **not required** for the diagnosis of Thalassemia. **Clinical Pearls for NEET-PG:** * **HPLC (High-Performance Liquid Chromatography)** is now preferred over electrophoresis in modern practice for its precision. * **X-ray finding:** Look for "Hair-on-end" appearance (due to extramedullary hematopoiesis) and "Chipmunk facies." * **Mentzer Index:** (MCV/RBC count) <13 suggests Thalassemia trait; >13 suggests Iron Deficiency Anemia.

Question 28: All are seen in Thalassemia major EXCEPT:

A. Transfusion dependency
B. Splenoheptatomegaly
C. Ineffective erythropoiesis
D. Macrocytic anemia (Correct Answer)

Explanation: **Explanation:** **Why Macrocytic Anemia is the Correct Answer:** Thalassemia major is characterized by **microcytic hypochromic anemia**, not macrocytic anemia. The underlying pathology involves a genetic defect in the synthesis of globin chains (specifically $\beta$-chains in $\beta$-Thalassemia). This leads to a decrease in hemoglobin production within the red blood cells, resulting in smaller (microcytic) and paler (hypochromic) cells. Macrocytic anemia (MCV >100 fL) is typically seen in Vitamin B12 or Folate deficiency. **Analysis of Incorrect Options:** * **Transfusion dependency:** In Thalassemia major, the severe imbalance of globin chains leads to profound anemia. Patients typically present in the first year of life and require lifelong regular blood transfusions to maintain hemoglobin levels and suppress ineffective erythropoiesis. * **Splenohepatomegaly:** This occurs due to two mechanisms: **extramedullary hematopoiesis** (the body attempting to produce RBCs outside the bone marrow) and **hemosiderosis** (iron overload from chronic transfusions). * **Ineffective erythropoiesis:** The precipitation of unpaired $\alpha$-globin chains causes oxidative damage to the RBC precursors in the bone marrow, leading to their premature destruction before they reach circulation. **High-Yield Clinical Pearls for NEET-PG:** * **Peripheral Smear:** Look for target cells, nucleated RBCs, and Howell-Jolly bodies. * **Skeletal Changes:** "Chipmunk facies" and "Hair-on-end" appearance on X-ray due to compensatory bone marrow expansion. * **Diagnosis:** **Hb Electrophoresis** or HPLC is the gold standard (shows increased HbF and absent/low HbA). * **Mentzer Index:** (MCV/RBC count) <13 suggests Thalassemia; >13 suggests Iron Deficiency Anemia.

Question 29: What is true about iron deficiency anemia in children?

A. Iron absorption occurs from the terminal ileum.
B. Cow milk contains less iron than breast milk.
C. Serum ferritin depletes first. (Correct Answer)
D. Red cell distribution width (RDW) is decreased.

Explanation: In Iron Deficiency Anemia (IDA), the depletion of iron occurs in a predictable, sequential manner. Understanding these stages is high-yield for NEET-PG. ### **Why Option C is Correct** The progression of iron deficiency follows three distinct stages: 1. **Iron Depletion (Stage 1):** The body’s storage iron is exhausted first to maintain serum levels. **Serum ferritin** is the most sensitive marker for this stage and is the **earliest laboratory index to decline.** 2. **Iron Deficient Erythropoiesis (Stage 2):** Serum iron decreases, and Total Iron Binding Capacity (TIBC) increases. 3. **Iron Deficiency Anemia (Stage 3):** Hemoglobin falls, and microcytic hypochromic changes appear on the peripheral smear. ### **Why Other Options are Incorrect** * **Option A:** Iron is primarily absorbed in the **duodenum** and upper jejunum, not the terminal ileum (which is the site for Vitamin B12 absorption). * **Option B:** Both cow’s milk and breast milk have low iron content (approx. 0.5–1 mg/L). However, the **bioavailability** of iron in breast milk is much higher (50%) compared to cow’s milk (10%). Cow's milk is a major risk factor for IDA due to its low bioavailability and potential for microscopic intestinal blood loss. * **Option D:** RDW (Red Cell Distribution Width) is **increased** in IDA. It is one of the earliest signs of IDA and helps differentiate it from Thalassemia trait (where RDW is typically normal). ### **High-Yield Clinical Pearls for NEET-PG** * **Mentzer Index:** (MCV/RBC count) >13 suggests IDA; <13 suggests Thalassemia. * **Best screening test:** Hemoglobin. * **Most sensitive/earliest indicator:** Serum Ferritin. * **Gold Standard investigation:** Bone marrow iron stores (Prussian blue staining), though rarely done clinically. * **First response to oral iron:** Reticulocytosis (peaks at 7–10 days).

Question 30: Hereditary spherocytosis is best treated with?

A. Splenectomy (Correct Answer)
B. Immunoglobulins
C. Steroids
D. Blood transfusion

Explanation: **Explanation:** **Hereditary Spherocytosis (HS)** is an autosomal dominant disorder caused by defects in red blood cell (RBC) membrane proteins (most commonly **Ankyrin**, followed by Spectrin). These defects lead to a loss of membrane surface area, forcing RBCs to become spherical. These rigid spherocytes are trapped and destroyed by splenic macrophages, leading to extravascular hemolysis. **Why Splenectomy is the Correct Answer:** Splenectomy is the **definitive treatment** for moderate to severe HS. While it does not fix the underlying genetic membrane defect (spherocytes will still be present on a peripheral smear), it removes the primary site of RBC destruction. This significantly increases RBC lifespan, resolves anemia, and prevents the formation of pigment gallstones. **Why Other Options are Incorrect:** * **B & C (Immunoglobulins & Steroids):** These are used for **Autoimmune Hemolytic Anemia (AIHA)**, where hemolysis is mediated by antibodies. HS is a structural/genetic defect, not an immunological one; therefore, immunosuppression is ineffective. * **D (Blood Transfusion):** This is a supportive measure used only during acute hemolytic or aplastic crises. It is not a long-term treatment or cure. **High-Yield Clinical Pearls for NEET-PG:** * **Diagnosis:** Increased **MCHC** (>36 g/dL) is a classic finding. The **Osmotic Fragility Test** is the traditional screening test, but the **EMA Binding Test** (Flow cytometry) is now the gold standard. * **Complications:** Patients are prone to **Pigment (Calcium Bilirubinate) Gallstones** and aplastic crises (triggered by **Parvovirus B19**). * **Surgical Timing:** Splenectomy is usually delayed until **after age 5–6** to reduce the risk of Overwhelming Post-Splenectomy Infection (OPSI). * **Pre-op Requirement:** Immunization against encapsulated organisms (*S. pneumoniae, H. influenzae, N. meningitidis*) is mandatory 2 weeks prior to surgery.

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Hematology — MCQs

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