Hematology — MCQs

Hematology Indian Medical PG Practice Questions and MCQs

Question 121: A child with Thalassemia major, treated for cardiac arrhythmia due to iron overload, presents for regular transfusion. During the transfusion, the child becomes anxious and complains of back pain. What is the immediate next step in management?

A. Perform an ECG
B. Stop the transfusion and check for clerical errors (Correct Answer)
C. Continue transfusion while monitoring vital signs
D. Check for reddish discoloration of urine

Explanation: ### Explanation The clinical presentation of **anxiety and acute back pain** during a blood transfusion is a classic "red flag" for an **Acute Hemolytic Transfusion Reaction (AHTR)**. In a patient with Thalassemia Major, who is chronically transfused, the risk of alloimmunization and subsequent hemolytic reactions is significant. **1. Why Option B is Correct:** The most critical immediate action in any suspected transfusion reaction is to **stop the transfusion immediately** to limit the volume of incompatible blood infused. The most common cause of AHTR is **clerical error** (ABO incompatibility due to mislabeling or incorrect patient identification). Checking for clerical errors helps confirm if the wrong unit was administered, which guides further emergency management. **2. Why Other Options are Incorrect:** * **Option A (Perform an ECG):** While the patient has a history of iron-overload-induced arrhythmia, the temporal relationship with the transfusion and the specific symptom of back pain (flank pain) points toward hemolysis rather than a primary cardiac event. An ECG may be done later, but it is not the *immediate* priority. * **Option C (Continue transfusion):** This is dangerous and contraindicated. Continuing the transfusion in the setting of AHTR can lead to fatal disseminated intravascular coagulation (DIC), acute renal failure, and shock. * **Option D (Check for reddish urine):** Hemoglobinuria is a sign of intravascular hemolysis, but this is a *confirmatory* finding. Management must not be delayed to wait for the patient to void. ### Clinical Pearls for NEET-PG: * **Triad of AHTR:** Fever/chills, flank/back pain, and hypotension. * **Pathophysiology:** Type II Hypersensitivity reaction (Pre-formed antibodies attack donor RBCs). * **First Step in Management:** Stop transfusion → Maintain IV access with normal saline → Check clerical labels → Notify blood bank. * **Thalassemia specific:** Chronic transfusions lead to **Iron Overload** (monitored by Serum Ferritin) and **Alloimmunization** (making cross-matching difficult over time).

Question 122: What is the current practice guideline for the management of hereditary spherocytosis, EXCEPT?

A. Avoid splenectomy in mild cases
B. Delay splenectomy up to 4 years
C. Antipneumococcal vaccination before splenectomy
D. Penicillin prophylaxis post splenectomy (Correct Answer)

Explanation: ### Explanation The management of **Hereditary Spherocytosis (HS)** focuses on minimizing hemolysis and preventing complications of splenectomy. **Why Option D is the "Except" (Correct Answer):** While penicillin prophylaxis is traditionally recommended post-splenectomy to prevent **Overwhelming Post-Splenectomy Infection (OPSI)**, current guidelines (such as those from the British Society for Haematology) emphasize that **lifelong** prophylaxis is controversial. In many modern protocols, the emphasis has shifted toward **pre-splenectomy immunization** and "on-hand" antibiotics for febrile episodes rather than indefinite daily penicillin, especially in older children and adults. However, for NEET-PG purposes, the "Except" often highlights that the *primary* management goal in HS is to avoid or delay surgery rather than the post-operative antibiotic regimen itself. **Analysis of Other Options:** * **Option A (Avoid splenectomy in mild cases):** Correct practice. Patients with mild HS (compensated hemolysis) do not require splenectomy as the risks of surgery and sepsis outweigh the benefits. * **Option B (Delay splenectomy up to 4-6 years):** Correct practice. Splenectomy should be delayed until at least age 4 (ideally age 6) to allow the immune system to mature and reduce the risk of OPSI. * **Option C (Antipneumococcal vaccination):** Correct practice. Immunization against *S. pneumoniae*, *H. influenzae* type b, and *N. meningitidis* must be administered at least 2 weeks prior to elective splenectomy. **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Eosin-5-maleimide (EMA) binding test via flow cytometry (replaced the older Osmotic Fragility Test). * **Indication for Splenectomy:** Moderate to severe HS, growth retardation, or symptomatic cholelithiasis. * **Post-Splenectomy Blood Picture:** Presence of **Howell-Jolly bodies** (nuclear remnants in RBCs). * **Common Complication:** Aplastic crisis, most commonly triggered by **Parvovirus B19** infection.

Question 123: Which of the following conditions has the least impact on life expectancy since the point of diagnosis?

A. Chronic myeloid leukemia
B. Chronic lymphoid leukemia (Correct Answer)
C. Acute myeloid leukemia
D. Acute lymphoid leukemia

Explanation: **Explanation:** The question asks which leukemia has the **least impact on life expectancy** from the time of diagnosis. This refers to the disease with the most indolent (slow-growing) course and the highest overall survival rate. **Correct Option: B. Chronic Lymphoid Leukemia (CLL)** CLL is characterized by the slow accumulation of mature-appearing but functionally incompetent B-lymphocytes. It is often an incidental diagnosis in elderly patients (median age ~70 years). Many patients remain asymptomatic for years and may never require treatment. The disease progresses so slowly that many patients die *with* CLL rather than *of* it, meaning their life expectancy remains close to that of the age-matched general population. **Incorrect Options:** * **A. Chronic Myeloid Leukemia (CML):** While the prognosis has improved drastically with Imatinib (TKIs), CML is a progressive myeloproliferative disorder. Without treatment, it inevitably transforms into an "Accelerated Phase" or "Blast Crisis," which is fatal. * **C. Acute Myeloid Leukemia (AML):** This is an aggressive malignancy with a poor prognosis, especially in adults. Without immediate intensive chemotherapy, life expectancy is measured in weeks to months. * **D. Acute Lymphoid Leukemia (ALL):** Although it has a high cure rate in children (~90%), it is a medical emergency at diagnosis. It carries a much higher morbidity and mortality risk compared to the indolent nature of CLL. **NEET-PG High-Yield Pearls:** * **CLL Hallmark:** Presence of **Smudge cells** (Basket cells) on peripheral smear due to fragile lymphocytes. * **Staging:** Uses the **Rai** or **Binet** classification systems. * **Richter Transformation:** The sudden transformation of CLL into an aggressive High-Grade Lymphoma (usually DLBCL). * **Most Common Leukemia:** CLL is the most common leukemia in Western countries; however, in the pediatric age group (relevant to Pediatrics), **ALL** is the most common.

Question 124: A newborn baby presented with profuse bleeding from the umbilical stump. The rest of the examination and PT, APTT are within normal limits. What is the most probable diagnosis?

A. Factor X deficiency
B. Glanzmann thrombasthenia (Correct Answer)
C. Von Willebrand disease
D. Bernard-Soulier disease

Explanation: **Explanation:** The clinical presentation of profuse umbilical stump bleeding in a newborn with **normal PT (Prothrombin Time) and APTT (Activated Partial Thromboplastin Time)** points toward a primary hemostatic defect (platelet disorder) rather than a secondary hemostatic defect (clotting factor deficiency). **1. Why Glanzmann Thrombasthenia (GT) is correct:** GT is an autosomal recessive disorder caused by a deficiency or dysfunction of the **GPIIb/IIIa complex**, which is essential for platelet aggregation (binding to fibrinogen). While umbilical stump bleeding is classically associated with Factor XIII deficiency, it is also a recognized early manifestation of GT. Since PT and APTT assess the coagulation cascade and not platelet function, they remain normal in GT. **2. Why other options are incorrect:** * **Factor X deficiency:** This involves the common pathway of the coagulation cascade. Deficiency would result in **prolongation of both PT and APTT**. * **Von Willebrand Disease (vWD):** While it is a common bleeding disorder, it rarely presents with umbilical stump bleeding in the neonatal period. Furthermore, severe vWD often shows a **prolonged APTT** due to its role in stabilizing Factor VIII. * **Bernard-Soulier Disease:** This is a deficiency of **GPIb-IX-V** (platelet adhesion). While it presents with mucosal bleeding, it is characterized by **thrombocytopenia and giant platelets** on a peripheral smear, which distinguishes it from GT. **Clinical Pearls for NEET-PG:** * **Umbilical Stump Bleeding:** Think of Factor XIII deficiency (PT/APTT normal, but Clot Solubility test abnormal), Glanzmann Thrombasthenia, or Afibrinogenemia. * **GT Diagnosis:** Gold standard is **Platelet Aggregometry**, which shows absent aggregation with all agonists (ADP, collagen, epinephrine) except **Ristocetin** (which remains normal). * **Normal Platelet Count:** In GT, the platelet count and morphology are typically normal, unlike Bernard-Soulier Syndrome.

Question 125: A child died soon after birth. On examination, there was hepatosplenomegaly and edema all over the body. What is the most probable diagnosis?

A. beta-thalassemia
B. alpha-thalassemia (Correct Answer)
C. Hereditary spherocytosis
D. ABO incompatibility/sickle cell anemia

Explanation: ### Explanation The clinical presentation of a neonate with generalized edema (anasarca) and hepatosplenomegaly leading to death shortly after birth is the classic description of **Hydrops Fetalis**. **Why Alpha-Thalassemia is correct:** The most severe form of alpha-thalassemia is **Hb Bart’s (α-thalassemia major)**, where all four alpha-globin genes are deleted (--/--). Without alpha chains, the fetus cannot produce fetal hemoglobin (HbF: α2γ2) or adult hemoglobin (HbA: α2β2). Instead, gamma chains form tetramers (γ4), known as **Hb Bart’s**. This hemoglobin has an extremely high affinity for oxygen, refusing to release it to fetal tissues. This leads to severe intrauterine hypoxia, high-output cardiac failure, massive hepatosplenomegaly (due to extramedullary hematopoiesis), and ultimately, Hydrops Fetalis. **Why the other options are incorrect:** * **Beta-thalassemia:** Symptoms do not appear at birth because neonates primarily have HbF. Beta-chain production only becomes significant around 6 months of age, which is when Beta-thalassemia major typically manifests. * **Hereditary Spherocytosis:** While it causes hemolysis and jaundice, it rarely causes hydrops or immediate neonatal death. * **ABO Incompatibility:** This is usually mild. While Rh incompatibility can cause hydrops, ABO incompatibility typically presents as mild neonatal jaundice because A and B antigens are weakly expressed on fetal RBCs. * **Sickle Cell Anemia:** Like beta-thalassemia, it involves the beta-globin chain and does not manifest until HbF levels drop several months after birth. **High-Yield Clinical Pearls for NEET-PG:** * **Hb Bart’s:** Tetramer of gamma chains (γ4). * **HbH Disease:** Deletion of 3 alpha genes; tetramer of beta chains (β4). * **Hydrops Fetalis:** Defined as abnormal fluid accumulation in at least two fetal compartments (e.g., ascites, pleural effusion, pericardial effusion, or skin edema). * **Most common cause of non-immune hydrops in SE Asia:** Alpha-thalassemia major.

Question 126: What is the most common inherited bleeding disorder?

A. Bernard-Soulier syndrome
B. Glanzmann thrombasthenia
C. Idiopathic thrombocytopenic purpura (acute)
D. Von Willebrand disease (Correct Answer)

Explanation: **Explanation:** **Von Willebrand Disease (vWD)** is the most common inherited bleeding disorder worldwide, affecting approximately 1% of the general population. It is caused by a quantitative or qualitative deficiency of **Von Willebrand Factor (vWF)**, a large multimeric glycoprotein essential for platelet adhesion to the subendothelium (via GpIb receptors) and for stabilizing Factor VIII in the circulation. It is typically inherited in an **Autosomal Dominant** pattern (Types 1 and 2). **Analysis of Options:** * **Bernard-Soulier Syndrome:** A rare autosomal recessive disorder caused by a deficiency of the **GpIb-IX-V receptor**. It is characterized by giant platelets and thrombocytopenia. * **Glanzmann Thrombasthenia:** A rare autosomal recessive disorder caused by a deficiency of the **GpIIb/IIIa receptor**, leading to defective platelet aggregation. * **Idiopathic Thrombocytopenic Purpura (ITP):** While common, this is an **acquired** autoimmune condition, not an inherited one. **High-Yield Clinical Pearls for NEET-PG:** * **Clinical Presentation:** Mucocutaneous bleeding (epistaxis, menorrhagia, gingival bleeding). * **Screening Tests:** Bleeding Time (BT) is prolonged; Platelet count is usually normal (except in Type 2B). * **Confirmatory Tests:** Ristocetin Cofactor Activity (decreased) and vWF Antigen levels. * **APTT:** May be prolonged because vWF stabilizes Factor VIII. * **Treatment of Choice:** **Desmopressin (DDAVP)** for Type 1 (releases stored vWF from Weibel-Palade bodies); Cryoprecipitate or vWF-containing concentrates for severe cases.

Question 127: Infantile polycythemia is seen in which of the following conditions?

A. Cerebellar hemangioma (Correct Answer)
B. Retinoblastoma
C. Hepatoblastoma
D. All of the above

Explanation: **Explanation:** The correct answer is **Cerebellar hemangioma**. **1. Why Cerebellar Hemangioma is Correct:** Infantile polycythemia (erythrocytosis) in this context is a **paraneoplastic syndrome** caused by the ectopic production of **Erythropoietin (EPO)**. Cerebellar hemangiomas (specifically Hemangioblastomas) are highly vascular tumors often associated with **Von Hippel-Lindau (VHL) syndrome**. These tumor cells can autonomously secrete EPO, which stimulates the bone marrow to increase red blood cell production, leading to polycythemia. **2. Why the Other Options are Incorrect:** * **Retinoblastoma:** While this is the most common intraocular tumor of childhood, it is not associated with ectopic EPO production or polycythemia. It typically presents with leukocoria (white pupillary reflex). * **Hepatoblastoma:** Although some liver tumors (like Hepatocellular Carcinoma in adults) can cause polycythemia, Hepatoblastoma in infants is more commonly associated with precocious puberty (due to β-hCG secretion) or thrombocytosis, rather than erythrocytosis. **3. NEET-PG High-Yield Pearls:** * **Differential Diagnosis of Ectopic EPO Production:** Remember the mnemonic **"Potentially Really High Hematocrit"**: **P**heochromocytoma, **R**enal Cell Carcinoma (RCC), **H**epatocellular Carcinoma (HCC), and **H**emangioblastoma. * **VHL Syndrome Triad:** Cerebellar hemangioblastoma, Retinal angiomas, and Renal Cell Carcinoma. * **Infantile Polycythemia (Non-neoplastic):** More commonly caused by delayed cord clamping, maternal-fetal transfusion, or intrauterine growth restriction (IUGR) leading to fetal hypoxia. * **Clinical Presentation:** Infants with polycythemia may present with "ruddy" complexion (plethora), lethargy, or jaundice due to increased RBC turnover.

Question 128: What is the laboratory test used in blood to monitor low molecular weight heparins (LMWH) therapy?

A. Anti-Xa activity (Correct Answer)
B. Factor Xa level
C. Thrombin time
D. Antithrombin level

Explanation: **Explanation:** The correct answer is **Anti-Xa activity**. **Why it is correct:** Low Molecular Weight Heparins (LMWH), such as Enoxaparin, primarily exert their anticoagulant effect by binding to Antithrombin III, which then selectively inhibits **Factor Xa**. Unlike Unfractionated Heparin (UFH), LMWH has a minimal effect on Thrombin (Factor IIa). Therefore, standard tests like aPTT (which measures the intrinsic pathway and is sensitive to Thrombin inhibition) are not elevated by LMWH. The **Chromogenic Anti-Xa assay** is the specific functional test used to measure the plasma concentration of LMWH and its ability to inhibit Factor Xa. **Why other options are incorrect:** * **Factor Xa level:** This measures the amount of the factor present, not the inhibitory activity of the drug. We monitor the *neutralization* of the factor (Anti-Xa), not the level of the factor itself. * **Thrombin Time (TT):** This measures the conversion of Fibrinogen to Fibrin. It is highly sensitive to Unfractionated Heparin but remains normal with LMWH because LMWH does not significantly inhibit Thrombin. * **Antithrombin level:** While LMWH requires Antithrombin to work, measuring its level does not quantify the anticoagulant effect of the heparin dose administered. **NEET-PG High-Yield Pearls:** * **Monitoring:** LMWH does not require routine monitoring due to its predictable pharmacokinetics. Monitoring via Anti-Xa is indicated only in **pregnancy, obesity, renal failure, or neonates.** * **Timing:** Blood for Anti-Xa levels should be drawn at **peak effect** (4 hours after the subcutaneous dose). * **Antidote:** Protamine sulfate only partially neutralizes LMWH (approx. 60-70%), unlike its complete neutralization of UFH.

Question 129: A one-year-old baby, exclusively breastfed, presents with pallor, hepatomegaly, and no splenomegaly. What is the most important investigation?

A. Vitamin B12 estimation
B. Serum iron estimation (Correct Answer)
C. Folic acid estimation
D. Fetal hemoglobin estimation

Explanation: **Explanation:** The clinical presentation of a one-year-old child with pallor and hepatomegaly without splenomegaly, especially with a history of **exclusive breastfeeding**, is a classic scenario for **Iron Deficiency Anemia (IDA)**. Breast milk has low iron content, and physiological iron stores are typically exhausted by 4–6 months of age. If complementary feeding (weaning) is not started timely, IDA develops. **Why Serum Iron Estimation is correct:** In the context of nutritional anemia in infants, IDA is the most common cause worldwide. While both IDA and Megaloblastic anemia can cause pallor, the absence of splenomegaly and the specific age group strongly point toward IDA. Serum iron estimation (along with Serum Ferritin and TIBC) is the gold standard for confirming iron deficiency. **Analysis of Incorrect Options:** * **Vitamin B12 & Folic Acid Estimation:** While exclusive breastfeeding can lead to B12 deficiency if the mother is a strict vegetarian, Megaloblastic anemia often presents with hyperpigmentation of knuckles and neurological symptoms. IDA is statistically more prevalent in this demographic. * **Fetal Hemoglobin (HbF) Estimation:** This is used to diagnose Thalassemia. However, Thalassemia major typically presents with **significant splenomegaly** (due to extramedullary hematopoiesis) and a "chipmunk facies," which are absent here. **Clinical Pearls for NEET-PG:** * **Most common cause of anemia in India:** Iron Deficiency Anemia. * **Milk & Iron:** Cow’s milk is a poor source of iron and can cause occult GI blood loss in infants. * **Hepatomegaly in IDA:** Mild hepatomegaly can occur in severe nutritional anemia due to fatty changes or congestive heart failure, but **splenomegaly** is usually absent. * **Mentzer Index:** (MCV/RBC count) <13 suggests Thalassemia trait; >13 suggests IDA.

Question 130: Which of the following haemoglobin estimations will be diagnostically helpful in a case of beta-thalassemia trait?

A. Hb-F
B. Hb-A1c
C. Hb-A2 (Correct Answer)
D. Hb-H

Explanation: **Explanation:** The hallmark of **Beta-thalassemia trait (minor)** is a compensatory increase in **Hb-A2** levels. In this condition, there is a reduced synthesis of beta-globin chains. To compensate for the lack of Hb-A (α2β2), the body increases the production of delta-globin chains, which combine with alpha chains to form Hb-A2 (α2δ2). * **Diagnostic Criteria:** A Hb-A2 level **>3.5%** (typically 4–8%) on Hb electrophoresis or HPLC is the gold standard for diagnosing Beta-thalassemia trait. **Analysis of Incorrect Options:** * **Hb-F (α2γ2):** While Hb-F can be mildly elevated in the trait (1–5%), it is significantly elevated (up to 90%) in **Beta-thalassemia Major**. It is not as specific or consistent as Hb-A2 for the trait. * **Hb-A1c:** This is a measure of glycated hemoglobin used to monitor long-term glucose control in diabetes; it has no diagnostic value for thalassemia. * **Hb-H (β4):** This tetramer of beta-chains is the characteristic finding in **Alpha-thalassemia (3-gene deletion)**, not beta-thalassemia. **NEET-PG High-Yield Pearls:** 1. **Mentzer Index:** (MCV/RBC count) <13 suggests Thalassemia; >13 suggests Iron Deficiency Anemia (IDA). 2. **Iron Deficiency Masking:** Co-existing iron deficiency can lower Hb-A2 levels, potentially masking a Beta-thalassemia trait diagnosis. Always correct iron deficiency before testing. 3. **Peripheral Smear:** Shows microcytic hypochromic anemia with **target cells** and basophilic stippling.

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Hematology — MCQs

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