Hematology Practice Questions

Q: Hereditary spherocytosis is best treated with?

Splenectomy. **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.

Q: Which of the following is NOT true about polycythemia vera?

Infection. **Explanation:** Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by the autonomous overproduction of all three myeloid cell lines (erythrocytosis, leukocytosis, and thrombocytosis), primarily driven by the **JAK2 V617F mutation** [1]. **Why "Infection" is the correct answer:** While PV involves an increase in the total white blood cell count (leukocytosis), these cells are generally mature and functional. Unlike conditions like leukemia or myelodysplastic syndromes where cells are dysfunctional, or states of neutropenia, **infection is not a characteristic feature or a common complication of PV.** Patients are more likely to suffer from vascular events than infectious ones. **Analysis of incorrect options:** * **Bleeding:** Paradoxically, despite high platelet counts, PV patients are at risk for bleeding. This is often due to **acquired von Willebrand Syndrome**, where high shear stress and massive platelet surfaces lead to the depletion of large vWF multimers. * **Thrombosis:** This is the **most common cause of morbidity and mortality** in PV. Hyperviscosity (due to high hematocrit) and qualitative platelet abnormalities lead to both arterial (stroke, MI) and venous (DVT, Budd-Chiari syndrome) thrombosis [1]. * **Decreased ESR:** The Erythrocyte Sedimentation Rate (ESR) is essentially zero in PV. Since ESR depends on the rate at which RBCs fall, the extreme overcrowding of cells (high hematocrit) prevents them from settling, resulting in a **near-zero ESR**. **High-Yield Clinical Pearls for NEET-PG:** * **Hallmark symptom:** Aquagenic pruritus (itching after a warm bath) due to mast cell degranulation [1]. * **Physical Exam:** Splenomegaly (present in 75% of cases) and facial plethora [1]. * **Diagnosis:** Low serum Erythropoietin (EPO) levels and JAK2 mutation (95% of cases) [1]. * **Treatment of choice:** Phlebotomy (target Hct <45%) and low-dose Aspirin. Hydroxyurea is used for high-risk patients.

Q: All of the following cause microcytic anemia except?

Orotic aciduria. **Explanation:** The classification of anemia is primarily based on the **Mean Corpuscular Volume (MCV)**. Microcytic anemias (MCV 100 fL) result from defects in DNA synthesis or erythrocyte maturation. **Why Orotic Aciduria is the correct answer:** Orotic aciduria is a rare autosomal recessive disorder caused by a deficiency of **UMP synthase**. This enzyme is crucial for **de novo pyrimidine synthesis**. A defect leads to impaired DNA synthesis, which manifests as **Megaloblastic Anemia** (a type of macrocytic anemia). Unlike nutritional megaloblastic anemias, it does not respond to Vitamin B12 or Folate but responds to **Uridine supplementation**. **Analysis of Incorrect Options (Causes of Microcytic Anemia):** * **Hookworm infestation:** Causes chronic gastrointestinal blood loss, leading to **Iron Deficiency Anemia (IDA)**, the most common cause of microcytic hypochromic anemia worldwide [2]. * **Thalassemia:** A genetic defect in the synthesis of globin chains (alpha or beta), leading to ineffective erythropoiesis and microcytosis [1]. * **Pyridoxine (Vitamin B6) deficiency:** Vitamin B6 is a mandatory cofactor for **ALAS**, the rate-limiting enzyme in heme synthesis. Deficiency leads to **Sideroblastic Anemia**, which is characteristically microcytic. **NEET-PG High-Yield Pearls:** * **Mnemonic for Microcytic Anemia (TAILS):** **T**halassemia, **A**nemia of chronic disease (some cases), **I**ron deficiency, **L**ead poisoning, **S**ideroblastic anemia [2]. * **Orotic Aciduria vs. OTC Deficiency:** Both present with high orotic acid in urine. However, **OTC deficiency** (Urea cycle defect) presents with **hyperammonemia** and NO megaloblastic anemia, whereas **Orotic Aciduria** presents with **megaloblastic anemia** and NO hyperammonemia.

Q: Which of the following conditions has the least 5-year survival rate following allogeneic bone marrow transplantation?

Blast crisis in Chronic Myeloid Leukemia. **Explanation:** The success of allogeneic bone marrow transplantation (BMT) depends primarily on the underlying disease state, the patient’s age, and the degree of HLA matching. **Why C is Correct:** **Blast crisis in Chronic Myeloid Leukemia (CML)** represents the terminal phase of the disease, characterized by a high burden of immature blasts and complex genetic mutations. Even with aggressive conditioning and BMT, the risk of **relapse** is extremely high, and the 5-year survival rate is generally the lowest among the options (often **less than 10-20%**) [1]. In contrast, BMT performed during the chronic phase of CML yields much higher survival rates (70-80%). **Why the other options are incorrect:** * **A. Severe Combined Immunodeficiency (SCID):** These patients have the best outcomes (often >80-90% survival) because they are naturally "immunosuppressed," reducing the risk of graft rejection, and the transplant is curative for their genetic defect. * **B. Aplastic Anemia:** In young patients with a matched sibling donor, the 5-year survival rate for BMT is excellent, typically ranging from **70% to 90%**. * **D. Chronic Lymphocytic Leukemia (CLL):** While CLL is usually managed with targeted therapies, BMT is reserved for high-risk cases. However, because CLL is a more indolent (slow-growing) disease compared to CML blast crisis, the survival outcomes are significantly better [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Best prognosis for BMT:** Non-malignant conditions like SCID and Aplastic Anemia. * **CML Timing:** The single most important prognostic factor for BMT in CML is the **stage of disease** at the time of transplant (Chronic phase > Accelerated phase > Blast crisis). * **Graft-versus-Leukemia (GVL) effect:** This is beneficial in CML, but in blast crisis, the disease progression often outpaces the immune system's ability to clear the malignant cells.

Q: A 78-year-old man presents with anorexia, fatigue, and generally feeling unwell. On examination, he has a large spleen, appears pale, and the rest of the examination is normal. His blood count is abnormal; the WBC is 46000/ml with increased mature granulocytes, hemoglobin 9.0 g/dL, and platelets 450,000/mL. Further investigations reveal BCR-ABL transcripts. T315I mutation is also seen. What is the drug of choice for this condition?

Ponatinib. The clinical presentation of a 78-year-old man with massive splenomegaly, leukocytosis (46,000/mL) with mature granulocytes, and the presence of **BCR-ABL transcripts** (Philadelphia chromosome) confirms the diagnosis of **Chronic Myeloid Leukemia (CML)** [1]. The critical finding in this case is the presence of the **T315I mutation** (often called the "gatekeeper mutation"). This specific mutation involves a threonine-to-isoleucine substitution at position 315, which creates steric hindrance that prevents first-generation (Imatinib) and second-generation (Dasatinib, Nilotinib, Bosutinib) Tyrosine Kinase Inhibitors (TKIs) from binding to the ATP-binding pocket of the BCR-ABL protein [1]. **Why Ponatinib is correct:** **Ponatinib** is a third-generation TKI specifically designed with a carbon-carbon triple bond that bypasses the steric hindrance caused by the T315I mutation. It is currently the only approved TKI effective against CML harboring this specific mutation. **Why other options are incorrect:** * **Bosutinib:** A second-generation TKI. While effective against many mutations, it is ineffective against the T315I mutation [1]. * **Bortezomib:** A proteasome inhibitor used primarily in Multiple Myeloma, not CML. * **Chlorambucil:** An alkylating agent used in Chronic Lymphocytic Leukemia (CLL), not indicated for BCR-ABL positive CML [2]. **Clinical Pearls for NEET-PG:** * **T315I Mutation:** The most common cause of resistance to 1st and 2nd generation TKIs in CML. * **Drug of Choice for CML (General):** Imatinib (1st gen) or Nilotinib/Dasatinib (2nd gen) [1]. * **Drug of Choice for T315I Mutation:** Ponatinib. * **Side Effect Note:** Ponatinib is associated with a high risk of arterial thrombotic events (prothrombotic state). * **Asciminib:** A newer "STAMP" inhibitor (Specifically Targeting the ABL Myristoyl Pocket) is also used for T315I mutations in patients resistant to other therapies.

Q: What is a characteristic feature of sickle cell trait?

Isosthenuria. **Explanation:** **Sickle Cell Trait (HbAS)** occurs when an individual inherits one normal hemoglobin gene (HbA) and one sickle gene (HbS). Unlike Sickle Cell Disease (HbSS), the trait is generally a benign, asymptomatic carrier state. **Why Isosthenuria is the Correct Answer:** The most common clinical manifestation of sickle cell trait is **isosthenuria** (the inability to concentrate or dilute urine, resulting in a fixed specific gravity). This occurs because the renal medulla is a hypoxic and hypertonic environment, which triggers localized sickling in the vasa recta. This leads to subclinical micro-infarctions (renal papillary necrosis), impairing the countercurrent exchange mechanism. Another common finding in trait patients is **painless hematuria**. **Why the Other Options are Incorrect:** * **A. Anemia:** Patients with sickle cell trait have normal hemoglobin levels, normal RBC indices, and a normal peripheral smear [1]. Significant anemia suggests HbSS or another hemoglobinopathy. * **B. Bony pain & C. Dactylitis:** These are hallmarks of **Vaso-occlusive Crises (VOC)**. In HbAS, the concentration of HbS is typically <40%, which is insufficient to cause spontaneous sickling or systemic VOC under physiological conditions [2]. These symptoms only occur in HbAS under extreme circumstances (e.g., severe hypoxia at high altitudes or extreme physical exertion). **High-Yield Clinical Pearls for NEET-PG:** * **Screening Test:** Solubility test (Sodium dithionite test) is positive in both trait and disease. * **Confirmatory Test:** Hb Electrophoresis (HbAS shows HbA > HbS; HbSS shows no HbA). * **Renal Association:** Sickle cell trait is a recognized risk factor for **Renal Medullary Carcinoma**, a highly aggressive malignancy. * **Protection:** HbAS provides a survival advantage against severe *Plasmodium falciparum* malaria.

Q: Gum hypertrophy is a clinical feature of which of the following conditions?

Acute Myeloid Leukemia (AML). **Explanation:** **1. Why AML is the correct answer:** Gum hypertrophy (gingival hyperplasia) is a classic clinical sign associated with **Acute Myeloid Leukemia (AML)**, specifically the monocytic subtypes [1]. Under the French-American-British (FAB) classification, these are **M4 (Acute Myelomonocytic Leukemia)** and **M5 (Acute Monocytic Leukemia)**. The underlying mechanism involves the infiltration of the gingival tissues by malignant leukemic blasts (monoblasts and myeloblasts). This leads to swelling, redness, and sometimes bleeding of the gums. **2. Why the other options are incorrect:** * **Mitral Stenosis (MS):** This is a valvular heart disease. While it can cause "Malar flush" (pinkish-purple patches on the cheeks) due to low cardiac output and systemic vasoconstriction, it has no association with gingival changes. * **Acute Lymphocytic Leukemia (ALL):** While ALL is the most common leukemia in children and presents with bone pain and lymphadenopathy, it rarely causes tissue infiltration like gum hypertrophy. * **Non-Hodgkin Lymphoma (NHL):** NHL primarily presents with painless lymphadenopathy or extranodal involvement (like the GI tract or skin), but gingival hypertrophy is not a characteristic diagnostic feature. **3. NEET-PG High-Yield Pearls:** * **Mnemonic for Gum Hypertrophy in AML:** Remember **"M4 and M5"** as the "Mouth" subtypes. * **Other causes of Gum Hypertrophy:** Often tested as a "Differential Diagnosis" question: 1. **Drugs:** Phenytoin (Antiepileptic) [2], Cyclosporine (Immunosuppressant), and Nifedipine/Verapamil (Calcium Channel Blockers). 2. **Scurvy:** Vitamin C deficiency. 3. **Pregnancy:** Due to hormonal changes. * **Auer Rods:** These are pathognomonic for AML (especially M3) but are **absent** in ALL.

Q: Which of the following statements regarding hereditary hemorrhagic telangiectasia is true?

It is associated with cerebral arteriovenous malformations.. **Hereditary Hemorrhagic Telangiectasia (HHT)**, also known as **Osler-Weber-Rendu Syndrome**, is an autosomal dominant disorder characterized by multisystem vascular malformations. ### **Explanation of the Correct Option** **Option B is correct** because HHT involves the formation of direct connections between arteries and veins, bypassing the capillary bed. These **Arteriovenous Malformations (AVMs)** commonly occur in the **lungs (50%), liver (70%), and brain (10%)**. Cerebral AVMs are high-yield clinical features as they can lead to life-threatening complications like seizures or intracranial hemorrhage. ### **Analysis of Incorrect Options** * **Option A:** While GI bleeding occurs in about 25% of patients (usually later in life), **epistaxis (nosebleeds)** is the most common and earliest presenting feature, seen in over 90% of cases. * **Option C:** HHT involves both **mucosal and cutaneous** surfaces. Characteristic telangiectasias are typically found on the lips, tongue, face, and fingertips. * **Option D:** Estrogen was historically used to reduce bleeding, but it is **not first-line**. Management focuses on local measures (cautery, laser) and **Bevacizumab** (anti-VEGF antibody) for systemic involvement. ### **NEET-PG High-Yield Pearls** * **Curacao Criteria:** Used for diagnosis (Epistaxis, Telangiectasias, Visceral AVMs, and First-degree relative with HHT). 3/4 criteria confirm the diagnosis. * **Genetics:** Mutations in **ENG** (HHT1 - more pulmonary AVMs) or **ACVRL1** (HHT2 - more hepatic involvement). * **Complication:** Pulmonary AVMs can lead to paradoxical embolism, resulting in **brain abscesses** or strokes.

Q: Christmas disease is treated by?

Fresh frozen plasma. Explanation: **Christmas disease**, also known as **Hemophilia B**, is an X-linked recessive bleeding disorder caused by a deficiency of **Clotting Factor IX** [1]. 1. **Why Fresh Frozen Plasma (FFP) is correct:** FFP contains all coagulation factors, including Factor IX. In the absence of specific recombinant Factor IX concentrates (which is the gold standard but often not listed in basic MCQ options), FFP is the primary source used to replenish Factor IX levels and achieve hemostasis [1]. 2. **Why other options are incorrect:** * **Fresh frozen blood:** This is not a standard component therapy. Whole blood is rarely used because it can lead to volume overload and contains lower concentrations of specific factors compared to plasma. * **Cryoprecipitate:** This is a **high-yield trap**. Cryoprecipitate is rich in Factor VIII, Fibrinogen, von Willebrand Factor, and Factor XIII. It **does not contain Factor IX**. Therefore, it is used for Hemophilia A and von Willebrand disease, but is ineffective for Christmas disease. * **Steroids:** These have no role in replacing clotting factors; they are used in immune-mediated hematological conditions like ITP. **Clinical Pearls for NEET-PG:** * **Hemophilia A:** Factor VIII deficiency (Treated with Cryoprecipitate or Factor VIII concentrate). * **Hemophilia B:** Factor IX deficiency (Treated with FFP or Factor IX concentrate) [1]. * **Inheritance:** Both are X-linked recessive (mostly affects males) [1]. * **Lab Findings:** Prolonged **aPTT**, but normal PT and Bleeding Time (BT). * **Most common site of bleeding:** Hemarthrosis (bleeding into joints, most commonly the knee) [1].

Question 1

Hereditary spherocytosis is best treated with?

Accepted Answer

Splenectomy

Answer

Immunoglobulins

Answer

Steroids

Answer

Blood transfusion

Question 2

Which of the following is NOT true about polycythemia vera?

Accepted Answer

Infection

Answer

Bleeding

Answer

Thrombosis

Answer

Decreased ESR

Question 3

All of the following cause microcytic anemia except?

Accepted Answer

Orotic aciduria

Answer

Hookworm infestation

Answer

Thalassemia

Answer

Pyridoxine deficiency

Question 4

Which of the following conditions has the least 5-year survival rate following allogeneic bone marrow transplantation?

Accepted Answer

Blast crisis in Chronic Myeloid Leukemia

Answer

Severe Combined Immunodeficiency state

Answer

Aplastic Anemia

Answer

Chronic lymphocytic leukemia

Question 5

A 78-year-old man presents with anorexia, fatigue, and generally feeling unwell. On examination, he has a large spleen, appears pale, and the rest of the examination is normal. His blood count is abnormal; the WBC is 46000/ml with increased mature granulocytes, hemoglobin 9.0 g/dL, and platelets 450,000/mL. Further investigations reveal BCR-ABL transcripts. T315I mutation is also seen. What is the drug of choice for this condition?

Accepted Answer

Ponatinib

Answer

Bosutinib

Answer

Bortezomib

Answer

Chlorambucil

Question 6

What is a characteristic feature of sickle cell trait?

Accepted Answer

Isosthenuria

Answer

Anemia

Answer

Bony pain

Answer

Dactylitis

Question 7

All are true regarding fetal RBCs, EXCEPT:

Accepted Answer

Elevated 2,3-DPG

Answer

Decreased carbonic anhydrase activity

Answer

Decreased life span

Answer

High RBC volume

Question 8

Gum hypertrophy is a clinical feature of which of the following conditions?

Accepted Answer

Acute Myeloid Leukemia (AML)

Answer

Mitral Stenosis (MS)

Answer

Acute Lymphocytic Leukemia (ALL)

Answer

Non-Hodgkin Lymphoma (NHL)

Question 9

Which of the following statements regarding hereditary hemorrhagic telangiectasia is true?

Accepted Answer

It is associated with cerebral arteriovenous malformations.

Answer

Gastrointestinal bleeding is the major presenting feature.

Answer

Only mucosal involvement is present, and the skin is usually spared.

Answer

Estrogen is the first-line treatment.

Question 10

Christmas disease is treated by?

Accepted Answer

Fresh frozen plasma

Answer

Fresh frozen blood

Answer

Cryoprecipitate

Answer

Steroids

Hematology — MCQs

Hematology — MCQs

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