Hematology Practice Questions

Q: What is the primary drug used in the treatment of Acute Promyelocytic Leukemia?

Arsenic trioxide. Acute Promyelocytic Leukemia (APL), a subtype of AML (M3), is characterized by the chromosomal translocation **t(15;17)**, which results in the **PML-RARα** fusion gene [1]. This fusion protein arrests myeloid differentiation at the promyelocyte stage. **Why Arsenic Trioxide (ATO) is the correct answer:** While both All-trans retinoic acid (ATRA/Tretinoin) and Arsenic Trioxide (ATO) are used, **Arsenic Trioxide** is now considered the primary backbone of therapy, especially in low-to-intermediate risk patients. ATO works by binding directly to the PML moiety of the fusion protein, inducing its degradation and promoting apoptosis of the leukemic cells. When combined with ATRA, it achieves high cure rates without the need for traditional chemotherapy. **Analysis of Incorrect Options:** * **B. Tretinoin (ATRA):** While ATRA is a standard first-line agent that induces differentiation of promyelocytes, modern protocols (like the Lo-Coco regimen) emphasize the ATO-ATRA combination as the primary curative strategy. In many recent clinical guidelines, ATO is highlighted for its potent role in achieving molecular remission. * **C. Gefitinib:** This is a Tyrosine Kinase Inhibitor (TKI) targeting the **EGFR** mutation, primarily used in Non-Small Cell Lung Cancer (NSCLC) [2]. * **D. Dasatinib:** This is a second-generation TKI used for **Chronic Myeloid Leukemia (CML)** and Philadelphia chromosome-positive ALL. **High-Yield Clinical Pearls for NEET-PG:** * **DIC Risk:** APL is a medical emergency due to the high risk of Disseminated Intravascular Coagulation (DIC) triggered by the release of procoagulants from granules. * **Differentiation Syndrome:** A major side effect of ATRA/ATO therapy, characterized by fever, respiratory distress, and pulmonary infiltrates; treated with **Dexamethasone**. * **Monitoring:** ATO can cause **QT prolongation**; baseline and periodic ECGs are mandatory.

Q: Which of the following statements is incorrect regarding Hemophilia A?

APTT is decreased. ### Explanation **1. Why Option A is the Correct (Incorrect Statement):** Hemophilia A is a deficiency of **Factor VIII**, which is a key component of the **intrinsic pathway** of the coagulation cascade. In clinical practice, the **Activated Partial Thromboplastin Time (APTT)** measures the integrity of the intrinsic and common pathways [4]. Therefore, a deficiency in Factor VIII leads to a **prolonged (increased) APTT**, not a decreased one [4]. Bleeding time and PT (Prothrombin Time) remain normal in isolated Hemophilia A. **2. Analysis of Other Options:** * **Option B (Factor VIII is decreased):** This is the hallmark of Hemophilia A (an X-linked recessive disorder) [1]. * **Option C (Factor IX is normal):** Factor IX deficiency characterizes Hemophilia B (Christmas Disease). In Hemophilia A, Factor IX levels are unaffected [2]. * **Option D (VWF is normal):** While Factor VIII circulates bound to Von Willebrand Factor (VWF), Hemophilia A is a primary genetic defect of Factor VIII itself. VWF levels and function (Ristocetin cofactor activity) are normal, which helps distinguish it from Von Willebrand Disease (VWD) [5]. **3. NEET-PG High-Yield Clinical Pearls:** * **Inheritance:** X-linked recessive (affects males; females are typically asymptomatic carriers) [1]. * **Clinical Presentation:** Characterized by deep tissue bleeding, **hemarthrosis** (bleeding into joints, most commonly the knee), and delayed postsurgical bleeding [3]. * **Mixing Study:** If APTT is prolonged, a mixing study is performed. If the APTT **corrects** with normal plasma, it confirms a factor deficiency (like Hemophilia) [4]. If it does not correct, an inhibitor (antibody) is present. * **Treatment:** Recombinant Factor VIII concentrate. Desmopressin (DDAVP) can be used in mild cases to release stored Factor VIII from endothelial cells [2].

Q: Splenomegaly is a feature of all except:

Aplastic anemia. Explanation: Splenomegaly is a critical clinical sign used to differentiate various types of anemia and hematological disorders [1]. 1. Why Aplastic Anemia is the correct answer: Aplastic anemia is characterized by pancytopenia resulting from bone marrow failure (hypocellular marrow). Since the pathology is a primary failure of production rather than peripheral destruction or infiltration, the spleen is typically not enlarged. In fact, the presence of splenomegaly in a patient with pancytopenia should lead a clinician to look for alternative diagnoses like Aleukemic Leukemia, Myelofibrosis, or Portal Hypertension. 2. Analysis of incorrect options: * CML (Chronic Myeloid Leukemia): This is a myeloproliferative neoplasm where massive splenomegaly is a hallmark feature due to extramedullary hematopoiesis and infiltration by leukemic cells. * Thalassemia: Chronic hemolysis and ineffective erythropoiesis lead to compensatory extramedullary hematopoiesis and iron overload, both of which cause significant splenomegaly. * Hypersplenism: By definition, hypersplenism involves an enlarged spleen that is overactive, leading to the sequestration and destruction of blood cells (causing cytopenias). Clinical Pearls for NEET-PG: * Massive Splenomegaly (Spleen >8cm below costal margin): Remember the mnemonic "C-M-K" — CML, Myelofibrosis, Kala-azar (Visceral Leishmaniasis). Other causes include Malaria and Gaucher’s disease. * Pancytopenia WITHOUT Splenomegaly: Think Aplastic Anemia, PNH, or Vitamin B12 deficiency. * Pancytopenia WITH Splenomegaly: Think Kala-azar, Cirrhosis (Portal HTN), or Myelofibrosis.

Q: Which of the following features may be used to differentiate hemophilia A from von Willebrand disease?

Bleeding time. To differentiate between Hemophilia A and von Willebrand Disease (vWD), one must distinguish between a **coagulation factor deficiency** and a **platelet adhesion defect**. [1] ### **Why Bleeding Time is the Correct Answer** * **Bleeding Time (BT)** measures primary hemostasis (platelet plug formation). * In **vWD**, von Willebrand Factor is deficient or defective. Since vWF is essential for anchoring platelets to the subendothelium, the BT is **prolonged**. [2] * In **Hemophilia A**, the defect is strictly in the coagulation cascade (secondary hemostasis). Platelet function is normal; therefore, the BT is **normal**. ### **Why Other Options are Incorrect** * **B. Prothrombin Time (PT):** PT measures the extrinsic and common pathways (Factors VII, X, V, II, I). Both Hemophilia A and vWD involve the intrinsic pathway, so PT is **normal in both**. [1] * **C. Partial Thromboplastin Time (aPTT):** aPTT measures the intrinsic pathway. Since Factor VIII activity is reduced in both Hemophilia A and vWD (vWF stabilizes Factor VIII), the aPTT can be **prolonged in both**. [1][2] * **D. Factor VIII levels:** Factor VIII levels are decreased in Hemophilia A (primary deficiency) and often decreased in vWD (secondary to low vWF). Thus, it cannot reliably differentiate the two without further testing (like vWF Ag or Ristocetin cofactor assay). [2] ### **NEET-PG High-Yield Pearls** * **Inheritance:** Hemophilia A is **X-linked recessive** (mostly males); vWD is **Autosomal Dominant** (most common inherited bleeding disorder). * **Clinical Presentation:** Hemophilia presents with deep tissue bleeds and **hemarthrosis**. vWD presents with **mucocutaneous bleeding** (epistaxis, menorrhagia). [3] * **Ristocetin Cofactor Assay:** The gold standard for diagnosing vWD (measures vWF-induced platelet agglutination). [2] * **Mixing Study:** Both will show correction of aPTT when mixed with normal plasma (indicating a deficiency, not an inhibitor). [1]

Q: What is the single most important predictor of survival in multiple myeloma?

Serum b2-microglobulin. **Explanation:** The prognosis of Multiple Myeloma (MM) is primarily determined by the tumor burden and renal function. **Serum $\beta_2$-microglobulin** is the single most important predictor of survival because it reflects both of these factors [1]. It is a component of the MHC Class I molecule shed by myeloma cells; thus, high levels correlate with a high tumor mass and poor renal clearance. It is the cornerstone of the **International Staging System (ISS)** for MM [1]. **Analysis of Options:** * **A. IL-6 levels:** While IL-6 is the major growth factor for myeloma cells and correlates with disease activity, it is not used as a standardized prognostic marker in clinical practice compared to $\beta_2$-microglobulin. * **B. Bence Jones proteinuria:** This represents free light chains in the urine. While it is a diagnostic hallmark and can lead to renal failure (Myeloma kidney), the absolute quantity of proteinuria is a less reliable independent predictor of overall survival than serum markers [1]. * **C. CD 138 positivity:** Also known as Syndecan-1, this is a highly specific marker used for the **diagnosis** (immunophenotyping) of plasma cells. It does not serve as a primary prognostic indicator for survival. **High-Yield Clinical Pearls for NEET-PG:** * **ISS Staging:** * Stage I: $\beta_2$-microglobulin < 3.5 mg/L and Albumin $\geq$ 3.5 g/dL [1]. * Stage III: $\beta_2$-microglobulin $\geq$ 5.5 mg/L [1]. * **Revised ISS (R-ISS):** Now includes **LDH levels** and **high-risk cytogenetics** [t(4;14), t(14;16), and del(17p)] for more accurate prognosis. * **Most common cause of death:** Infection (due to hypogammaglobulinemia), followed by renal failure.

Q: Which of the following is the first symptom of iron deficiency anemia?

Reduced ferritin. ### Explanation Iron deficiency anemia (IDA) develops in a sequential manner, progressing through three distinct stages [3]. Understanding these stages is crucial for identifying the earliest markers of the disease. **1. Why Reduced Ferritin is Correct:** The first stage of iron deficiency is **Iron Depletion**. In this stage, the body’s iron stores are exhausted to maintain normal serum iron levels for erythropoiesis [3]. **Serum Ferritin** is the most sensitive and earliest laboratory marker because it directly reflects the body's total iron stores. When ferritin levels drop, it indicates that the "reserve tank" is emptying, even though circulating iron and hemoglobin levels remain normal. **2. Why the Other Options are Incorrect:** * **Low iron concentration in blood (Serum Iron):** This occurs during the second stage, **Iron Deficient Erythropoiesis**. Serum iron only drops after the storage iron (ferritin) is significantly depleted [2]. * **Reduced Hemoglobin (Hb):** This is a late manifestation occurring in the third stage, **Iron Deficiency Anemia**. Hb only falls after iron stores are gone and the supply to the bone marrow is insufficient to produce mature red cells [1], [2]. * **Reduced PCV (Packed Cell Volume):** Like hemoglobin, PCV (Hematocrit) decreases only in the final stage of the disease [2]. **Clinical Pearls for NEET-PG:** * **Sequence of Depletion:** Ferritin ↓ → Serum Iron ↓ & TIBC ↑ → Hemoglobin ↓ (Microcytic Hypochromic). * **Best Screening Test:** Serum Ferritin (Note: It is an acute-phase reactant, so it may be falsely normal in inflammation). * **Gold Standard Test:** Bone marrow aspiration with **Prussian Blue staining** (demonstrates absent hemosiderin), though rarely done clinically. * **Earliest Morphological Change:** An increase in **Red Cell Distribution Width (RDW)** often precedes the drop in MCV.

Question 1

A 36-year-old man from China presents with increasing fatigue. He has a 3-year history of tuberculosis, and CBC shows a mild microcytic anemia. Blood work-up demonstrates low serum iron, low iron-binding capacity, and increased serum ferritin. What is the most likely underlying mechanism for the anemia in this patient?

Accepted Answer

Impaired utilization of iron from storage sites

Answer

Clonal stem cell defect

Answer

Hypoxemia

Answer

Synthesis of structurally abnormal globin chains

Question 2

The Schilling Test is abnormal in which of the following conditions?

Accepted Answer

Intrinsic factor deficiency

Answer

Amylase deficiency

Answer

Lipase deficiency

Answer

Pancreatic insufficiency

Question 3

What is the primary drug used in the treatment of Acute Promyelocytic Leukemia?

Accepted Answer

Arsenic trioxide

Answer

Tretinoin

Answer

Gefitinib

Answer

Dasatinib

Question 4

Which of the following statements is incorrect regarding Hemophilia A?

Accepted Answer

APTT is decreased

Answer

Factor VIII is decreased

Answer

Factor IX is normal

Answer

Von Willebrand Factor (VWF) is normal

Question 5

Splenomegaly is a feature of all except:

Accepted Answer

Aplastic anemia

Answer

CML

Answer

Thalassemia

Answer

Hypersplenism

Question 6

What is the most effective treatment for the accelerated phase of chronic myeloid leukaemia?

Accepted Answer

Allogeneic bone marrow transplantation

Answer

Autologous bone marrow transplantation

Answer

Chemotherapy

Answer

Hydroxyurea and interferon

Question 7

Which of the following features may be used to differentiate hemophilia A from von Willebrand disease?

Accepted Answer

Bleeding time

Answer

Prothrombin time

Answer

Partial thromboplastin time

Answer

Factor VIII levels

Question 8

All of the following statements about the treatment of typical Chronic Lymphocytic Leukemia (CLL) are true, except:

Accepted Answer

Treatment should be initiated as soon as diagnosis is established.

Answer

Chlorambucil and Fludarabine are commonly used agents.

Answer

Fludarabine is generally preferred in younger patients, while Chlorambucil may be preferred in the elderly.

Answer

Younger patients may be candidates for bone marrow transplantation.

Question 9

What is the single most important predictor of survival in multiple myeloma?

Accepted Answer

Serum b2-microglobulin

Answer

IL-6 levels

Answer

Bence jones proteinuria

Answer

CD 138 positivity

Question 10

Which of the following is the first symptom of iron deficiency anemia?

Accepted Answer

Reduced ferritin

Answer

Low iron concentration in blood

Answer

Reduced hemoglobin

Answer

Reduced PCV

Hematology — MCQs

Hematology — MCQs

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