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 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.

Q: A 56-year-old female presents with headache, dizziness, generalized itching, especially after hot showers, and intense burning in her hands and feet. She reports relief with aspirin. Physical examination reveals splenomegaly and elevated blood pressure. Investigations show HB: 20.1 g/dl, Hematocrit: 60%, WBC: 15800, Platelet count: 500,000/µl, low spO2: 98%, and increased LAP. Which of the following is the most common mutation seen in the condition described?

JAK2. **Explanation:** The clinical presentation is a classic case of **Polycythemia Vera (PV)**, a myeloproliferative neoplasm (MPN). The patient exhibits the hallmark "triad" of PV: **panmyelosis** (elevated RBCs, WBCs, and platelets), **aquagenic pruritus** (itching after hot showers), and **erythromelalgia** (burning pain in extremities relieved by aspirin) [1]. Splenomegaly and low serum erythropoietin (implied by high Hb with normal SpO2) further support this diagnosis. **1. Why JAK2 is correct:** The **JAK2 V617F mutation** (located on exon 14) is the molecular hallmark of Polycythemia Vera, present in **>95% of cases** [1]. A small minority of the remaining cases (~3-4%) carry a mutation in **JAK2 exon 12**. This mutation leads to constitutive activation of the Janus Kinase 2 signaling pathway, causing erythropoietin-independent proliferation of myeloid lineages. **2. Why other options are incorrect:** * **CALR (Calreticulin):** Mutations are common in Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF) but are **never** seen in PV. * **RAS:** Mutations are associated with various solid tumors and certain leukemias (like CMML) but are not the primary drivers of MPNs. * **MYC:** This oncogene is associated with aggressive lymphomas, most notably Burkitt Lymphoma (t(8;14)). **Clinical Pearls for NEET-PG:** * **Diagnostic Criteria:** PV requires meeting 3 major criteria (High Hb/Hct, BM hypercellularity, JAK2 mutation) OR 2 major and 1 minor (Low EPO level). * **LAP Score:** Leukocyte Alkaline Phosphatase (LAP) is **increased** in PV and Leukemoid reaction, but **decreased** in CML. * **Treatment:** Phlebotomy (target Hct <45%) and low-dose aspirin are first-line. Hydroxyurea is used for high-risk patients.

Q: A decrease in serum iron and a decrease in total iron-binding capacity (TIBC) is seen in which condition?

Anemia of chronic disease. **Explanation:** The correct answer is **Anemia of Chronic Disease (ACD)**. The hallmark of ACD is the sequestration of iron within the reticuloendothelial system (macrophages), primarily driven by the inflammatory cytokine **IL-6**. IL-6 stimulates the liver to produce **Hepcidin**, which degrades ferroportin (the iron export channel). This leads to: 1. **Decreased Serum Iron:** Iron is trapped inside cells and cannot be released into the plasma. 2. **Decreased TIBC:** TIBC is a surrogate measure of Transferrin. In chronic inflammation, Transferrin (a negative acute-phase reactant) decreases. Furthermore, the body downregulates TIBC to limit iron availability to potential pathogens. **Analysis of Incorrect Options:** * **Iron Deficiency Anemia (IDA):** Characterized by **decreased** serum iron but **increased** TIBC. The body increases Transferrin production to maximize the transport of any available iron. * **Thalassemia:** Typically presents with **normal to increased** serum iron and TIBC, as it is a defect in globin chain synthesis, not iron metabolism. * **Sideroblastic Anemia:** Characterized by iron overload. Serum iron is **increased**, and TIBC is usually **normal or decreased** due to saturation. **NEET-PG High-Yield Pearls:** * **Ferritin:** The most crucial differentiator. Ferritin is **low** in IDA but **normal or high** in ACD (as it is a positive acute-phase reactant). * **Soluble Transferrin Receptor (sTfR):** Elevated in IDA but **normal** in ACD. This is the most sensitive test to distinguish the two when they coexist. * **Mentzer Index (MCV/RBC):** 13 suggests IDA.

Q: A 23-year-old female has presented with anemia and jaundice for 2 years. Peripheral smear shows spherocytes. What is the most appropriate investigation to be done?

Coombs test. ### Explanation The clinical presentation of anemia, jaundice, and the presence of **spherocytes** on a peripheral smear indicates **extravascular hemolysis** [1]. In such cases, the primary differential diagnosis lies between **Hereditary Spherocytosis (HS)** and **Autoimmune Hemolytic Anemia (AIHA)**. **1. Why Coombs Test is the Correct Answer:** The most critical step in evaluating spherocytosis is to differentiate between an inherited membrane defect (HS) and an acquired immune-mediated destruction (AIHA). The **Direct Antiglobulin Test (Coombs test)** is the initial investigation of choice because AIHA is more common in young females and is a treatable condition [1]. A positive Coombs test confirms AIHA, while a negative test points toward HS [1]. **2. Why Other Options are Incorrect:** * **Reticulocyte count:** While this will be elevated in any hemolytic anemia, it is a non-specific marker of marrow response and does not help in identifying the specific etiology of spherocytosis [1]. * **Osmotic fragility test (OFT):** Historically used for HS, it is now largely replaced by the **Eosin-5-maleimide (EMA) binding test**. Furthermore, OFT can be positive in both HS and AIHA, making it less specific than the Coombs test for initial differentiation. * **Bone marrow aspiration:** This is generally not indicated in the workup of hemolytic anemias unless a primary bone marrow failure or malignancy is suspected. It would merely show erythroid hyperplasia. **Clinical Pearls for NEET-PG:** * **Spherocytes** lack a central pallor because they have the lowest surface-area-to-volume ratio [1]. * **AIHA vs. HS:** If the question mentions a positive family history or splenomegaly since childhood, think HS. If it mentions a young female (potential SLE association), think AIHA [1]. * **Gold Standard for HS:** The EMA Binding test (Flow cytometry) is now preferred over the Osmotic Fragility Test.

Question 1

High serum uric acid, high serum phosphates, and high serum creatinine in a patient will be observed in which of the following conditions?

Accepted Answer

Chronic lymphocytic leukemia

Answer

Obstructive jaundice

Answer

Status epilepticus

Answer

Typhoid

Question 2

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?

Accepted Answer

Autoimmune hemolytic anemia

Answer

Thalassemia major

Answer

Thalassemia minor

Answer

Thalassemia intermedia

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

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 5

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

Question 6

A 56-year-old female presents with headache, dizziness, generalized itching, especially after hot showers, and intense burning in her hands and feet. She reports relief with aspirin. Physical examination reveals splenomegaly and elevated blood pressure. Investigations show HB: 20.1 g/dl, Hematocrit: 60%, WBC: 15800, Platelet count: 500,000/µl, low spO2: 98%, and increased LAP. Which of the following is the most common mutation seen in the condition described?

Accepted Answer

JAK2

Answer

CALR

Answer

RAS

Answer

MYC

Question 7

A decrease in serum iron and a decrease in total iron-binding capacity (TIBC) is seen in which condition?

Accepted Answer

Anemia of chronic disease

Answer

Iron deficiency anemia

Answer

Thalassemia

Answer

Sideroblastic anemia

Question 8

A young female presents with a history of abdominal pain, headache, nausea, and vomiting, along with sudden loss of movement of the right upper limb. What is the most likely diagnosis?

Accepted Answer

Acute intermittent porphyria

Answer

Conversion disorder

Answer

Migraine

Answer

Right lower limb paralysis

Question 9

A patient presents with a platelet count of 700x10^9/L with abnormalities in size, shape, and granularity of platelets. The WBC count is 12x10^9/L, hemoglobin is 11g/dL, and the Philadelphia chromosome is absent. What is the most likely diagnosis?

Accepted Answer

Essential thrombocythemia

Answer

Polycythemia vera

Answer

Chronic myeloid leukemia

Answer

Leukemoid reaction

Question 10

A 23-year-old female has presented with anemia and jaundice for 2 years. Peripheral smear shows spherocytes. What is the most appropriate investigation to be done?

Accepted Answer

Coombs test

Answer

Reticulocyte count

Answer

Osmotic fragility test

Answer

Bone marrow aspiration

Hematology — MCQs

Hematology — MCQs

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