Hematology Practice Questions

Q: Which of the following is NOT a feature of polycythemia vera?

None of the above. **Explanation:** Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by the autonomous production of red blood cells, independent of erythropoietin levels. The correct answer is **None of the above** because all three options (A, B, and C) are classic features of the disease. 1. **Increased Red Cell Mass (Option A):** This is the hallmark of PV [1]. Unlike relative polycythemia (caused by dehydration), PV involves a true absolute increase in the total number of erythrocytes, leading to hyperviscosity. 2. **Normal Arterial Oxygen Saturation (Option B):** This is a crucial diagnostic differentiator. In secondary polycythemia (caused by chronic lung disease or high altitude), arterial oxygen saturation ($SaO_2$) is typically low ($ 92\%$) because the erythropoiesis is primary and neoplastic. 3. **High Leukocyte Alkaline Phosphatase (LAP) Score (Option C):** PV is a panmyelosis. Along with red cells, there is often a reactive increase in mature granulocytes. This results in an elevated LAP score, which helps distinguish PV from Chronic Myeloid Leukemia (CML), where the LAP score is characteristically low. **High-Yield Clinical Pearls for NEET-PG:** * **JAK2 Mutation:** $>95\%$ of PV patients carry the **JAK2 V617F** mutation in exon 14 [1]. * **Erythropoietin (EPO) Levels:** Characteristically **low** in PV (negative feedback), whereas they are high in secondary polycythemia. * **Clinical Sign:** **Aquagenic pruritus** (itching after a warm bath) is a highly specific symptom [1]. * **Major Complications:** Thrombosis (Budd-Chiari syndrome) and transformation to myelofibrosis or Acute Myeloid Leukemia (AML) [1].

Q: Which enzyme is used in the treatment of Leukemia?

Asparaginase. **Explanation:** **L-Asparaginase** is a cornerstone enzyme therapy used primarily in the treatment of **Acute Lymphoblastic Leukemia (ALL)** [1]. **Mechanism of Action:** Normal cells can synthesize the amino acid **L-asparagine** from aspartate using the enzyme *asparagine synthetase*. However, leukemic lymphoblasts are deficient in this enzyme and rely entirely on exogenous (extracellular) sources of asparagine for protein synthesis. L-Asparaginase catalyzes the conversion of serum asparagine into aspartic acid and ammonia. By depleting the circulating pool of asparagine, the enzyme "starves" the leukemic cells, leading to inhibited protein synthesis and subsequent apoptosis. **Analysis of Incorrect Options:** * **B. Lipase:** An enzyme that breaks down dietary fats into fatty acids and glycerol. It is a diagnostic marker for acute pancreatitis but has no role in leukemia therapy. * **C. Amylase:** Responsible for the hydrolysis of starch into sugars. Like lipase, it is used to diagnose pancreatic pathology. * **D. Transaminase (AST/ALT):** These enzymes are involved in amino acid metabolism and serve as markers of hepatocellular injury; they are not therapeutic agents [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Primary Indication:** Induction protocol of childhood ALL [1]. * **Unique Side Effects:** 1. **Hypofibrinogenemia & Thrombosis:** Due to decreased synthesis of clotting factors (Protein C, S, and Antithrombin III). 2. **Acute Pancreatitis:** A classic board-exam association. 3. **Anaphylaxis:** Since it is a bacterial product (derived from *E. coli* or *Erwinia*), it is highly immunogenic. * **Cell Cycle Specificity:** It is **G1 phase-specific**.

Q: A 23-year-old man presents with prolonged nosebleeds. He has always noted easy bruising and ongoing bleeding after minor cuts. There is no prior history of surgery or dental procedures. His hemoglobin is 14.5 g/dL, platelets are 200,000/mL, and PT/PTT are normal. Further testing reveals that the bleeding time is elevated; the Factor VIII level is reduced, as is the ristocetin cofactor assay. What is the most likely diagnosis for this patient with a bleeding disorder?

von Willebrand's disease. ### Explanation **Correct Answer: A. von Willebrand's disease (vWD)** The patient presents with a classic **mucocutaneous bleeding pattern** (epistaxis, easy bruising, prolonged bleeding from minor cuts) [1]. The key to the diagnosis lies in the laboratory profile: 1. **Normal Platelet Count:** Rules out thrombocytopenia. 2. **Elevated Bleeding Time:** Indicates a defect in primary hemostasis (platelet adhesion) [2]. 3. **Reduced Ristocetin Cofactor Assay:** This is the most specific test for vWD; it measures the ability of von Willebrand Factor (vWF) to agglutinate platelets [1]. 4. **Reduced Factor VIII:** vWF acts as a carrier protein for Factor VIII, protecting it from degradation [1]. Therefore, a deficiency in vWF often leads to a secondary decrease in Factor VIII. **Why Incorrect Options are Wrong:** * **B & C (Hemophilia A & B):** These are disorders of secondary hemostasis (clotting factors). They typically present with deep-tissue bleeding (hemarthrosis, muscle hematomas) rather than mucosal bleeding [1]. In Hemophilia, the Bleeding Time is **normal**, but the **aPTT is prolonged**. * **D (TTP):** TTP is characterized by the pentad of microangiopathic hemolytic anemia, **thrombocytopenia** (which is absent here), neurological symptoms, fever, and renal failure. **High-Yield Clinical Pearls for NEET-PG:** * **Inheritance:** vWD is the most common inherited bleeding disorder (usually Autosomal Dominant). * **vWF Functions:** 1) Platelet-to-subendothelial adhesion (via GpIb receptor); 2) Carrier for Factor VIII [1]. * **Lab Findings:** Normal PT, Normal/Prolonged aPTT (depending on Factor VIII levels), and prolonged Bleeding Time/PFA-100. * **Treatment:** **Desmopressin (DDAVP)** is the drug of choice for Type 1 vWD as it releases stored vWF from Weibel-Palade bodies in endothelial cells [1].

Q: If a blood transfusion reaction develops due to incompatibility, what is the first immediate action to be taken?

Stop the transfusion. ### Explanation **1. Why "Stop the Transfusion" is Correct:** The most critical step in managing any suspected transfusion reaction—whether it is a simple febrile reaction or a life-threatening acute hemolytic reaction—is to **immediately stop the transfusion** [1]. This prevents further exposure to the offending antigen or antibody, limiting the severity of the immune response. Following this, the IV line must be maintained with normal saline using a new administration set to ensure vascular access for emergency medications [1]. **2. Analysis of Incorrect Options:** * **B & C (Hydrocortisone and Chlorpheniramine):** These are secondary treatments. Antihistamines (Chlorpheniramine) are used for allergic reactions (Urticaria), and steroids (Hydrocortisone) are used for severe allergic or febrile reactions. However, they should never be administered before stopping the offending agent. * **D (Furosemide):** This is indicated in cases of Transfusion-Associated Circulatory Overload (TACO) to reduce fluid volume or to maintain renal blood flow in hemolytic reactions. It is a supportive measure, not the primary immediate action. **3. NEET-PG High-Yield Clinical Pearls:** * **Acute Hemolytic Transfusion Reaction (AHTR):** Most commonly due to **ABO incompatibility** (clerical error). Classic triad: Fever, chills, and flank pain [1]. * **First Step:** Stop transfusion $\rightarrow$ Check vitals $\rightarrow$ Maintain IV access with NS $\rightarrow$ Notify blood bank [1]. * **Investigation:** Perform a **Direct Antiglobulin Test (DAT/Coombs test)** on the post-transfusion sample and check for hemoglobinuria [1]. * **Febrile Non-Hemolytic Reaction (FNHRT):** The most common reaction overall; caused by cytokines released from donor leukocytes. Prevented by **leukoreduction** [1].

Q: What is the classification proposed by the International Lymphoma Study Group for non-Hodgkin's lymphoma?

REAL classification. The **REAL (Revised European-American Lymphoma) classification** was proposed in 1994 by the **International Lymphoma Study Group (ILSG)**. This classification marked a paradigm shift in hematopathology by defining lymphomas as distinct clinico-pathologic entities based on a combination of morphology, immunophenotype, genetic features, and clinical presentation, rather than just histological patterns. **Analysis of Options:** * **REAL Classification (Correct):** It was specifically developed by the ILSG to resolve discrepancies between previous systems and forms the foundational basis for the current WHO classification. * **Kiel Classification:** Developed by Lennert in Europe, this system focused primarily on the cytology (cell of origin) and divided lymphomas into low-grade and high-grade based on the presence of "blasts." * **WHO Classification:** While the WHO classification is the current "gold standard," it is actually an **extension and update** of the REAL classification [1]. It was not the original proposal by the ILSG but a subsequent international consensus. * **Rappaport Classification:** This is an older, obsolete system (1956) based purely on architectural patterns (nodular vs. diffuse) and cell size, which failed to account for B-cell or T-cell lineage. **NEET-PG High-Yield Pearls:** * The **REAL classification** was the first to recognize that lymphomas are distinct diseases, not just different grades of the same process. * The **Working Formulation** (1982) was another historical system used primarily for clinical trials, categorizing lymphomas by prognosis (Low, Intermediate, High grade). * **Current Standard:** The 5th edition of the **WHO Classification of Haematolymphoid Tumours (2022)** is the most recent update used in clinical practice today [1].

Q: Autoimmune hemolytic anemia is a feature of which of the following conditions?

Lymphoma. Autoimmune Hemolytic Anemia (AIHA), specifically the **Warm-antibody type (IgG)**, is a well-recognized paraneoplastic manifestation of B-cell malignancies [1]. **1. Why Lymphoma is Correct:** Lymphoproliferative disorders, particularly **Chronic Lymphocytic Leukemia (CLL)** and **Non-Hodgkin Lymphomas (NHL)** (such as Small Lymphocytic Lymphoma), are the most common neoplastic causes of AIHA [1]. The underlying mechanism involves the production of autoantibodies by dysfunctional B-lymphocytes against Rh antigens on the red blood cell surface, leading to extravascular hemolysis in the spleen [1]. In NEET-PG contexts, "Lymphoma" is the classic association for secondary AIHA. **2. Analysis of Incorrect Options:** * **ALL (Acute Lymphoblastic Leukemia):** While it involves lymphoid cells, AIHA is exceptionally rare in ALL. The primary hematological issues here are bone marrow failure (anemia, thrombocytopenia) due to blast crowding. * **CML (Chronic Myeloid Leukemia):** This is a myeloproliferative neoplasm. Anemia in CML is typically due to marrow replacement or hypersplenism, not autoimmune destruction. * **Burkitt’s Lymphoma:** Although a B-cell lymphoma, it is characterized by extremely rapid cell turnover and "starry sky" morphology. It is not classically associated with AIHA compared to indolent B-cell lymphomas [2]. **Clinical Pearls for NEET-PG:** * **CLL** is the single most common leukemia associated with AIHA (up to 10% of patients) [1]. * **Evans Syndrome:** The combination of AIHA and Immune Thrombocytopenic Purpura (ITP). * **Diagnosis:** The **Direct Antiglobulin Test (Direct Coombs Test)** is the gold standard for diagnosing AIHA [1]. * **Other Associations:** Systemic Lupus Erythematosus (SLE) is the most common non-malignant cause of secondary warm AIHA.

Question 1

Which of the following is NOT a feature of polycythemia vera?

Accepted Answer

None of the above

Answer

Increased red cell mass

Answer

Normal arterial oxygen saturation

Answer

High leukocyte alkaline phosphatase score

Question 2

Which enzyme is used in the treatment of Leukemia?

Accepted Answer

Asparaginase

Answer

Lipase

Answer

Amylase

Answer

Transaminase

Question 3

A 23-year-old man presents with prolonged nosebleeds. He has always noted easy bruising and ongoing bleeding after minor cuts. There is no prior history of surgery or dental procedures. His hemoglobin is 14.5 g/dL, platelets are 200,000/mL, and PT/PTT are normal. Further testing reveals that the bleeding time is elevated; the Factor VIII level is reduced, as is the ristocetin cofactor assay. What is the most likely diagnosis for this patient with a bleeding disorder?

Accepted Answer

von Willebrand's disease

Answer

hemophilia A

Answer

hemophilia B

Answer

thrombotic thrombocytopenic purpura (TTP)

Question 4

Which action by a nursing assistant when caring for a patient who is pancytopenic indicates a need for the nurse to intervene?

Accepted Answer

The nursing assistant assists the patient to use dental floss after eating.

Answer

The nursing assistant makes an oral rinse using 1 teaspoon of salt in a liter of water.

Answer

The nursing assistant adds baking soda to the patient’s saline oral rinses.

Answer

The nursing assistant puts fluoride toothpaste on the patient’s toothbrush.

Question 5

Which of the following are causes of iron deficiency anemia?

Accepted Answer

Chronic renal failure, Celiac sprue, Hookworm, Carcinoma of the cecum

Answer

Chronic renal failure, Young male, Celiac sprue

Answer

Chronic renal failure, Young male, Celiac sprue, Hookworm

Answer

Chronic renal failure, Young male, Hookworm, Carcinoma of the cecum

Question 6

What is the treatment of choice for aplastic anemia?

Accepted Answer

Bone marrow transplantation

Answer

Blood transfusion

Answer

Oxymethalone

Answer

Azathioprine

Question 7

If a blood transfusion reaction develops due to incompatibility, what is the first immediate action to be taken?

Accepted Answer

Stop the transfusion

Answer

Administer hydrocortisone injection

Answer

Administer chlorpheniramine maleate injection

Answer

Administer furosemide injection

Question 8

A patient has Hb 6 gm%, folic acid 82 ng/ml, vitamin B12 60 pg/ml, serum iron 180 
g/dL, and MCV of 104 fL. What is the most likely diagnosis?

Accepted Answer

Vitamin B12 deficiency

Answer

Iron deficiency anaemia

Answer

Folic acid deficiency

Answer

Pyridoxine deficiency

Question 9

What is the classification proposed by the International Lymphoma Study Group for non-Hodgkin's lymphoma?

Accepted Answer

REAL classification

Answer

Kiel classification

Answer

WHO classification

Answer

Rappapo classification

Question 10

Autoimmune hemolytic anemia is a feature of which of the following conditions?

Accepted Answer

Lymphoma

Answer

ALL

Answer

CML

Answer

Burkitt's Lymphoma

Hematology — MCQs

Hematology — MCQs

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