Hematology Practice Questions

Q: A 50-year-old patient presents with abdominal discomfort for the last 2 years and a large spleen weighing 3 kg. Bone marrow aspiration is difficult. What is the most probable diagnosis?

Myelofibrosis. ### Explanation The clinical presentation of massive splenomegaly (3 kg) and a "difficult" bone marrow aspiration (Dry Tap) is a classic triad for **Primary Myelofibrosis (PMF)** [1]. **1. Why Myelofibrosis is correct:** * **Massive Splenomegaly:** PMF is characterized by extensive extramedullary hematopoiesis (EMH), leading to some of the largest spleens seen in clinical practice (often crossing the midline) [1]. * **Dry Tap:** The hallmark of PMF is reactive fibroblast proliferation and collagen deposition in the bone marrow [1]. This fibrosis makes it impossible to aspirate marrow fluid, resulting in a "dry tap." Diagnosis is confirmed via a bone marrow trephine biopsy showing increased reticulin or collagen fibrosis [1]. **2. Why the other options are incorrect:** * **Sickle Cell Anemia:** In adults, this typically presents with a small, shrunken, and fibrotic spleen (**autosplenectomy**) due to repeated infarctions, rather than massive splenomegaly. * **Hemochromatosis:** While it can cause hepatomegaly and occasionally mild splenomegaly (due to portal hypertension from cirrhosis), it does not cause a 3 kg spleen or bone marrow fibrosis. * **Iron Deficiency Anemia:** This usually presents with a normal-sized spleen. If splenomegaly is present, it is mild and occurs in only about 10% of cases. **3. NEET-PG High-Yield Pearls:** * **Causes of Massive Splenomegaly:** Myelofibrosis, Chronic Myeloid Leukemia (CML), Visceral Leishmaniasis (Kala-azar), Malaria (Hyperreactive Malarial Splenomegaly), and Gaucher’s disease [1]. * **Peripheral Smear in PMF:** Look for **Leukoerythroblastic picture** (immature WBCs and RBCs) and **Teardrop RBCs (Dacrocytes)** [1]. * **Common Causes of Dry Tap:** Myelofibrosis, Hairy Cell Leukemia, Secondary deposits (Metastasis), and Aplastic Anemia.

Q: A 32-year-old male is diagnosed with acute myeloid leukemia. His total WBC count was less than normal initially. Which of the following factors indicates a poor prognosis in AML?

Monosomies of chromosomes. **Explanation:** The prognosis of Acute Myeloid Leukemia (AML) is primarily determined by cytogenetic and molecular abnormalities. **1. Why Monosomies are the correct answer:** Cytogenetics is the most important prognostic factor in AML. **Monosomal karyotype (MK)**, defined as the presence of two or more distinct autosomal monosomies or a single monosomy in the presence of other structural abnormalities, is associated with an extremely poor prognosis and very low survival rates [1]. Specifically, monosomies of chromosomes 5 or 7 (-5, -7) are classic indicators of high-risk disease and poor response to standard chemotherapy [1]. **2. Analysis of Incorrect Options:** * **Young age:** Age is a significant prognostic factor, but **younger age (<60 years)** is generally a **favorable** factor [1]. Older patients often have poor performance status and higher rates of adverse cytogenetics. * **t(15;17):** This translocation is characteristic of **Acute Promyelocytic Leukemia (APL/M3)**. It is considered a **favorable-risk** cytogenetic marker because it responds exceptionally well to All-trans Retinoic Acid (ATRA) and Arsenic Trioxide [1]. * **Low WBC count:** In AML, a **high initial WBC count** (typically >50,000–100,000/µL) is a marker of high tumor burden and is associated with a **poor** prognosis. A low or normal initial WBC count is not a poor prognostic indicator. **Clinical Pearls for NEET-PG:** * **Favorable Prognosis:** t(8;21), inv(16), t(15;17), and NPM1 mutation (without FLT3-ITD) [1]. * **Poor Prognosis:** Monosomy 5 or 7, del(5q), 11q23 abnormalities, and **FLT3-ITD** mutations [1]. * **Auer Rods:** Pathognomonic for AML (especially M3); they are clumps of azurophilic granules. * **Treatment of choice for M3:** ATRA + Arsenic Trioxide.

Q: A patient has a Hemoglobin of 6 gm%. Which of the following should not be administered?

Fresh frozen plasma. **Explanation:** The patient presents with a Hemoglobin (Hb) of **6 gm%**, which indicates significant anemia. According to standard clinical guidelines, a Hb level below 7 gm% is a common threshold for a blood transfusion to restore oxygen-carrying capacity. **Why Fresh Frozen Plasma (FFP) is the Correct Answer:** FFP contains all coagulation factors but **does not contain red blood cells (RBCs)**. Therefore, it has no oxygen-carrying capacity and cannot correct anemia. Administering FFP to a patient with isolated anemia is inappropriate and carries risks of volume overload (TACO) and transfusion-related acute lung injury (TRALI) without providing any therapeutic benefit for the low hemoglobin. **Analysis of Incorrect Options:** * **Packed Red Cells (PRBCs):** This is the **treatment of choice** for symptomatic anemia. It provides a concentrated dose of RBCs to increase Hb levels while minimizing volume. * **Whole Blood:** While less commonly used today than component therapy, whole blood contains RBCs and can effectively raise hemoglobin levels, especially in cases of acute massive hemorrhage. * **Random Donor Platelets (RDP):** While RDPs are used for thrombocytopenia, they are not "contraindicated" in the same sense as FFP for anemia. However, in the context of this question, FFP is the most distinctively incorrect choice because it is often misused as a "volume expander," which is a major clinical pitfall. **NEET-PG High-Yield Pearls:** * **1 unit of PRBC** increases Hb by **1 gm/dL** and Hematocrit by **3%** in an average adult. * **FFP Indications:** Correction of multiple coagulation factor deficiencies (e.g., liver disease, DIC, Warfarin reversal), not for volume expansion or nutritional support. * **Transfusion Threshold:** Generally **7 gm/dL** in stable patients and **8 gm/dL** in patients with preexisting cardiovascular disease.

Q: A person presents with severe pruritis while taking a hot shower and deep vein thrombosis. Laboratory investigations reveal a JAK 2 mutation and hypertension. What is the most likely diagnosis?

Polycythemia Vera. ### Explanation The clinical presentation of **Polycythemia Vera (PV)** is classically defined by the triad of erythrocytosis, thrombotic complications, and specific systemic symptoms. **1. Why Polycythemia Vera is Correct:** * **Aquagenic Pruritus:** Severe itching after a hot shower is a pathognomonic symptom of PV, caused by mast cell degranulation and histamine release triggered by temperature changes [2]. * **Thrombosis:** Increased blood viscosity and hypercellularity lead to both arterial (hypertension, stroke) and venous (DVT, Budd-Chiari syndrome) thrombosis [2]. * **JAK2 Mutation:** Over 95% of PV cases are associated with the **JAK2 V617F mutation**, which leads to erythropoietin-independent proliferation of red blood cells [2]. **2. Why Other Options are Incorrect:** * **Myelofibrosis:** While JAK2 positive, it typically presents with massive splenomegaly, "teardrop" RBCs (dacrocytes), and cytopenias due to bone marrow fibrosis, rather than erythrocytosis [1]. * **Chronic Myeloid Leukemia (CML):** Characterized by the **Philadelphia chromosome [t(9;22)]** and BCR-ABL fusion gene. It presents with marked leukocytosis (neutrophils, basophils) rather than isolated erythrocytosis. * **Acute Myeloid Leukemia (AML):** An aggressive malignancy defined by >20% blasts in the bone marrow. Patients present with "bone marrow failure" (anemia, infections, bleeding) rather than thrombotic events and pruritus. **Clinical Pearls for NEET-PG:** * **First-line treatment:** Therapeutic phlebotomy (Target Hematocrit <45%). * **Low Serum Erythropoietin (EPO):** A key diagnostic marker to differentiate PV from secondary polycythemia (where EPO is high) [2]. * **Hyperuricemia:** Common in PV due to high cell turnover, potentially leading to secondary Gout [2].

Q: A 38-year-old man presented with episodes of severe headache. CNS imaging shows evidence of cortical sinus venous thrombosis (CSVT). His mother has a history of spontaneous recurrent deep venous thrombosis of bilateral lower limbs. Which of the following tests is the least likely to be indicated in this patient?

Antiphospholipid antibodies. The patient presents with **Cortical Sinus Venous Thrombosis (CSVT)** and a significant **family history** of recurrent venous thromboembolism (VTE) [2]. This clinical picture strongly suggests an **inherited (hereditary) thrombophilia**. [1] **Why Option A is the Correct Answer:** **Antiphospholipid Antibody Syndrome (APS)** is an **acquired** thrombophilia. While it is a common cause of CSVT, it is typically sporadic and does not follow a strong hereditary pattern. Since the question highlights a strong family history (mother with recurrent DVT), the diagnostic focus should be on genetic/inherited causes rather than acquired ones. Therefore, testing for APLA is the *least* likely to be the primary investigation in a suspected familial case. **Analysis of Incorrect Options:** * **B. Factor V Leiden Mutation:** This is the **most common inherited thrombophilia** worldwide. It causes resistance to activated protein C and is a classic cause of familial VTE and CSVT. * **C. Plasma Homocysteine Level:** Hyperhomocysteinemia can be caused by genetic mutations (e.g., MTHFR gene) and is a known risk factor for both arterial and venous thrombosis, including CSVT. * **D. Plasma Antithrombin Level:** Inherited Antithrombin III deficiency is a potent genetic risk factor for thrombosis [1]. While rarer than Factor V Leiden, it carries a very high risk of VTE. **NEET-PG Clinical Pearls:** * **Most common inherited cause of CSVT:** Factor V Leiden mutation. * **Most common acquired cause of CSVT:** Oral contraceptive pills (OCPs) or Pregnancy/Puerperium. * **Gold Standard Investigation for CSVT:** MR Venogram (MRV). * **Treatment of Choice:** Low Molecular Weight Heparin (LMWH), even in the presence of hemorrhagic infarcts. * **Inherited vs. Acquired:** Always look for "family history" in the stem to differentiate between genetic (Factor V, Protein C/S, Antithrombin) and acquired (APLA, Malignancy) states.

Q: Which of the following is used to treat Paroxysmal nocturnal hemoglobinuria?

Eculizumab. ### Explanation **Correct Option: A. Eculizumab** Paroxysmal Nocturnal Hemoglobinuria (PNH) is an acquired clonal hematopoietic stem cell disorder characterized by a deficiency of glycosylphosphatidylinositol (GPI) anchored proteins (like CD55 and CD59) on red blood cells. This deficiency makes RBCs highly susceptible to complement-mediated lysis. **Eculizumab** is a humanized monoclonal antibody that binds to the **C5 complement protein**. By inhibiting the cleavage of C5 into C5a and C5b, it prevents the formation of the **Membrane Attack Complex (MAC)**, thereby stopping intravascular hemolysis in PNH patients. **Incorrect Options:** * **B. Avelumab:** A programmed death-ligand 1 (PD-L1) blocking antibody used in the treatment of Merkel cell carcinoma and urothelial carcinoma. * **C. Natalizumab:** An alpha-4 ($\alpha$4) integrin antagonist used primarily in Multiple Sclerosis and Crohn’s disease. It prevents leukocyte adhesion and migration into inflamed tissue. * **D. Sarilumab:** An Interleukin-6 (IL-6) receptor antagonist used in the treatment of moderate-to-severe Rheumatoid Arthritis. **High-Yield Clinical Pearls for NEET-PG:** * **Black Box Warning:** Eculizumab significantly increases the risk of **Meningococcal infections**. Patients must be vaccinated against *Neisseria meningitidis* at least 2 weeks prior to starting therapy. * **Ravulizumab:** A long-acting C5 inhibitor (longer half-life than Eculizumab) also approved for PNH. * **Pegcetacoplan:** A newer agent that targets **C3**, used for patients who still have clinically significant anemia while on C5 inhibitors. * **Triad of PNH:** Hemolytic anemia, Pancytopenia, and Venous thrombosis (Budd-Chiari syndrome is a common complication).

Q: All-trans retinoic acid is useful in the treatment of which hematologic malignancy?

Promyelocytic leukemia. **Acute Promyelocytic Leukemia (APL)**, a subtype of Acute Myeloid Leukemia (FAB classification M3), is characterized by a specific chromosomal translocation **t(15;17)** [1]. This translocation fuses the Promyelocytic Leukemia (*PML*) gene with the Retinoic Acid Receptor Alpha (*RARα*) gene. The resulting PML-RARα fusion protein blocks myeloid differentiation at the promyelocyte stage by repressing gene transcription. **All-trans retinoic acid (ATRA)** works by binding to this fusion receptor, inducing the malignant promyelocytes to differentiate into mature neutrophils (differentiation therapy), thereby inducing remission. **Analysis of Incorrect Options:** * **A. Myelodysplastic Leukemia:** This refers to AML arising from MDS. It typically involves complex cytogenetics (e.g., deletions of chromosome 5 or 7) and does not involve the RARα receptor, making it unresponsive to ATRA [1]. * **C. Myelomonocytic Leukemia (AML-M4):** This subtype is characterized by both granulocytic and monocytic differentiation, often associated with inv(16) [1]. It lacks the t(15;17) translocation. * **D. Chronic Myelocytic Leukemia (CML):** CML is defined by the **Philadelphia chromosome t(9;22)** and the *BCR-ABL1* fusion gene [1]. The standard of care is Tyrosine Kinase Inhibitors (TKIs) like Imatinib, not ATRA. **High-Yield Clinical Pearls for NEET-PG:** * **DOC:** The current standard treatment for APL is a combination of **ATRA + Arsenic Trioxide (ATO)**. * **Emergency:** APL is a medical emergency due to the high risk of **DIC (Disseminated Intravascular Coagulation)** triggered by the release of procoagulants from granules. * **Side Effect:** Watch for **Differentiation Syndrome** (fever, dyspnea, pulmonary infiltrates), treated with high-dose Dexamethasone. * **Morphology:** Look for **Auer rods** (often in clumps called "Faggot cells").

Question 1

A 59-year-old male patient presents with multiple neck swellings, fever, and weight loss. General examination reveals painless lymph node enlargement in the neck, and blood investigations show anemia. Serum albumin is 32 gm/l. Lymph node biopsy shows Reed-Sternberg cells. What is the 5-year rate of overall survival (in %) for this patient, according to the Hasenclever prognostic index for advanced Hodgkin lymphoma?

Accepted Answer

74

Answer

90

Answer

70

Answer

59

Question 2

A 50-year-old patient presents with abdominal discomfort for the last 2 years and a large spleen weighing 3 kg. Bone marrow aspiration is difficult. What is the most probable diagnosis?

Accepted Answer

Myelofibrosis

Answer

Sickle cell anemia

Answer

Hemochromatosis

Answer

Iron deficiency anemia

Question 3

A 32-year-old male is diagnosed with acute myeloid leukemia. His total WBC count was less than normal initially. Which of the following factors indicates a poor prognosis in AML?

Accepted Answer

Monosomies of chromosomes

Answer

Young age

Answer

Patients with t(15;17)

Answer

Low WBC count

Question 4

A patient has a Hemoglobin of 6 gm%. Which of the following should not be administered?

Accepted Answer

Fresh frozen plasma

Answer

Whole blood

Answer

Random donor platelets

Answer

Packed red cells

Question 5

A person presents with severe pruritis while taking a hot shower and deep vein thrombosis. Laboratory investigations reveal a JAK 2 mutation and hypertension. What is the most likely diagnosis?

Accepted Answer

Polycythemia Vera

Answer

Myelofibrosis

Answer

Chronic Myeloid Leukemia (CML)

Answer

Acute Myeloid Leukemia (AML)

Question 6

A 22-year-old man presents with a history of bleeding from gums for the last 6 months. On investigation, the hemoglobin was found to be 8.2 gm%, TLC 4400/mm
3, DLC N 64%, L 27%, E 3%, M 6%, and platelet count of 20,000/cu mm. Which one of the following investigations would be most useful in establishing the diagnosis?

Accepted Answer

Bone marrow examination

Answer

Bleeding time and clotting time

Answer

Prothrombin time

Answer

Partial thromboplastin time

Question 7

A 38-year-old man presented with episodes of severe headache. CNS imaging shows evidence of cortical sinus venous thrombosis (CSVT). His mother has a history of spontaneous recurrent deep venous thrombosis of bilateral lower limbs. Which of the following tests is the least likely to be indicated in this patient?

Accepted Answer

Antiphospholipid antibodies

Answer

Factor V Leiden mutation

Answer

Plasma homocysteine level

Answer

Plasma antithrombin level

Question 8

Low serum haptoglobin in hemolysis can be masked by which of the following conditions?

Accepted Answer

Bile duct obstruction

Answer

Liver disease

Answer

Malnutrition

Answer

Pregnancy

Question 9

Which of the following is used to treat Paroxysmal nocturnal hemoglobinuria?

Accepted Answer

Eculizumab

Answer

Avelumab

Answer

Natalizumab

Answer

Sarilumab

Question 10

All-trans retinoic acid is useful in the treatment of which hematologic malignancy?

Accepted Answer

Promyelocytic leukemia

Answer

Myelodysplastic leukemia

Answer

Myelomonocytic leukemia

Answer

Chronic myelocytic leukemia

Hematology — MCQs

Hematology — MCQs

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