Hematology Practice Questions

Q: JAK-2 mutation is strongly associated with which of the following conditions?

Polycythemia vera. **Explanation:** **JAK2 (Janus Kinase 2)** is a non-receptor tyrosine kinase involved in the signal transduction pathway of hematopoietic growth factor receptors (like erythropoietin and thrombopoietin). The **V617F mutation** leads to constitutive activation of this pathway, causing autonomous production of blood cells independent of growth factors. * **Polycythemia Vera (PV):** This is the correct answer because the JAK2 V617F mutation is found in **>95% of cases**. It is a major diagnostic criterion according to WHO classification. It is also found in Essential Thrombocythemia (ET) and Primary Myelofibrosis (PMF), but with lower frequency (~50-60%). **Why other options are incorrect:** * **Burkitt’s Lymphoma:** Characterized by the **t(8;14)** translocation involving the **c-myc** oncogene. * **Multiple Myeloma:** A plasma cell dyscrasia primarily associated with chromosomal abnormalities like trisomies or translocations involving the IgH locus (e.g., t(11;14), t(4;14)). * **Mantle Cell Lymphoma:** Defined by the **t(11;14)** translocation, leading to the overexpression of **Cyclin D1**. **High-Yield Clinical Pearls for NEET-PG:** 1. **JAK2 V617F:** Substitution of Valine (V) by Phenylalanine (F) at codon 617. 2. **PV Diagnosis:** Low serum Erythropoietin (EPO) levels + JAK2 mutation + Increased Red Cell Mass. 3. **Aquagenic Pruritus:** Itching after a hot bath is a classic clinical sign of PV. 4. **Treatment:** Ruxolitinib is a JAK1/2 inhibitor used in patients resistant to hydroxyurea.

Q: Platelet count is decreased in all of the following conditions except?

Henoch-Schönlein purpura. **Explanation:** The core concept tested here is the distinction between **quantitative** and **qualitative** platelet disorders. **1. Why Henoch-Schönlein Purpura (HSP) is the correct answer:** HSP is a **small-vessel vasculitis** mediated by IgA immune complex deposition. The hallmark of HSP is **palpable purpura** occurring in the presence of a **normal platelet count** [1]. The bleeding into the skin occurs because the blood vessel walls are inflamed and "leaky," not because platelets are deficient. Therefore, it is a vascular cause of purpura, not a thrombocytopenic one. **2. Analysis of Incorrect Options:** * **Idiopathic Thrombocytopenic Purpura (ITP):** Characterized by isolated thrombocytopenia due to anti-platelet antibodies (IgG) leading to premature destruction of platelets in the spleen [2]. * **Thrombotic Thrombocytopenic Purpura (TTP):** A pentad of symptoms (including microangiopathic hemolytic anemia and thrombocytopenia) caused by a deficiency of ADAMTS13, leading to extensive platelet-rich microthrombi that consume platelets [2]. * **Systemic Lupus Erythematosus (SLE):** An autoimmune condition where hematological abnormalities are common. Thrombocytopenia in SLE is usually immune-mediated (secondary ITP) or part of Evans syndrome [2]. **NEET-PG High-Yield Pearls:** * **HSP Tetrad:** Palpable purpura (buttocks/legs), Arthralgia, Abdominal pain (intussusception risk), and Renal involvement (IgA nephropathy). * **Platelet Count in Vasculitis:** Always remember that in most vasculitides (like HSP or Polyarteritis Nodosa), the platelet count is normal or even elevated (as an acute phase reactant) [1]. * **Rule of Thumb:** If the question mentions "palpable purpura," think Vasculitis (Normal Platelets). If it mentions "flat/non-palpable purpura/petechiae," think Thrombocytopenia (Low Platelets) [2].

Q: What is the earliest hematologic manifestation of megaloblastic anemia?

Hypersegmented polymorphs. **Explanation:** The earliest hematologic manifestation of megaloblastic anemia is the appearance of **hypersegmented neutrophils (polymorphs)** on the peripheral blood smear. **1. Why Hypersegmented Polymorphs are Correct:** Megaloblastic anemia results from impaired DNA synthesis (most commonly due to Vitamin B12 or Folate deficiency), while RNA and protein synthesis remain intact [1]. This leads to "nuclear-cytoplasmic asynchrony." In the bone marrow, granulocytic precursors are affected early. A neutrophil is considered hypersegmented if it has **$\geq$ 6 lobes** or if more than **5% of neutrophils have $ageq$ 5 lobes**. This change often precedes the development of macrocytosis and the drop in hemoglobin levels. **2. Analysis of Incorrect Options:** * **Increase in MCV (Macrocytosis):** While a hallmark of the disease, an increase in Mean Corpuscular Volume (MCV) typically occurs *after* the appearance of hypersegmented neutrophils. It is a sensitive indicator but not the earliest. * **Thrombocytopenia & Leukopenia:** These occur in later, more severe stages of the disease due to ineffective hematopoiesis affecting all cell lines (pancytopenia). They are never the initial manifestations. **3. High-Yield Clinical Pearls for NEET-PG:** * **Earliest Sign:** Hypersegmented neutrophils. * **Earliest Bone Marrow Change:** Megaloblastic erythropoiesis (specifically, the presence of megaloblasts with "open" sieve-like chromatin). * **MCV in Megaloblastic Anemia:** Usually >110 fL. * **Biochemical Markers:** Elevated **Homocysteine** (seen in both B12 and Folate deficiency) and elevated **Methylmalonic Acid (MMA)** (specific to B12 deficiency). * **Ineffective Erythropoiesis:** Leads to increased indirect bilirubin and LDH (mimicking hemolysis).

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.

Question 1

JAK-2 mutation is strongly associated with which of the following conditions?

Accepted Answer

Polycythemia vera

Answer

Burkitt's lymphoma

Answer

Multiple myeloma

Answer

Mantle cell lymphoma

Question 2

A 68-year-old patient developed atrophic gastritis and, 2 years later, developed a macrocytic, hyperchromic anemia. His anemia has most likely occurred due to which one of the following reasons?

Accepted Answer

The atrophic gastritis leads to vitamin B12 malabsorption.

Answer

The atrophic gastritis raises the pH in the duodenum, leading to folate malabsorption.

Answer

The terminal ileum is also involved, so iron is malabsorbed.

Answer

The atrophic gastritis leads to increased red blood cell absorption by the spleen.

Question 3

A 54-year-old man presents with minor fatigue on exertion for the past 9 months. Physical examination reveals no remarkable findings. Laboratory studies show hemoglobin of 11.7 g/dL, hematocrit of 34.8%, MCV of 73 mm3, platelet count of 315,000/mm3, and WBC count of 8035/mm3. Which of the following is the most sensitive and cost-effective test to help determine the cause of these findings?

Accepted Answer

Serum ferritin

Answer

Bone marrow biopsy

Answer

Hemoglobin electrophoresis

Answer

Serum haptoglobin

Question 4

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

Accepted Answer

Low arterial oxygen saturation

Answer

Increased red cell mass

Answer

Presence of JAK2 mutation

Answer

Splenomegaly

Question 5

What is the most common cause of precipitation of renal failure in multiple myeloma?

Accepted Answer

Hypercalcemia

Answer

Hyperuricemia

Answer

Infection

Answer

Amyloidosis

Question 6

Platelet count is decreased in all of the following conditions except?

Accepted Answer

Henoch-Schönlein purpura

Answer

Idiopathic thrombocytopenic purpura

Answer

Thrombotic thrombocytopenic purpura

Answer

Systemic lupus erythematosus

Question 7

What is the earliest hematologic manifestation of megaloblastic anemia?

Accepted Answer

Hypersegmented polymorphs

Answer

Increase in MCV

Answer

Thrombocytopenia

Answer

Leukopenia

Question 8

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 9

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 10

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

Hematology — MCQs

Hematology — MCQs

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