Hematology Practice Questions

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: Which of the following statements regarding apheresis platelet donation is FALSE?

A person with a below-normal platelet count can donate.. ### Explanation **1. Why Option A is the Correct (False) Statement:** The primary prerequisite for apheresis platelet donation (Single Donor Platelets or SDP) is a **normal baseline platelet count**. According to standard guidelines (including WHO and NBTC India), a donor must have a platelet count of at least **1.5 lakh/mm³ (150,000/µL)**. Donating with a below-normal count poses a significant risk of post-procedure thrombocytopenia to the donor and results in an inadequate yield for the recipient. **2. Analysis of Other Options (True Statements):** * **Option B:** Platelets are replenished rapidly by the bone marrow. Therefore, a donor can safely donate again after an interval of **48 hours (2 days)**. * **Option C:** To prevent donor exhaustion and ensure safety, the frequency is limited to a maximum of **2 procedures per week**. * **Option D:** The cumulative annual limit for plateletpheresis is **24 donations per year** [1]. This is significantly higher than whole blood donation (which is once every 3 months) because red cell loss is minimal during apheresis [1]. **3. Clinical Pearls for NEET-PG:** * **Minimum Weight:** The donor should weigh at least **50 kg**. * **Aspirin Rule:** Donors must not have taken Aspirin or other NSAIDs (which irreversibly inhibit platelet function) for at least **48–72 hours** prior to donation. * **Yield:** One unit of SDP typically increases the recipient's platelet count by **30,000–60,000/µL**, whereas one unit of Random Donor Platelets (RDP) only increases it by 5,000–10,000/µL. * **Anticoagulant:** **Acid Citrate Dextrose (ACD)** is the anticoagulant used during the apheresis procedure; watch for signs of hypocalcemia (citrate toxicity) in the donor.

Q: In a patient with sickle cell anemia, in which of the following conditions is blood transfusion indicated?

All of the above. In Sickle Cell Anemia (SCA), blood transfusion is not a routine treatment for stable anemia but is strategically used to reduce the concentration of Hemoglobin S (HbS) and improve oxygen delivery [1]. The goal is typically to maintain HbS levels below 30-50% while keeping total hemoglobin around 10 g/dL. **Why "All of the Above" is correct:** Transfusion therapy in SCA is categorized into acute (emergency) and prophylactic (preventative) indications. * **Frequent Sickling Episodes (Option A):** Patients experiencing recurrent Vaso-occlusive Crises (VOC) that significantly impair quality of life benefit from chronic transfusion programs to suppress endogenous erythropoiesis and reduce the frequency of crises [1]. * **Twin Pregnancy (Option B):** Multiple gestations significantly increase the metabolic demand and risk of complications (like pre-eclampsia or severe anemia). Prophylactic transfusion is indicated to ensure fetal growth and maternal stability. * **Poor Obstetrical Outcome (Option C):** A history of previous fetal loss, preterm birth, or intrauterine growth restriction (IUGR) in a sickle cell patient warrants aggressive management, including transfusions, to optimize placental perfusion and prevent recurrent adverse outcomes. **Clinical Pearls for NEET-PG:** * **Absolute Indications for Exchange Transfusion:** Stroke (acute or primary prevention), Acute Chest Syndrome (severe), and Multi-organ failure [1]. * **Contraindications:** Do not transfuse for asymptomatic anemia or uncomplicated pain crises, as this increases the risk of **Iron Overload** and **Alloimmunization**. * **Target Hb:** Avoid increasing total Hb >10-11 g/dL to prevent hyperviscosity, which can paradoxically trigger a stroke.

Q: Which of the following is the most common presentation of hemophilia A?

hemarthrosis. **Explanation:** Hemophilia A is an X-linked recessive bleeding disorder caused by a deficiency of **Coagulation Factor VIII**. The hallmark of secondary hemostasis defects (clotting factor deficiencies) is deep-tissue bleeding, as opposed to the mucosal bleeding seen in primary hemostasis defects (platelet disorders) [3]. **Why Hemarthrosis is Correct:** **Hemarthrosis (bleeding into joint spaces)** is the most common clinical manifestation of Hemophilia A, occurring in approximately 75–90% of patients. The most frequently affected joints are the **knees** (most common), followed by elbows, ankles, shoulders, and hips [1]. Recurrent hemarthrosis leads to "Hemophilic Arthropathy," characterized by synovial hypertrophy and cartilage destruction [1]. **Analysis of Incorrect Options:** * **A & B (Hematuria and Melena):** While internal bleeding can occur in severe hemophilia, gastrointestinal and genitourinary bleeds are less common than musculoskeletal bleeds. They usually occur secondary to an underlying lesion or severe trauma. * **D (Pressure Neuropathy):** This is a known complication, typically occurring due to an **iliopsoas hematoma** compressing the femoral nerve [1]. While high-yield for exams, it is a specific complication rather than the most common presentation. **NEET-PG High-Yield Pearls:** * **Inheritance:** X-linked recessive (affects males; females are carriers). * **Lab Findings:** Prolonged **aPTT**, normal PT, normal bleeding time, and normal platelet count. * **Mixing Study:** aPTT will **correct** upon mixing with normal plasma (distinguishes deficiency from inhibitors). * **Treatment:** Recombinant Factor VIII concentrate [2]. Desmopressin (DDAVP) can be used in mild cases to release stored Factor VIII from endothelial cells [2].

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").

Q: Hypersplenism is associated with which of the following hematological findings?

Pancytopenia. ### Explanation **Concept Overview:** Hypersplenism is a clinical syndrome characterized by an overactive spleen that excessively sequesters and destroys circulating blood cells. It is defined by the classic **Dameshek’s Criteria**, which includes: 1. Splenomegaly [2]. 2. Reduction in one or more cell lines (anemia, leukopenia, and/or thrombocytopenia). 3. Normal or hypercellular bone marrow (compensatory response). 4. Correction of cytopenias following splenectomy. **Why Pancytopenia is Correct:** While hypersplenism can manifest as an isolated reduction in one cell line (most commonly thrombocytopenia), the hallmark of the syndrome is **Pancytopenia**. The enlarged spleen acts as a massive reservoir; normally, it holds 30% of the body's platelets, but in hypersplenism, it can sequester up to 90% [1]. Simultaneously, increased splenic macrophages lead to the premature destruction of erythrocytes and leukocytes. Therefore, a reduction in all three cell lines is the most characteristic finding. **Analysis of Incorrect Options:** * **B & C (Thrombocytopenia/Leukopenia):** These are components of hypersplenism, but they are incomplete. "Pancytopenia" is the more comprehensive and superior answer as it encompasses the reduction of all hematopoietic lineages. * **D (Polycythemia):** This is the opposite of hypersplenism. Polycythemia involves an increase in red cell mass, whereas hypersplenism leads to anemia. **NEET-PG High-Yield Pearls:** * **Most common cause:** Portal hypertension (due to Cirrhosis) is the leading cause of congestive splenomegaly and hypersplenism. * **Peripheral Smear:** Unlike hyposplenism (where you see Howell-Jolly bodies), hypersplenism typically shows a "clean" smear but with cytopenias. * **Treatment:** Splenectomy is only indicated if the cytopenia is symptomatic (e.g., severe bleeding or recurrent infections) or if the primary disease requires it.

Q: Macrocytic anemia is not seen in which of the following conditions?

Anemia of chronic disease. The classification of anemia is primarily based on the **Mean Corpuscular Volume (MCV)**. Macrocytic anemia is defined by an MCV >100 fL. **Why Anemia of Chronic Disease (ACD) is the correct answer:** ACD is typically a **normocytic, normochromic anemia**. In long-standing cases, it may progress to a **microcytic** pattern, but it is **never macrocytic** [2]. The pathophysiology involves high levels of **Hepcidin** (induced by IL-6), which sequesters iron in macrophages and decreases intestinal iron absorption, mimicking a functional iron deficiency [1]. **Why the other options are incorrect:** * **Vitamin B12 deficiency:** This is a classic cause of **megaloblastic macrocytic anemia** [1]. Lack of B12 impairs DNA synthesis, leading to nuclear-cytoplasmic asynchrony where the nucleus matures slower than the cytoplasm, resulting in large cells. * **Hemolytic Anemia & Post-hemorrhagic Anemia:** Both conditions trigger a robust bone marrow response, leading to **reticulocytosis** (increased young RBCs). Since reticulocytes are larger than mature erythrocytes, a high reticulocyte count falsely elevates the MCV, causing a **non-megaloblastic macrocytic** picture. **High-Yield Clinical Pearls for NEET-PG:** * **Megaloblastic Macrocytosis:** Characterized by hypersegmented neutrophils (≥5 lobes) on peripheral smear. Causes: B12/Folate deficiency, Drugs (Methotrexate, Phenytoin, Zidovudine). * **Non-megaloblastic Macrocytosis:** No hypersegmented neutrophils. Causes: Alcoholism (most common), Liver disease, Hypothyroidism, and Reticulocytosis. * **ACD Hallmark:** Low Serum Iron, **Low TIBC**, and **High/Normal Ferritin** (distinguishes it from Iron Deficiency Anemia where Ferritin is low).

Question 1

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 2

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 3

Which of the following statements regarding apheresis platelet donation is FALSE?

Accepted Answer

A person with a below-normal platelet count can donate.

Answer

A person can donate again after 2 days.

Answer

A person can donate a maximum of 2 times per week.

Answer

Not more than 24 donations can be done in 1 year.

Question 4

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 5

Which of the following is false regarding Transfusion-Related Acute Lung Injury (TRALI)?

Accepted Answer

All of the above

Answer

It is due to the release of mediators from neutrophils in the lungs.

Answer

It occurs within 6 hours of transfusion.

Answer

It occurs more commonly when the donor is a multiparous woman.

Question 6

In a patient with sickle cell anemia, in which of the following conditions is blood transfusion indicated?

Accepted Answer

All of the above

Answer

Frequent sickling episodes

Answer

Twin pregnancy

Answer

Poor obstetrical outcome

Question 7

Which of the following is the most common presentation of hemophilia A?

Accepted Answer

hemarthrosis

Answer

hematuria

Answer

melena

Answer

pressure neuropathy

Question 8

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

Question 9

Hypersplenism is associated with which of the following hematological findings?

Accepted Answer

Pancytopenia

Answer

Thrombocytopenia

Answer

Leukopenia

Answer

Polycythemia

Question 10

Macrocytic anemia is not seen in which of the following conditions?

Accepted Answer

Anemia of chronic disease

Answer

Vitamin B12 deficiency

Answer

Hemolytic anemia

Answer

Post-hemorrhagic anemia

Hematology — MCQs

Hematology — MCQs

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