Hematopathology Practice Questions

Q: In polycythemia vera, which of the following is not elevated?

Erythropoietin. Polycythemia Vera (PV) is a chronic myeloproliferative neoplasm characterized by the autonomous, clonal overproduction of red blood cells. The primary pathology is a mutation in the JAK2 gene (most commonly V617F), which makes hematopoietic stem cells hypersensitive to growth factors, independent of physiological regulation. Why Erythropoietin (EPO) is the correct answer: In PV, the massive increase in red cell mass is "primary," meaning it is not driven by Erythropoietin. Due to the high number of circulating RBCs, the body’s negative feedback mechanism suppresses the production of EPO in the kidneys. Therefore, serum Erythropoietin levels are characteristically low or undetectable in PV [1]. This is a crucial diagnostic criterion to differentiate it from secondary polycythemia (e.g., due to high altitude or smoking), where EPO levels are elevated. Analysis of Incorrect Options: * RBC Count & Hematocrit: These are hallmark findings. PV involves an absolute increase in red cell mass, leading to an elevated RBC count and a high hematocrit (often >52% in men, >48% in women). * Platelet Count: PV is a "panmyelosis," meaning all three myeloid lineages are typically increased. Most patients exhibit thrombocytosis (elevated platelets) and leukocytosis (elevated WBCs). High-Yield Clinical Pearls for NEET-PG: * JAK2 V617F Mutation: Present in >95% of PV cases. * Clinical Feature: Aquagenic pruritus (itching after a warm bath) is a classic symptom. * Complication: Increased risk of both arterial and venous thrombosis due to hyperviscosity. * Spent Phase: PV can progress to myelofibrosis or acute myeloid leukemia (AML).

Q: All are true about splenic marginal zone lymphoma, EXCEPT:

The clinical course is virulent.. **Splenic Marginal Zone Lymphoma (SMZL)** is a rare, low-grade B-cell neoplasm. Understanding its indolent nature is key to answering this question. ### **Explanation of the Correct Answer** **Option D is the correct answer (the false statement)** because SMZL typically follows an **indolent (slow-growing) clinical course**, not a virulent one. Most patients have a median survival exceeding 10 years. Treatment is often conservative ("watch and wait") or involves splenectomy/Rituximab, rather than aggressive chemotherapy. ### **Analysis of Incorrect Options** * **Option A:** Autoimmune manifestations are common in SMZL. Approximately 10–15% of patients present with **autoimmune hemolytic anemia (AIHA)** or **immune thrombocytopenia (ITP)**. * **Option B:** As the name implies, the tumor originates from the **marginal zone B-cells** of the splenic white pulp. These cells eventually infiltrate the red pulp and the peripheral blood. * **Option C:** While peripheral lymphadenopathy is rare in SMZL (a key diagnostic feature), **splenic hilar lymph nodes** are frequently involved as they are the primary drainage site for the spleen. ### **High-Yield NEET-PG Pearls** * **Clinical Presentation:** Massive splenomegaly is the hallmark. Peripheral lymphadenopathy is characteristically absent. * **Morphology:** Look for **"Villous lymphocytes"** (short, polar cytoplasmic projections) in the peripheral blood smear. * **Immunophenotype:** Typically **CD20+, CD5–, CD10–, CD23–, and CD103–**. This helps differentiate it from CLL (CD5+) and Hairy Cell Leukemia (CD103+). * **Association:** There is a strong clinical association with **Hepatitis C Virus (HCV)** infection; treating the virus can sometimes lead to lymphoma regression. * **Genetics:** Often associated with **7q31 deletion**.

Q: Platelets are stored at what temperature?

20-24°C. **Explanation:** **Correct Answer: A. 20-24°C** Platelets are stored at **room temperature (20-24°C)** under continuous agitation. This specific temperature range is critical to maintain platelet viability and metabolic activity. Agitation (using a platelet agitator) is necessary to prevent aggregation and to facilitate gas exchange ($O_2$ and $CO_2$), which prevents a drop in pH that would otherwise lead to the loss of platelet function (the "storage lesion"). **Analysis of Incorrect Options:** * **B. 2-6°C:** This is the storage temperature for **Whole Blood and Packed Red Blood Cells (PRBCs)**. Refrigerating platelets at this temperature causes irreversible structural changes (cold-induced activation and "chilling injury"), leading to rapid clearance from the recipient's circulation by hepatic macrophages. * **C. 37°C:** This is normal body temperature. Storing blood components at this temperature would promote rapid bacterial proliferation and protein denaturation. * **D. -2 to -4°C:** This is below the freezing point for blood components. Freezing without specialized cryoprotectants (like glycerol) causes ice crystal formation, which ruptures cell membranes. (Note: Fresh Frozen Plasma is stored at **-18°C or colder**). **High-Yield Clinical Pearls for NEET-PG:** * **Shelf Life:** Platelets have the shortest shelf life of all blood components—only **5 days**—due to the risk of bacterial contamination at room temperature. * **Transfusion Dose:** One unit of Random Donor Platelets (RDP) typically increases the platelet count by **5,000–10,000/µL** in an average adult. * **Apheresis:** Single Donor Platelets (SDP) are equivalent to 6–8 units of RDP. * **Best Indicator of Viability:** The "swirling phenomenon" observed when a platelet bag is held against light indicates healthy, discoid platelets.

Q: Transient myeloproliferative disorder of the newborn is seen in association with which of the following genetic conditions?

Down syndrome. **Explanation:** **Transient Myeloproliferative Disorder (TMD)**, also known as Transient Abnormal Myelopoiesis (TAM), is a unique hematologic condition characterized by the clonal proliferation of megakaryoblasts. It occurs almost exclusively in neonates with **Down syndrome (Trisomy 21)**. **Why Down Syndrome is Correct:** The pathogenesis involves a synergistic relationship between the extra copy of chromosome 21 and an acquired somatic mutation in the **GATA1 gene**. This mutation leads to the production of a truncated protein (GATA1s), which triggers uncontrolled proliferation of immature megakaryocytes in the fetal liver and bone marrow. While TMD usually resolves spontaneously within the first 3 months of life, approximately 20-30% of these children later develop **Acute Megakaryoblastic Leukemia (AMKL/M7)**. **Why Other Options are Incorrect:** * **Turner Syndrome (45, XO):** Associated with coarctation of the aorta and horseshoe kidney, but not specifically with TMD or neonatal leukemia. * **Neurofibromatosis (NF1):** While NF1 increases the risk of Juvenile Myelomonocytic Leukemia (JMML), it is not associated with TMD. * **Ataxia Telangiectasia:** This is a DNA repair defect (ATM gene) that predisposes patients to lymphomas and lymphocytic leukemias later in childhood, not neonatal TMD. **High-Yield Clinical Pearls for NEET-PG:** * **Gene Mutation:** GATA1 (Shortened/truncated version). * **Clinical Presentation:** Hepatosplenomegaly, jaundice, and high peripheral blast count in a neonate with Down syndrome features. * **Prognosis:** Most cases regress spontaneously; however, survivors must be monitored for the development of **AMKL (AML-M7)**. * **Treatment:** Usually observation; low-dose Cytarabine is reserved only for life-threatening organ dysfunction.

Question 1

Which of the following is NOT a cytogenetic abnormality found in myelodysplastic syndromes?

Accepted Answer

Chromosome 20q deletion

Answer

Chromosome 5q deletion

Answer

Monosomy 7

Answer

Trisomy 8

Question 2

Direct Coombs test detects which of the following?

Accepted Answer

Antibodies attached to the RBC surface

Answer

Antibodies in the serum

Answer

Antigens attached to the RBC surface

Answer

Antigens in the serum

Question 3

In polycythemia vera, which of the following is not elevated?

Accepted Answer

Erythropoietin

Answer

Hematocrit

Answer

Platelet count

Answer

RBC count

Question 4

All are true regarding Myelofibrosis EXCEPT?

Accepted Answer

Absence of splenic enlargement

Answer

Tear drop poikilocytes

Answer

Giant abnormal platelets

Answer

Leucoerythroblastic blood picture

Question 5

All are true about splenic marginal zone lymphoma, EXCEPT:

Accepted Answer

The clinical course is virulent.

Answer

Autoimmune anemia or thrombocytopenia is present.

Answer

This tumor of small lymphocytes originates in the marginal zone of the spleen white pulp.

Answer

Splenic hilar nodes are involved.

Question 6

CD-10 is seen in which of the following conditions?

Accepted Answer

Acute Lymphoblastic Leukemia (ALL)

Answer

Chronic Lymphocytic Leukemia (CLL)

Answer

Hairy Cell Leukemia (HCL)

Answer

Chronic Myeloid Leukemia (CML)

Question 7

Platelets are stored at what temperature?

Accepted Answer

20-24°C

Answer

2-6°C

Answer

37°C

Answer

-2 to -4°C

Question 8

A one-year-old boy presented with hepatosplenomegaly and delayed milestones. Liver biopsy and bone marrow biopsy revealed histiocytes with PAS-positive material. Electron microscopic examination of these histiocytes is most likely to reveal what?

Accepted Answer

Birbeck granules in the cytoplasm

Answer

Myelin figures in the cytoplasm

Answer

Parallel arrays of tubular structures in lysosomes

Answer

Electron dense deposits in the mitochondria

Question 9

Transient myeloproliferative disorder of the newborn is seen in association with which of the following genetic conditions?

Accepted Answer

Down syndrome

Answer

Turner syndrome

Answer

Neurofibromatosis

Answer

Ataxia telangiectasia

Question 10

Translocation of the bcr-abl gene is characteristically seen in which of the following conditions?

Accepted Answer

Chronic myeloid leukemia

Answer

Acute myeloid leukemia

Answer

Chronic lymphatic leukemia

Answer

Acute lymphatic leukemia

Hematopathology — MCQs

Hematopathology — MCQs

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