Hematopathology Practice Questions

Q: Isolated deletion of which chromosome causes myelodysplastic syndrome?

5q. **Explanation:** **Myelodysplastic Syndrome (MDS)** is a group of clonal hematopoietic stem cell disorders characterized by cytopenia, ineffective hematopoiesis, and a risk of transformation to Acute Myeloid Leukemia (AML) [1]. **Why 5q is correct:** The **isolated deletion of the long arm of chromosome 5 [del(5q)]** defines a specific clinical entity known as **"5q-minus syndrome."** This is the most common cytogenetic abnormality in MDS. It typically affects elderly women and is characterized by: * Refractory macrocytic anemia. * Normal or elevated platelet counts (thrombocytosis). * Hypolobated micromegakaryocytes in the bone marrow. * A favorable prognosis and excellent response to **Lenalidomide**. **Why other options are incorrect:** * **2q:** Deletions or translocations involving 2q are rare in MDS and do not define a specific isolated syndrome. * **8q:** While **Trisomy 8 (+8)** is a common cytogenetic finding in MDS, isolated deletions of the long arm of chromosome 8 are not characteristic of the disease. * **11q:** Deletions of 11q (specifically involving the *CBL* or *MLL* genes) can occur in various myeloid neoplasms, but they are not the classic "isolated deletion" associated with a specific MDS subtype like 5q. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cytogenetic abnormality in MDS:** del(5q). * **Other common abnormalities:** Trisomy 8 (+8), Monosomy 7 (-7), and del(20q) [1]. * **Poor Prognostic Marker:** Monosomy 7 (-7) or complex karyotypes (>3 abnormalities) [1]. * **Drug of Choice for 5q- syndrome:** Lenalidomide (a thalidomide analogue). * **Morphological Hallmark:** "Pawn ball" megakaryocytes (small, mononuclear) are highly suggestive of MDS. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 622-624.

Q: What is the typical lifespan of transfused platelets?

3-5 days. **Explanation:** The correct answer is **3-5 days (Option C)**. **1. Understanding the Concept:** In a healthy individual, the physiological lifespan of endogenous platelets is approximately **7–10 days**. However, transfused platelets have a significantly shorter survival time. This reduction is due to the "storage lesion"—biochemical and structural changes that occur during collection, processing, and storage at room temperature (20–24°C). Once infused, these platelets are cleared more rapidly by the recipient's reticuloendothelial system. In a stable patient without complications, the expected survival of transfused platelets is typically **3 to 5 days**. **2. Analysis of Incorrect Options:** * **Option A (<24 hours):** This is seen in pathological states of increased consumption or destruction, such as Disseminated Intravascular Coagulation (DIC), ITP, or high-grade fever/sepsis, but it is not the "typical" lifespan. * **Option B (1-3 days):** While survival can be this short in moderately ill patients, 3-5 days is the standard textbook duration for a stable recipient. * **Option D (7-14 days):** This reflects the lifespan of natural, endogenous platelets in a healthy person, not transfused ones. **3. NEET-PG High-Yield Pearls:** * **Storage:** Platelets are stored at **20–24°C (Room Temperature)** with continuous agitation to prevent aggregation and maintain pH. * **Shelf Life:** Due to the risk of bacterial contamination at room temperature, the maximum storage life is only **5 days**. * **Dose Response:** One unit of Random Donor Platelets (RDP) typically increases the platelet count by **5,000–10,000/µL**, whereas one unit of Single Donor Platelets (SDP) increases it by **30,000–60,000/µL**. * **Refractoriness:** If the 1-hour post-transfusion increment is less than expected on two occasions, it suggests platelet refractoriness (often due to HLA alloimmunization).

Q: Which of the following markers are seen in Primary effusion lymphoma?

CD30, CD138. **Primary Effusion Lymphoma (PEL)** is a rare, aggressive B-cell non-Hodgkin lymphoma caused by **Human Herpesvirus-8 (HHV-8)**, typically occurring in HIV-positive or immunocompromised patients [1]. ### **Why Option A is Correct?** PEL has a unique "null" phenotype or a **plasmablastic** immunophenotype. Although it is a B-cell malignancy, it characteristically lacks traditional B-cell markers (like CD19, CD20, and CD79a) [1]. Instead, it expresses markers of terminal B-cell differentiation: * **CD138 (Syndecan-1):** A plasma cell marker consistently expressed in PEL. * **CD30:** Frequently expressed (approx. 70% of cases), reflecting its activated/transformed state. * **Other markers:** EMA (Epithelial Membrane Antigen) and CD45 are often positive. ### **Why Other Options are Incorrect?** * **Options B & C (CD19, CD20):** These are pan-B-cell markers. PEL is defined by the **absence** of these surface markers (downregulated during plasmablastic transformation) [1]. Their presence would point toward other B-cell lymphomas like DLBCL. * **Option D (CD29):** CD29 is an integrin subunit not used as a diagnostic marker for PEL. ### **High-Yield Clinical Pearls for NEET-PG** 1. **Viral Association:** 100% of cases are associated with **HHV-8** (KSHV) [1]. Many are also co-infected with **EBV**. 2. **Clinical Presentation:** Presents as malignant effusions (pleural, pericardial, or peritoneal) **without** a detectable solid tumor mass or lymphadenopathy [1]. 3. **Morphology:** Cells show "plasmablastic" or "immunoblastic" features. 4. **Key Diagnostic Step:** Detection of **LANA-1** (Latent Nuclear Antigen-1) via immunohistochemistry confirms HHV-8 infection. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of White Blood Cells, Lymph Nodes, Spleen, and Thymus, pp. 604-605.

Question 1

Isolated deletion of which chromosome causes myelodysplastic syndrome?

Accepted Answer

5q

Answer

2q

Answer

8q

Answer

11q

Question 2

A 30-year-old female presents with an RBC count of 4.5 million/µL, MCV of 55 fL, and TLC of 8000/µL. She has no history of blood transfusion. What is the most likely diagnosis?

Accepted Answer

Thalassemia minor

Answer

Iron deficiency anemia

Answer

Thalassemia major

Answer

Megaloblastic anemia

Question 3

In alpha-thalassemia trait, what is shown on electrophoresis?

Accepted Answer

Normal HbF and normal HbA2

Answer

Increased HbF and normal HbA2

Answer

Normal HbF and decreased HbA2

Answer

Decreased HbF and normal HbA2

Question 4

In sickle cell trait, how many hemoglobin bands are typically found?

Accepted Answer

2

Answer

1

Answer

4

Answer

5

Question 5

Decreased LAP score is found in:

Accepted Answer

Pernicious anemia

Answer

Leukaemoid reaction

Answer

Myelofibrosis

Answer

Polycythemia

Question 6

A 60-year-old man is referred because of splenomegaly and generalized lymphadenopathy. The total white blood cell count is markedly elevated, and the differential count reveals a preponderance of mature appearing lymphocytes. Bone marrow examination reveals a diffuse infiltration with similar-appearing lymphocytes. Which of the following statements best characterizes this disorder?

Accepted Answer

Bacterial infections are common early in the disease due to hypogammaglobulinemia.

Answer

A progressive increase in the number of myeloblasts and promyelocytes is indicative of acceleration of the disease process.

Answer

Mean survival is less than 1 year after diagnosis.

Answer

Myelofibrosis is a common complication.

Question 7

What is the typical lifespan of transfused platelets?

Accepted Answer

3-5 days

Answer

<24 hours

Answer

1-3 days

Answer

7-14 days

Question 8

Which of the following is a cause for disseminated intravascular coagulation (DIC)?

Accepted Answer

Malignancy

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Lymphoma

Answer

Massive blood transfusion

Question 9

According to WHO, which of the following is not a B cell lymphoma?

Accepted Answer

Anaplastic large cell lymphoma

Answer

Burkitt's lymphoma

Answer

Follicular lymphoma

Answer

Mantle cell lymphoma

Question 10

Which of the following markers are seen in Primary effusion lymphoma?

Accepted Answer

CD30, CD138

Answer

CD20, CD28

Answer

CD19, CD20

Answer

CD29, CD30

Hematopathology — MCQs

Hematopathology — MCQs

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