Hematopathology Practice Questions

Q: A 3-year-old child presented with anemia and thrombocytopenia. On examination, there was massive splenomegaly. A bone marrow aspiration revealed specific cellular findings. What is the diagnosis?

Gaucher disease. ***Gaucher disease*** - The combination of **anemia**, **thrombocytopenia**, and **massive splenomegaly** with specific cellular findings on bone marrow aspiration strongly suggests Gaucher disease. - Bone marrow reveals pathognomonic **Gaucher cells** with characteristic **wrinkled tissue paper cytoplasm** due to **glucocerebrosidase deficiency** leading to glucocerebroside accumulation. *Mucopolysaccharidosis* - Typically presents with **coarse facial features**, **hepatosplenomegaly**, and **developmental delay** rather than isolated hematological abnormalities. - Bone marrow shows **foamy macrophages** filled with **glycosaminoglycans**, not the characteristic wrinkled cytoplasm seen in Gaucher cells. *Acute Leukemia* - Would show **blast cells** on bone marrow aspiration with high **white blood cell count** and **lymphadenopathy**. - The **specific cellular findings** mentioned in the question point toward storage cells rather than malignant blasts. *Thalassemia* - Primarily causes **anemia** with **target cells** and **nucleated RBCs** on peripheral smear, but **thrombocytopenia** is uncommon. - Bone marrow shows **erythroid hyperplasia** rather than the specific storage cells characteristic of lysosomal storage disorders.

Q: Which of the following is NOT a B-cell marker?

CD15. The identification of cell surface markers (Cluster of Differentiation or CD) is a high-yield topic in NEET-PG, particularly for differentiating lymphocyte lineages. **Why CD15 is the correct answer:** **CD15** is primarily a marker for **Granulocytes** (Neutrophils and Eosinophils) and is also expressed on **Reed-Sternberg cells** in Hodgkin Lymphoma (along with CD30). It is not expressed on B-cells. Therefore, it is the correct "NOT" option. **Why the other options are incorrect (B-cell markers):** * **CD19:** This is the most ubiquitous B-cell marker. It is expressed from the earliest stages of B-cell development (pro-B cell) until just before terminal differentiation into plasma cells. * **CD21:** Also known as Complement Receptor 2 (CR2). It is found on mature B-cells and serves as the receptor for the **Epstein-Barr Virus (EBV)**. * **CD24:** This is a glycoprotein expressed on the surface of B-lymphocytes from the pre-B to the mature B-cell stage. **High-Yield Clinical Pearls for NEET-PG:** * **Pan-B cell markers:** CD19, CD20, CD22. * **CD10:** Also known as **CALLA** (Common Acute Lymphoblastic Leukemia Antigen), found on pre-B cells. * **CD5:** Normally a T-cell marker, but its expression on B-cells is characteristic of **Chronic Lymphocytic Leukemia (CLL)** and **Mantle Cell Lymphoma**. * **CD15 & CD30:** The classic "duo" for identifying Reed-Sternberg cells in Hodgkin Lymphoma (except for the Lymphocyte Predominant type).

Q: Leukoreduced blood products have lower WBCs than normal by what fold?

3 log reduction. **Explanation:** **Leukoreduction** is the process of removing white blood cells (WBCs) from whole blood or blood components (red cells and platelets) prior to transfusion. According to international standards (AABB and FDA), a blood product is considered "leukoreduced" if the residual WBC count is **less than 5 × 10⁶ per unit**. 1. **Why 3 log reduction is correct:** A standard unit of whole blood contains approximately $10^9$ WBCs. To reach the threshold of $<5 \times 10^6$, a **3 log reduction** (99.9% removal) is required. This level of depletion is clinically significant because it crosses the threshold necessary to prevent most leukocyte-mediated transfusion reactions. 2. **Analysis of Incorrect Options:** * **1 log (90%) and 2 log (99%) reduction:** These levels leave behind too many residual WBCs ($10^8$ and $10^7$ respectively), which are still sufficient to cause HLA sensitization and transmit CMV. * **4 log (99.99%) reduction:** While achievable with advanced filtration, it is not the standard requirement for labeling a product as leukoreduced. **Clinical Pearls for NEET-PG:** * **Indications for Leukoreduction:** 1. Prevention of **Febrile Non-Hemolytic Transfusion Reactions (FNHTR)** – the most common indication. 2. Prevention of **HLA Alloimmunization** (useful in patients requiring chronic transfusions). 3. Prevention of **CMV transmission** (as CMV resides within leukocytes). * **Note:** Leukoreduction does **NOT** prevent Graft-vs-Host Disease (GVHD). To prevent GVHD, **Gamma Irradiation** is required to inactivate T-lymphocytes. * **Timing:** "Pre-storage" leukoreduction (done within 48 hours of collection) is superior to "bedside" filtration as it prevents the release of inflammatory cytokines during storage.

Question 1

A 3-year-old child presented with anemia and thrombocytopenia. On examination, there was massive splenomegaly. A bone marrow aspiration revealed specific cellular findings. What is the diagnosis?

Accepted Answer

Gaucher disease

Answer

Mucopolysaccharidosis

Answer

Acute Leukemia

Answer

Thalassemia

Question 2

What is the investigation of choice to confirm sickle cell disease?

Accepted Answer

High-performance liquid chromatography

Answer

Tandem mass spectrometry

Answer

Gel electrophoresis

Answer

Naked eye single tube red cell osmotic fragility test

Question 3

What type of antibodies are typically found in Idiopathic Thrombocytopenic Purpura (ITP)?

Accepted Answer

IgG

Answer

IgM

Answer

IgE

Answer

IgD

Question 4

Which of the following is NOT a B-cell marker?

Accepted Answer

CD15

Answer

CD19

Answer

CD21

Answer

CD24

Question 5

A single nucleotide change in a codon on chromosome 11 causes valine to replace glutamic acid at the sixth position of the beta chain of hemoglobin. What is the resulting condition?

Accepted Answer

Sickle cell anemia

Answer

Thalassemia

Answer

Hereditary spherocytosis

Answer

Paroxysmal nocturnal hemoglobinuria

Question 6

Leukoreduced blood products have lower WBCs than normal by what fold?

Accepted Answer

3 log reduction

Answer

1 log reduction

Answer

2 log reduction

Answer

4 log reduction

Question 7

Which of the following conditions is not typically associated with leucocytosis?

Accepted Answer

Typhoid fever

Answer

Myocardial infarction

Answer

Appendicitis

Answer

Measles

Question 8

Which of the following is NOT an immunohistochemical marker of Langerhans cell histiocytosis?

Accepted Answer

None of the above

Answer

S-100

Answer

CD1a

Answer

CD207

Question 9

At autopsy, the spleen of a patient is noted to have a thickened capsule and many small, scarred areas. Microscopic examination of the scarred areas reveals fibrosis with hemosiderin and calcium deposition. This type of spleen is usually seen in conjunction with which of the following disorders?

Accepted Answer

Hepatic cirrhosis

Answer

Hodgkin’s disease

Answer

Rheumatoid arthritis

Answer

Sickle cell anemia

Question 10

A child with prolonged aPTT, who underwent laparotomy without bleeding complications, is most likely to have a deficiency in which clotting factor?

Accepted Answer

Factor XII

Answer

Factor VII

Answer

Factor X

Answer

Factor XIII

Hematopathology — MCQs

Hematopathology — MCQs

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