Hematopathology Practice Questions

Q: All are seen in Thalassemia major EXCEPT:

Macrocytic anemia. **Explanation:** **Why Macrocytic Anemia is the Correct Answer:** Thalassemia major is characterized by **microcytic hypochromic anemia**, not macrocytic anemia. The underlying pathology involves a genetic defect in the synthesis of globin chains (specifically $\beta$-chains in $\beta$-Thalassemia). This leads to a decrease in hemoglobin production within the red blood cells, resulting in smaller (microcytic) and paler (hypochromic) cells. Macrocytic anemia (MCV >100 fL) is typically seen in Vitamin B12 or Folate deficiency. **Analysis of Incorrect Options:** * **Transfusion dependency:** In Thalassemia major, the severe imbalance of globin chains leads to profound anemia. Patients typically present in the first year of life and require lifelong regular blood transfusions to maintain hemoglobin levels and suppress ineffective erythropoiesis. * **Splenohepatomegaly:** This occurs due to two mechanisms: **extramedullary hematopoiesis** (the body attempting to produce RBCs outside the bone marrow) and **hemosiderosis** (iron overload from chronic transfusions). * **Ineffective erythropoiesis:** The precipitation of unpaired $\alpha$-globin chains causes oxidative damage to the RBC precursors in the bone marrow, leading to their premature destruction before they reach circulation. **High-Yield Clinical Pearls for NEET-PG:** * **Peripheral Smear:** Look for target cells, nucleated RBCs, and Howell-Jolly bodies. * **Skeletal Changes:** "Chipmunk facies" and "Hair-on-end" appearance on X-ray due to compensatory bone marrow expansion. * **Diagnosis:** **Hb Electrophoresis** or HPLC is the gold standard (shows increased HbF and absent/low HbA). * **Mentzer Index:** (MCV/RBC count) 13 suggests Iron Deficiency Anemia.

Q: Which of the following immunophenotypic markers is characteristic of mantle cell lymphoma?

CD5+, CD23-. **Explanation:** Mantle Cell Lymphoma (MCL) is a B-cell neoplasm characterized by the proliferation of small to medium-sized lymphocytes originating from the follicular mantle zone [1]. The hallmark of MCL is the chromosomal translocation **t(11;14)**, leading to the overexpression of **Cyclin D1** [1]. **Why Option D is Correct:** Mantle cell lymphoma typically expresses pan-B-cell markers (CD19, CD20) and is characteristically **CD5 positive** but **CD23 negative** [1]. This "CD5+, CD23-" profile is the most critical diagnostic feature used to distinguish it from Chronic Lymphocytic Leukemia (CLL/SLL). **Analysis of Incorrect Options:** * **Option A (CD5+, CD25-):** While MCL is CD25 negative, CD25 positivity is a classic marker for Hairy Cell Leukemia and Adult T-cell Leukemia/Lymphoma (ATLL). * **Option B (CD5+, CD10+):** CD10 is a germinal center marker. Follicular Lymphoma and Burkitt Lymphoma are CD10+, but they are typically CD5 negative. * **Option C (CD5+, CD23+):** This is the classic immunophenotype for **CLL/SLL**. The presence of CD23 is the primary flow cytometry finding that differentiates CLL from MCL [1]. **High-Yield Clinical Pearls for NEET-PG:** 1. **Genetics:** t(11;14) involving the *CCND1* gene and *IGH* locus [1]. 2. **Morphology:** Look for "pink" histiocytes and a lack of transformed centroblasts (unlike Follicular Lymphoma) [1]. 3. **Variant:** The **Blastoid variant** is highly aggressive with a high mitotic index [1]. 4. **Gastrointestinal Involvement:** MCL can present as **Lymphomatous Polyposis** (multiple polyps in the GI tract). 5. **Marker:** **SOX11** is a highly specific nuclear marker for MCL, especially useful in Cyclin D1-negative cases. **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. 610-612.

Q: Leucocyte alkaline phosphatase (LAP) is raised in all conditions except?

Chronic myeloid leukemia. **Explanation:** The **Leucocyte Alkaline Phosphatase (LAP) score** (also known as the Neutrophil Alkaline Phosphatase/NAP score) is a measure of the enzyme activity within mature neutrophils. It is a crucial diagnostic tool used to differentiate a **Leukemoid reaction** or **Myeloproliferative Neoplasms (MPNs)** from **Chronic Myeloid Leukemia (CML)**. **Why CML is the correct answer:** In **Chronic Myeloid Leukemia**, the neoplastic neutrophils are biochemically abnormal and lack the LAP enzyme [1]. Therefore, a **low or zero LAP score** is a hallmark of CML (specifically in the chronic phase). This helps distinguish it from a Leukemoid reaction, where the score is significantly elevated. **Analysis of other options:** * **Polycythemia Vera (PV), Essential Thrombocythemia (ET), and Myelofibrosis:** These are all BCR-ABL negative Myeloproliferative Neoplasms [1]. Unlike CML, the mature neutrophils produced in these conditions are functionally and biochemically capable of producing the LAP enzyme, leading to a **raised or normal LAP score**. * **Leukemoid Reaction:** Though not an option here, it is the most common cause of a highly raised LAP score. **High-Yield Clinical Pearls for NEET-PG:** * **LAP Score in CML:** It is **decreased** in the chronic phase but may **increase** during a Blast Crisis or if there is a concurrent infection. * **Other causes of Low LAP Score:** Paroxysmal Nocturnal Hemoglobinuria (PNH), Hypophosphatasia, and Acute Myeloid Leukemia (AML) [1]. * **Causes of High LAP Score:** Pregnancy, Oral Contraceptive Pills (OCPs), Leukemoid reaction, and MPNs (PV, ET, Myelofibrosis) [1]. * **Modern Diagnosis:** While the LAP score is high-yield for exams, modern diagnosis of CML relies on demonstrating the **Philadelphia chromosome t(9;22)** or the **BCR-ABL1** fusion gene via FISH or PCR [1].

Question 1

What is the most common translocation found in follicular lymphomas?

Accepted Answer

t(14;18)

Answer

t(17;19)

Answer

t(11;14)

Answer

t(22;09)

Question 2

Mega platelets are characteristic findings in which of the following conditions?

Accepted Answer

Bernard-Soulier syndrome

Answer

Glanzmann thrombasthenia

Answer

Von Willebrand disease

Answer

Wiskott-Aldrich syndrome

Question 3

A 23-year-old woman presents with cervical and mediastinal lymphadenopathy. Biopsy of a cervical lymph node reveals a nodular appearance with fibrous bands, effacement of the lymph node architecture, and numerous lacunar cells. Which of the following is true regarding this disorder?

Accepted Answer

Relatively favorable clinical course

Answer

Benign neoplasm

Answer

Frequent association with EBV infection

Answer

Lacunar cells are positive for CD20 and CD45

Question 4

Warm antibody hemolytic anemia is seen in all of the following except?

Accepted Answer

Stibophen

Answer

Methyl dopa

Answer

Penicillin

Answer

Quinidine

Question 5

A 52-year-old man presents with fatigue. Physical examination is normal, but his hemoglobin is low at 8.9 mg/dL. The reticulocyte count is 0.5%, serum iron and TIBC are normal, and ferritin is elevated. A bone marrow aspirate reveals erythroid precursors with accumulated abnormal amounts of mitochondrial iron. For this patient with hypochromic microcytic anemia, what is the most likely diagnosis?

Accepted Answer

Sideroblastic anemia

Answer

Iron deficiency anemia

Answer

Beta-thalassemia trait

Answer

Anemia of chronic disease

Question 6

Histologic sections from a rapidly enlarged cervical lymph node in a 35-year-old female reveal a diffuse, monotonous proliferation of small, noncleaved lymphocytes, which are forming a "starry sky" appearance because numerous tingible-body macrophages are present. A touch prep reveals that many of these cells have cytoplasmic vacuoles. Which stain would most likely react with these cytoplasmic vacuoles?

Accepted Answer

Oil red O

Answer

Myeloperoxidase

Answer

Nonspecific esterase

Answer

Chloracetate esterase

Question 7

All are seen in Thalassemia major EXCEPT:

Accepted Answer

Macrocytic anemia

Answer

Transfusion dependency

Answer

Splenoheptatomegaly

Answer

Ineffective erythropoiesis

Question 8

Punctate basophilia is characteristically seen in which condition?

Accepted Answer

Lead poisoning

Answer

Acute myeloid leukaemia

Answer

Chronic myeloid leukaemia

Answer

Megaloblastic anaemia

Question 9

Which of the following immunophenotypic markers is characteristic of mantle cell lymphoma?

Accepted Answer

CD5+, CD23-

Answer

CD5+, CD25-

Answer

CD5+, CD10+

Answer

CD5+, CD23+

Question 10

Leucocyte alkaline phosphatase (LAP) is raised in all conditions except?

Accepted Answer

Chronic myeloid leukemia

Answer

Myelofibrosis

Answer

Essential thrombocythemia

Answer

Polycythemia vera

Hematopathology — MCQs

Hematopathology — MCQs

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