Hematopathology Practice Questions

Q: A 13-year-old female attends a hematology clinic for follow-up of a coagulation defect in the extrinsic pathway. Which coagulation factor is defective?

VII. **Explanation:** The coagulation cascade is traditionally divided into the intrinsic, extrinsic, and common pathways. To answer this question, one must identify which factor is unique to the **extrinsic pathway**. 1. **Why Factor VII is correct:** Factor VII is the only factor involved exclusively in the extrinsic pathway [2]. This pathway is initiated when subendothelial **Tissue Factor (Factor III)** is exposed to circulating Factor VII following vascular injury. The resulting TF-VIIa complex then activates Factor X (the start of the common pathway). In clinical practice, a defect in the extrinsic pathway is characterized by an isolated prolongation of **Prothrombin Time (PT)**, while the Activated Partial Hypotension Time (aPTT) remains normal. 2. **Why the other options are incorrect:** * **Factors VIII, IX, and XI** are all components of the **intrinsic pathway**. * Deficiencies in these factors (e.g., Hemophilia A [VIII], Hemophilia B [IX], or Hemophilia C [XI]) result in a prolonged **aPTT**, while the PT remains normal [2]. **High-Yield Clinical Pearls for NEET-PG:** * **Mnemonic for PT/Extrinsic:** "PET" (PT, Extrinsic, Tissue Factor/Factor VII). * **Mnemonic for aPTT/Intrinsic:** "PITT" (PTT, Intrinsic, Twelve, Eleven, Ten, Nine, Eight—though note Factor X is common). * **Factor VII** has the **shortest half-life** (approx. 4–6 hours) among all clotting factors [1]. Therefore, PT is the first lab value to become deranged in acute liver failure or early Warfarin therapy. * **Vitamin K-dependent factors:** II, VII, IX, and X (Factor VII is the most sensitive to Vitamin K deficiency) [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 582-583. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Hemodynamic Disorders, Thromboembolic Disease, and Shock, pp. 128-130.

Q: The NESTROFT test is used in the screening of which condition?

Thalassemia. **Explanation:** **NESTROFT** (Naked Eye Single Tube Red Cell Osmotic Fragility Test) is a simple, cost-effective, and rapid screening tool used primarily for **Thalassemia Minor (Trait)**. **Why Thalassemia is Correct:** In Thalassemia, there is a defect in globin chain synthesis leading to **hypochromic microcytic** red blood cells [1]. These cells have a high surface-area-to-volume ratio, making them more resistant to hemolysis in hypotonic solutions compared to normal RBCs. In the NESTROFT test, blood is added to a 0.36% buffered saline solution. If the solution remains **turbid** (meaning the line behind the tube is not visible), the test is positive, indicating decreased osmotic fragility characteristic of Thalassemia. **Why Other Options are Incorrect:** * **Autoimmune Hemolytic Anemia:** This is characterized by antibody-mediated destruction; screening usually involves the Coombs test, not osmotic fragility. * **Spherocytosis:** Hereditary Spherocytosis is characterized by **increased** osmotic fragility (cells burst easily). While an Osmotic Fragility Test (OFT) is used for diagnosis, NESTROFT specifically screens for the *resistance* seen in Thalassemia. * **G6PD Deficiency:** This is an enzyme defect. Screening is done using the Fluorescent Spot Test or Methemoglobin Reduction Test. **Clinical Pearls for NEET-PG:** * **Sensitivity:** NESTROFT has a high sensitivity (approx. 95-98%) making it an ideal mass screening tool in resource-limited settings. * **Confirmatory Test:** A positive NESTROFT must be followed by **Hb Electrophoresis** or HPLC (showing HbA2 > 3.5%) for confirmation of Thalassemia Trait. * **Differential:** Iron Deficiency Anemia (IDA) can also show a positive NESTROFT, but the turbidity is usually less marked than in Thalassemia.

Q: Sézary-Lutzner cells are characteristic of which condition?

Mycosis fungoides. **Explanation:** **Sézary-Lutzner cells** are the hallmark cytological feature of **Mycosis Fungoides (MF)** and its leukemic variant, Sézary Syndrome. These are atypical T-helper cells (CD4+) characterized by a **cerebriform nucleus**—a nucleus with deep indentations and folding that resembles the surface of the brain. In MF, these cells infiltrate the epidermis, forming pathognomonic clusters known as **Pautrier’s microabscesses**. **Analysis of Options:** * **Mycosis Fungoides (Correct):** The most common primary cutaneous T-cell lymphoma. The presence of these malignant lymphocytes in the skin (and blood in Sézary Syndrome) is diagnostic. * **Anaplastic Large Cell Lymphoma (ALCL):** Characterized by "Hallmark cells" (kidney or horseshoe-shaped nuclei) and strong expression of CD30. * **Hairy Cell Leukemia:** A B-cell neoplasm characterized by "hairy" cytoplasmic projections and a positive TRAP (Tartrate-Resistant Acid Phosphatase) stain. * **Peripheral T-cell Lymphoma (PTCL):** A heterogeneous group of aggressive nodal lymphomas; while they are T-cell derived, they do not typically feature the classic cerebriform Sézary-Lutzner morphology. **High-Yield Clinical Pearls for NEET-PG:** * **Sézary Syndrome Triad:** Erythroderma (exfoliative dermatitis), generalized lymphadenopathy, and the presence of >1000/mm³ Sézary cells in the peripheral blood. * **Immunophenotype:** Typically **CD3+, CD4+, and CD8-**. * **Histology:** Look for "epidermotropism" (T-cells migrating into the epidermis without significant spongiosis). * **Staging:** MF progresses through three clinical stages: Patch $\rightarrow$ Plaque $\rightarrow$ Tumor.

Question 1

Maturation defect of RBCs is seen in which of the following conditions?

Accepted Answer

Vitamin B12 deficiency

Answer

Vitamin B6 deficiency

Answer

Iron deficiency

Answer

Protein deficiency

Question 2

A 13-year-old female attends a hematology clinic for follow-up of a coagulation defect in the extrinsic pathway. Which coagulation factor is defective?

Accepted Answer

VII

Answer

VIII

Answer

IX

Answer

XI

Question 3

Multiple myeloma is diagnosed by which of the following criteria?

Accepted Answer

Greater than 10% plasmacytosis

Answer

24-hour urine protein measurement

Answer

Kidney biopsy

Answer

Rouleaux formation in peripheral blood smear

Question 4

Ham's test is used in the screening of which of the following conditions?

Accepted Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Hereditary spherocytosis

Answer

G6PD deficiency

Answer

Any hemolytic disorder

Question 5

Massive splenomegaly is defined if the spleen weighs greater than:

Accepted Answer

1 kg

Answer

500 grams

Answer

1.5 kg

Answer

2 kg

Question 6

The NESTROFT test is used in the screening of which condition?

Accepted Answer

Thalassemia

Answer

Autoimmune hemolytic anemia

Answer

Spherocytosis

Answer

G6PD deficiency

Question 7

A four-year-old boy presented with a two-month history of abdominal pain and fever, a ten-day history of maculopapular rash, and three days of dry cough, dyspnea, and wheezing. On examination, the liver and spleen were enlarged, measuring 4 cm and 3 cm below the costal margin, respectively. His hemoglobin was 10.0 g/dL, platelet count was 3.7 x 10^5/µL, and total leukocyte count was 70 x 10^3/µL, with 80% eosinophils. Bone marrow examination revealed a cellular marrow with 45% blasts, 34% eosinophils, and eosinophilic precursors. The blasts stained negative for myeloperoxidase and non-specific esterase and were positive for CD19, CD10, CD22, and CD20. Which one of the following statements is not true about the disease?

Accepted Answer

Inv(16) is often detected in the blasts and the eosinophils.

Answer

Eosinophils are not part of the neoplastic clone.

Answer

t(5;14) rearrangement may be detected in the blasts.

Answer

Peripheral blood eosinophilia may normalize with chemotherapy.

Question 8

Which of the following is a poor prognostic factor in Acute Myeloid Leukemia (AML)?

Accepted Answer

Preceding Myelodysplastic Syndrome (MDS)

Answer

Inversion 16 (Inv 16)

Answer

Auer rods

Answer

Myelomonocytic leukemia (Type M4)

Question 9

Sézary-Lutzner cells are characteristic of which condition?

Accepted Answer

Mycosis fungoides

Answer

Anaplastic large cell lymphoma (ALCL)

Answer

Hairy cell leukemia

Answer

Peripheral T-cell lymphoma (PTCL)

Question 10

What is the earliest change observed in iron deficiency anemia?

Accepted Answer

Decreased serum ferritin

Answer

Decreased serum iron

Answer

Decreased TIBC

Answer

Decreased hemoglobin

Hematopathology — MCQs

Hematopathology — MCQs

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