Hematopathology Practice Questions

Q: Which of the following is NOT true about histiocytosis X?

Associated with angiomyolipoma of the kidney. **Explanation:** **Histiocytosis X**, now more commonly known as **Langerhans Cell Histiocytosis (LCH)**, is a clonal proliferation of Langerhans cells [1]. **Why Option D is the correct answer:** Langerhans Cell Histiocytosis is **not** associated with renal angiomyolipoma. Angiomyolipoma of the kidney is a classic feature of **Tuberous Sclerosis**, a neurocutaneous syndrome [2]. LCH, while multisystemic, primarily involves the bone, skin, and lungs, but does not typically manifest with renal hamartomas [2]. **Analysis of Incorrect Options:** * **Option A:** Seborrheic-like dermatitis (scaly, erythematous rash) is a classic cutaneous presentation of LCH, particularly in the Letterer-Siwe disease variant seen in infants. * **Option B:** **Birbeck granules** are the pathognomonic ultrastructural hallmark of LCH [1]. Seen on electron microscopy, they are "tennis-racket" shaped pentalaminar structures containing the protein **Langerin (CD207)** [1]. * **Option C:** Lytic "punched-out" bony lesions (especially in the skull) are the most common clinical presentation, particularly in the Eosinophilic Granuloma variant. **High-Yield Clinical Pearls for NEET-PG:** * **Immunophenotype:** LCH cells are positive for **S100, CD1a, and Langerin (CD207)** [1]. * **Hand-Schüller-Christian Disease:** A clinical triad of LCH consisting of: 1. Lytic skull lesions, 2. Exophthalmos, and 3. Diabetes Insipidus. * **Morphology:** Cells have characteristic "coffee-bean" nuclei (grooved nuclei) [1]. * **Prognosis:** Letterer-Siwe (Multisystem) has the worst prognosis; Eosinophilic Granuloma (Unifocal) has the best. **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. 629-630. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. The Central Nervous System, pp. 1318-1319.

Q: Which of the following statements is NOT true regarding polycythemia vera?

There is a decrease in PCV.. **Explanation:** Polycythemia Vera (PV) is a **chronic myeloproliferative neoplasm** characterized by the autonomous, clonal production of hematopoietic cells. The hallmark of the disease is an absolute increase in red blood cell (RBC) mass. **Why Option D is the Correct Answer (The False Statement):** In Polycythemia Vera, there is a significant **increase** in the Packed Cell Volume (PCV) or Hematocrit, often exceeding 52% in men and 48% in women. PCV represents the proportion of blood volume occupied by RBCs; since RBC production is pathologically elevated, the PCV must rise, not decrease. **Analysis of Other Options:** * **Option A (Neoplastic condition):** PV is a primary polycythemia caused by a mutation in the hematopoietic stem cell (most commonly the **JAK2 V617F mutation**), making it a neoplastic process. * **Option B (Increase in RBC count):** This is the defining feature of the condition. The bone marrow produces excessive erythrocytes independent of erythropoietin levels. * **Option C (Blood viscosity increases):** As the concentration of RBCs (PCV) rises, the blood becomes thicker. This hyperviscosity slows blood flow and increases the risk of thrombosis. **NEET-PG High-Yield Pearls:** * **Molecular Marker:** >95% of cases are associated with the **JAK2 mutation**. * **Erythropoietin (EPO) Levels:** Characteristically **low** (a key diagnostic differentiator from secondary polycythemia). * **Clinical Sign:** **Aquagenic pruritus** (itching after a warm bath) is a classic symptom. * **Complications:** Increased risk of thrombotic events (strokes, MI) and potential transformation into myelofibrosis or Acute Myeloid Leukemia (AML).

Question 1

In the Philadelphia chromosome, what genetic defect is present?

Accepted Answer

Long arm of chromosome 22

Answer

Short arm of chromosome 22

Answer

Short arm of chromosome 9

Answer

Chromosome 21

Question 2

Classical 'Rain drop' lesions are seen in which of the following conditions?

Accepted Answer

Multiple myeloma

Answer

Burkitt's lymphoma

Answer

Hodgkin's lymphoma

Answer

Haemophilia

Question 3

Which of the following is NOT true about histiocytosis X?

Accepted Answer

Associated with angiomyolipoma of the kidney

Answer

Seborrheic dermatitis is a known feature

Answer

Birbeck's granules are a characteristic feature

Answer

Lytic bony lesions are known

Question 4

A patient develops an ovarian mass that is picked up on pelvic examination. Resection of the mass demonstrates a lymphoma composed of small lymphocytes with interspersed macrophages surrounded by clear spaces. This lesion would be most likely to be associated with which of the following?

Accepted Answer

c-MYC activation

Answer

ABL-BCR hybrid

Answer

BCL-2 activation

Answer

t(9;22)

Question 5

Which of the following haemoglobin estimations will be diagnostically helpful in a case of beta thalassemia trait?

Accepted Answer

Hb-A2

Answer

Hb-F

Answer

Hb-C

Answer

Hb-H

Question 6

Pancytopenia with cellular bone marrow is seen in all EXCEPT?

Accepted Answer

Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency

Answer

Megaloblastic Anemia

Answer

Myelodysplastic Syndrome (MDS)

Answer

Paroxysmal Nocturnal Hemoglobinuria (PNH)

Question 7

Which of the following agents is a prerequisite for Prothrombin time (PT) assay?

Accepted Answer

Tissue thromboplastin

Answer

Kaolin

Answer

Glass beads

Answer

Silica

Question 8

What is not true regarding Waldenstrom's macroglobulinemia?

Accepted Answer

Hypercalcemia

Answer

Lymphadenopathy is usually present

Answer

Blood viscosity is increased

Answer

IgM immunoglobulin is increased

Question 9

Which of the following statements is NOT true regarding polycythemia vera?

Accepted Answer

There is a decrease in PCV.

Answer

It is a neoplastic condition.

Answer

There is an increase in red blood cell count.

Answer

Blood viscosity increases.

Question 10

Increased fetal hemoglobin is seen in which of the following conditions?

Accepted Answer

Juvenile chronic myeloid leukemia

Answer

Congenital red cell aplasia

Answer

Hereditary spherocytosis

Answer

Acute myeloid leukemia

Hematopathology — MCQs

Hematopathology — MCQs

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