Hematopathology Practice Questions

Q: Birbeck's granule is seen in which of the following conditions?

Langerhans cell histiocytosis. **Explanation:** **Langerhans Cell Histiocytosis (LCH)** is the correct answer because **Birbeck granules** are the pathognomonic ultrastructural hallmark of Langerhans cells [1]. On electron microscopy, these granules appear as rod-shaped, pentalaminar cytoplasmic organelles with a central striated line and a bulbous end, giving them a characteristic **"tennis racket" appearance** [1]. They contain the protein **Langerin (CD207)**, which is involved in endocytosis and antigen processing [1]. **Analysis of Incorrect Options:** * **Granulomatous vasculitis (e.g., Wegener’s):** Characterized by necrotizing granulomas and vasculitis. Diagnosis relies on ANCA testing and biopsy showing giant cells, not Birbeck granules. * **Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto Disease):** A benign condition presenting with cervical lymphadenopathy. Histology shows paracortical necrosis with karyorrhectic debris and histiocytes, but lacks Langerhans cells. * **Multiple myeloma:** A plasma cell neoplasm characterized by "clock-face" nuclei, perinuclear halos (Golgi zone), and Russell bodies (cytoplasmic Ig inclusions). **High-Yield Clinical Pearls for NEET-PG:** * **Immunophenotype of LCH:** Positive for **S100, CD1a, and Langerin (CD207)**. CD1a and Langerin are highly specific [1]. * **Clinical Presentation:** Can range from a solitary bone lesion (Eosinophilic granuloma) to multisystem involvement (Letterer-Siwe disease). * **Radiology:** Often presents as "punched-out" lytic lesions in the skull. * **BRAF Mutation:** Approximately 50% of LCH cases harbor the **BRAF V600E** mutation, which is a frequent target for molecular testing [1]. **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.

Question 1

All of the following are true about nodular sclerosis of Hodgkin's disease except?

Accepted Answer

CD20 positive

Answer

Well-formed fibrous bands

Answer

CD15 positive

Answer

Infiltration by plasma cells

Question 2

Birbeck's granule is seen in which of the following conditions?

Accepted Answer

Langerhans cell histiocytosis

Answer

Granulomatous vasculitis

Answer

Histiocytic necrotizing lymphadenitis

Answer

Multiple myeloma

Question 3

Erythrocyte Sedimentation Rate is zero in which of the following conditions?

Accepted Answer

Afibrinogenemia

Answer

Abetalipoproteinemia

Answer

Asplenia

Answer

Aplastic anemia

Question 4

What is true about acquired disorders of coagulation?

Accepted Answer

Shows defects in platelets as well

Answer

Shows specific clotting factor deficiency

Answer

Are less frequent than inherited disorders

Answer

Hemarthrosis is specifically seen

Question 5

A 19-year-old man has had recurrent bleeding occur in his knee when playing contact sports. He has no history of spontaneous bleeding, but his brother had similar problems. Consultation with a specialist reveals that he has "mild" hemophilia A. Which of the following factor abnormalities is consistent with this diagnosis?

Accepted Answer

decreased levels of functional factor VIII

Answer

abnormal factor VIII function

Answer

decreased factor IX level

Answer

decreased von Willebrand factor

Question 6

Diffuse large cell lymphoma is classified as which grade of non-Hodgkin's lymphoma?

Accepted Answer

Intermediate grade

Answer

Low grade

Answer

High grade

Answer

None of the above

Question 7

Why do patients with sickle cell trait not manifest the same symptoms as those with sickle cell disease?

Accepted Answer

50% HbS is required for the occurrence of sickling.

Answer

HbA prevents sickling.

Answer

50% sickles are formed.

Answer

HbA prevents polymerization of HbS.

Question 8

Schistocytes in peripheral smear are seen in all except:

Accepted Answer

Sickle cell anemia

Answer

Disseminated Intravascular Coagulation (DIC)

Answer

Thrombotic Thrombocytopenic Purpura (TTP)

Answer

Hemolytic Uremic Syndrome (HUS)

Question 9

Which of the following is NOT included in the myelodysplastic syndromes?

Accepted Answer

Erythroleukemia

Answer

Angiogenic myeloid metaplasia

Answer

Thrombocythemia

Answer

Megaloblastic hyperplasia

Question 10

Diagnosis of Hodgkin's disease is confirmed by?

Accepted Answer

Lymph node biopsy

Answer

CT scan

Answer

Bone marrow biopsy

Answer

Lymphangiography

Hematopathology — MCQs

Hematopathology — MCQs

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