Hematopathology Practice Questions

Q: Birbeck’s granules in the cytoplasm are seen in:

Langerhans cells. **Explanation:** **Langerhans cells (Option A)** are specialized dendritic cells found primarily in the skin (stratum spinosum) and other squamous epithelia [1]. The pathognomonic ultrastructural feature of these cells is the **Birbeck granule** [1]. Under electron microscopy, these are 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 the endocytosis and degradation of viruses [1]. **Why other options are incorrect:** * **Mast cells (Option B):** These are characterized by dense, membrane-bound cytoplasmic granules containing histamine, heparin, and tryptase. They do not contain Birbeck granules. * **Myelocytes (Option C):** These are precursors in the granulocytic series. They contain primary (azurophilic) and secondary (specific) granules, but lack Birbeck granules. * **Thrombocytes (Option D):** Platelets contain alpha-granules (fibrinogen, vWF) and dense granules (ADP, ATP, serotonin, calcium), but no Birbeck granules. **High-Yield Clinical Pearls for NEET-PG:** * **Langerhans Cell Histiocytosis (LCH):** A group of disorders (e.g., Letterer-Siwe disease, Hand-Schüller-Christian disease) characterized by the proliferation of these cells [1]. * **Immunohistochemistry (IHC) Markers:** LCH cells are characteristically positive for **S-100**, **CD1a**, and **CD207 (Langerin)** [1]. * **Radiology:** Often presents with "punched-out" lytic bone lesions, especially in the skull. **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.

Q: Which of the following statements regarding Fanconi anemia is incorrect?

It is an autosomal dominant disorder.. **Explanation** **1. Why Option A is the Correct (Incorrect Statement):** Fanconi Anemia (FA) is primarily an **autosomal recessive** disorder. While rare cases of X-linked inheritance (FANCB gene) exist, it is **not** an autosomal dominant condition. This is a common trap in NEET-PG; most inherited bone marrow failure syndromes are recessive. **2. Analysis of Other Options:** * **Option B & C:** FA is the most common cause of **inherited aplastic anemia** [1]. The defect leads to progressive bone marrow failure, which manifests as **pancytopenia** (reduction in RBCs, WBCs, and platelets). Bone marrow examination typically reveals hypocellularity with increased fat replacement [2]. * **Option D:** The fundamental pathogenesis involves mutations in the **FANC gene family**, which are responsible for the **DNA interstrand cross-link repair** pathway. This defect leads to genomic instability and hypersensitivity to DNA-damaging agents. **3. Clinical Pearls & High-Yield Facts for NEET-PG:** * **Physical Findings:** Look for "Thumb and Radius" defects (absent/hypoplastic), short stature, microcephaly, and **Café-au-lait spots**. * **Diagnostic Test:** The gold standard is the **Chromosomal Breakage Analysis** using Clastogenic agents like **Diepoxybutane (DEB)** or Mitomycin C. * **Malignancy Risk:** Patients have a significantly high risk of developing **AML** (Acute Myeloid Leukemia) and squamous cell carcinomas (head, neck, and anogenital). * **Treatment:** Hematopoietic stem cell transplant (HSCT) is the definitive treatment for hematologic manifestations. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 595-596. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 662-663.

Q: What is the characteristic shape of Birbeck granules?

Tennis racket. **Explanation:** **Birbeck granules** are the pathognomonic ultrastructural hallmark of **Langerhans Cell Histiocytosis (LCH)** [1]. Under an electron microscope, these cytoplasmic organelles appear as elongated, rod-shaped structures with a central striated line and a terminal bulbous expansion, giving them a classic **tennis racket** appearance [1]. * **Why Option A is correct:** Birbeck granules are composed of **Langerin (CD207)**, a protein involved in endocytocis and the transport of antigens [1]. The "handle" of the racket is formed by the pentalaminar fusion of membranes, while the "head" represents a vesicular dilation [1]. * **Why Options B, C, and D are incorrect:** These shapes do not correspond to any recognized ultrastructural organelles in hematopathology. While "hockey stick" or "drumstick" appearances are used to describe other medical findings (e.g., mitral stenosis on imaging or Barr bodies in neutrophils), they are not associated with Birbeck granules. **High-Yield Clinical Pearls for NEET-PG:** 1. **Immunohistochemistry (IHC):** Langerhans cells are characteristically positive for **S100, CD1a, and Langerin (CD207)** [1]. Langerin is the most specific marker. 2. **Morphology:** On light microscopy, the cells show characteristic **"coffee-bean" nuclei** (grooved nuclei) [1]. 3. **Clinical Presentation:** LCH can range from a single bone lesion (Eosinophilic granuloma) to multisystem involvement (Letterer-Siwe disease). 4. **Key Association:** Always look for the "tennis racket" description when an electron microscopy (EM) finding is mentioned in a case of suspected histiocytosis [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, p. 630.

Question 1

A 50-year-old woman presents with easy fatigability, a smooth sore tongue, numbness and tingling of the feet, and weakness of the legs. A complete blood count shows a megaloblastic anemia that is not reversed by folate therapy. Hemoglobin is 5.6 g/dL, WBC count is 5,100/mL, and platelets are 240,000/mL. This patient most likely has a deficiency of which of the following vitamins?

Accepted Answer

Vitamin B12

Answer

Vitamin B1 (thiamine)

Answer

Vitamin B2 (riboflavin)

Answer

Vitamin K

Question 2

Which of the following agents is of value in the postoperative care of the hemophilic patient?

Accepted Answer

Aminocaproic acid

Answer

Vitamin K

Answer

Monsel's solution

Answer

Factor 8 cryoprecipitate

Question 3

Which is the most common lymphoma in adults?

Accepted Answer

Diffuse large B-cell lymphoma (DLBCL)

Answer

Adult T-cell leukemia/lymphoma (ATLL)

Answer

Hodgkin lymphoma

Answer

Follicular lymphoma

Question 4

Birbeck’s granules in the cytoplasm are seen in:

Accepted Answer

Langerhans cells

Answer

Mast cells

Answer

Myelocytes

Answer

Thrombocytes

Question 5

Which of the following statements regarding Fanconi anemia is incorrect?

Accepted Answer

It is an autosomal dominant disorder.

Answer

Bone marrow examination typically shows pancytopenia.

Answer

It commonly presents as aplastic anemia.

Answer

It results from a defect in DNA repair.

Question 6

Osmotic fragility is increased in which of the following conditions?

Accepted Answer

Hereditary spherocytosis

Answer

Sickle cell anaemia

Answer

Thalassemia

Answer

Chronic lead poisoning

Question 7

What is the characteristic shape of Birbeck granules?

Accepted Answer

Tennis racket

Answer

Hockey stick

Answer

Bat

Answer

Ball

Question 8

What causes hemolysis in G6-PD deficiency?

Accepted Answer

Oxidative stress

Answer

Hemoglobin defect

Answer

Membrane defect

Answer

Vitamin B12 deficiency

Question 9

In which of the following conditions are Auer rods seen?

Accepted Answer

Granulocytic sarcoma

Answer

Myelodysplastic syndrome

Answer

Multiple myeloma

Answer

Leukemia cutis

Question 10

Thrombocytopenia is caused by which of the following conditions?

Accepted Answer

Disseminated Intravascular Coagulation (DIC)

Answer

Aspirin

Answer

Acyclovir

Answer

Henoch-Schonlein Purpura (HSP)

Hematopathology — MCQs

Hematopathology — MCQs

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