General Pathology Practice Questions

Q: Chediak Higashi syndrome is characterized by the following EXCEPT:

Neutrophilia. **Explanation:** **Chediak-Higashi Syndrome (CHS)** is a rare autosomal recessive disorder caused by a mutation in the **LYST gene** (Lysosomal Trafficking Regulator) [1]. This mutation leads to defective vesicle fusion and protein trafficking [1]. **Why "Neutrophilia" is the correct answer (the exception):** Patients with CHS actually present with **Neutropenia**, not neutrophilia [1]. The defect in lysosomal trafficking interferes with normal hematopoiesis in the bone marrow, leading to ineffective granulopoiesis and the premature destruction of white blood cells. **Analysis of other options:** * **Giant Granules (Option D):** This is the morphological hallmark of CHS. Due to disordered fusion of endosomes and lysosomes, massive, non-functional "giant" granules are seen in neutrophils, eosinophils, and melanocytes [1]. * **Defective Degranulation (Option B):** The giant granules are unable to fuse properly with phagosomes [1]. This prevents the release of hydrolytic enzymes into the phagocytic vacuole. * **Delayed Microbial Killing (Option C):** Because degranulation is impaired, the delivery of bactericidal enzymes to the ingested microbe is delayed or absent, leading to recurrent pyogenic infections [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Clinical Tetrad:** 1. Oculocutaneous albinism (melanocyte defect), 2. Recurrent pyogenic infections (staphylococci/streptococci), 3. Progressive neurological defects, 4. Bleeding tendencies (platelet dense body defect) [1]. * **Diagnosis:** Peripheral blood smear showing **giant peroxidase-positive granules** in neutrophils [1]. * **Accelerated Phase:** Most patients eventually enter a "hemophagocytic lymphohistiocytosis" (HLH) phase characterized by hepatosplenomegaly and pancytopenia. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Diseases of the Immune System, pp. 245-246.

Question 1

Which of the following are classified as small round cell tumors?

Accepted Answer

All of the above

Answer

Wilm's tumor

Answer

Retinoblastoma

Answer

Rhabdomyosarcoma

Question 2

Metaplasia is thought to be caused in most cases by:

Accepted Answer

Chronic irritation

Answer

Genetic mutation

Answer

Oncogenic virus

Answer

Immunologic reaction

Question 3

Which of the following conditions is characterized by elevated levels of Alpha-fetoprotein (AFP)?

Accepted Answer

Yolk sac tumor

Answer

Seminoma

Answer

Teratoma

Answer

Choriocarcinoma

Question 4

Which of the following is true about small silencing RNAs?

Accepted Answer

They are associated with gene knockdown technology.

Answer

They are mature oligomers in the human genome.

Answer

XIST is an example of a small interfering RNA (siRNA).

Answer

They are related to atherosclerosis.

Question 5

Besides metastasis, which feature is most reliable in differentiating a benign from a malignant tumor?

Accepted Answer

Local invasion

Answer

Anaplasia

Answer

Dysplasia

Answer

Metastasis

Question 6

Which of the following is an inhibitor of apoptosis?

Accepted Answer

Bcl-2

Answer

Bad

Answer

Bax

Answer

All of the above

Question 7

What is the most common primary immunodeficiency?

Accepted Answer

Isolated IgA immunodeficiency

Answer

Common variable immunodeficiency

Answer

Wiskott-Aldrich syndrome

Answer

Acquired immunodeficiency syndrome

Question 8

Chediak Higashi syndrome is characterized by the following EXCEPT:

Accepted Answer

Neutrophilia

Answer

Defective degranulation

Answer

Delayed microbial killing

Answer

Giant granules

Question 9

Petechiae in scurvy is due to which of the following?

Accepted Answer

Endothelial disintegration

Answer

Platelet dysfunction

Answer

Thrombocytopenia

Answer

Clotting factor deficiency

Question 10

The most frequent cause of clinically significant subarachnoid hemorrhage is rupture of which of the following?

Accepted Answer

Saccular aneurysm

Answer

Fusiform aneurysm

Answer

Mycotic aneurysm

Answer

Dissecting aneurysm

General Pathology — MCQs

General Pathology — MCQs

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