Hematopathology Practice Questions

Q: Langerhans cells are not seen in which of the following conditions?

Fabry disease. **Explanation:** The question tests your ability to differentiate between disorders of histiocytic proliferation and metabolic storage diseases. **Why Fabry Disease is the Correct Answer:** **Fabry disease** is an X-linked recessive **lysosomal storage disorder** caused by a deficiency of the enzyme **alpha-galactosidase A**. This leads to the systemic accumulation of globotriaosylceramide (Gb3) in vascular endothelium and other tissues. It is characterized clinically by angiokeratomas, acroparesthesia, hypohidrosis, and renal/cardiac failure. It does **not** involve the proliferation or presence of Langerhans cells. **Analysis of Incorrect Options:** * **Letterer-Siwe Disease:** This is the acute disseminated form of **Langerhans Cell Histiocytosis (LCH)**, typically seen in infants. It involves multi-organ proliferation of Langerhans cells (CD1a+, S100+, and CD207+). * **Histiocytosis:** This is a broad term for a group of syndromes characterized by the proliferation of histiocytes. Specifically, **Langerhans Cell Histiocytosis (LCH)** is the prototypical condition where these cells are the primary pathological finding. **High-Yield Clinical Pearls for NEET-PG:** * **Langerhans Cells (LCs):** These are dendritic, antigen-presenting cells located in the **Stratum Spinosum** of the epidermis. * **Electron Microscopy Gold Standard:** The presence of **Birbeck Granules** (tennis-racket shaped organelles) is pathognomonic for Langerhans cells. * **Immunohistochemistry (IHC) Markers:** LCs are positive for **S-100**, **CD1a**, and **Langerin (CD207)**. * **Fabry Disease "Mnemonic":** Remember "The **Fabry** **Alpha** male **Gal** acts **X-linked**" (Alpha-galactosidase A deficiency, X-linked inheritance). Look for "Maltese cross" appearance in urinary sediment.

Q: All of the following are seen in sickle cell anemia EXCEPT:

High hematocrit. **Explanation:** Sickle Cell Anemia (SCA) is a chronic hemolytic anemia caused by a point mutation in the $\beta$-globin gene (Glu $\to$ Val at position 6) [1]. The fundamental pathophysiology involves the polymerization of deoxygenated Hemoglobin S (HbS), leading to red blood cell (RBC) distortion and premature destruction [1]. **Why "High hematocrit" is the correct answer:** In SCA, the continuous destruction of sickled RBCs (extravascular and intravascular hemolysis) leads to a **low hematocrit** and low hemoglobin levels (typically 6–9 g/dL) [2]. A high hematocrit is characteristic of polycythemia, not a hemolytic state. **Analysis of incorrect options:** * **Target cells (Codocytes):** These are commonly seen on peripheral smears in SCA. They occur due to a decrease in hemoglobin volume relative to the cell membrane surface area, often exacerbated by functional asplenia. * **Jaundice:** Chronic hemolysis leads to increased breakdown of heme, resulting in **unconjugated hyperbilirubinemia**. This clinically manifests as icterus (jaundice) and predisposes patients to pigment gallstones. * **Reticulocytosis:** To compensate for the shortened lifespan of RBCs (10–20 days instead of 120), the bone marrow increases erythropoiesis, resulting in an elevated reticulocyte count. **Clinical Pearls for NEET-PG:** * **Peripheral Smear:** Look for Sickle cells (drepanocytes), Target cells, and **Howell-Jolly bodies** (indicating functional asplenia due to repeated splenic infarctions) [2]. * **Aplastic Crisis:** Usually triggered by **Parvovirus B19** infection, characterized by a sudden drop in hemoglobin and a *low* reticulocyte count [2]. * **Diagnosis:** Gold standard is **Hemoglobin Electrophoresis** (HbS >80%, HbF variable, HbA absent) [1]. * **Acute Chest Syndrome:** The leading cause of death in adult SCA patients.

Question 1

A mother is diagnosed with sickle cell disease, and her husband is normal. What are the chances of having children with sickle cell disease and sickle cell trait, respectively?

Accepted Answer

0% and 100%

Answer

25% and 25%

Answer

50% and 50%

Answer

10% and 50%

Question 2

Paroxysmal nocturnal hemoglobinuria (PNH) occurs due to a defect in which of the following?

Accepted Answer

CD 59

Answer

CD 15

Answer

CD 100

Answer

CD 20

Question 3

Langerhans cells are not seen in which of the following conditions?

Accepted Answer

Fabry disease

Answer

Letterer-Siwe disease

Answer

Histiocytosis

Answer

None of the above

Question 4

Reed-Sternberg cells of Hodgkin's disease, which are positive for CD15 and CD30, are seen in all of the following types EXCEPT:

Accepted Answer

Lymphocyte predominance

Answer

Nodular sclerosis

Answer

Mixed cellularity

Answer

Lymphocyte depletion

Question 5

Which of the following is not a myeloproliferative neoplasm?

Accepted Answer

Juvenile myelomonocytic leukemia

Answer

Polycythemia vera

Answer

Chronic myeloid leukemia

Answer

Essential thrombocythemia

Question 6

Severe hereditary spherocytosis is seen due to a defect in which of the following proteins?

Accepted Answer

Spectrin

Answer

Ankyrin

Answer

Band 3

Answer

Band 4.2

Question 7

In hereditary spherocytosis, the defect lies in which component?

Accepted Answer

Membrane cytoskeleton

Answer

Hemoglobin

Answer

Enzyme

Answer

None of the above

Question 8

Hereditary spherocytosis is characterized by all the following features, except:

Accepted Answer

Increased surface area to volume ratio

Answer

Increased osmotic fragility

Answer

Autosomal dominant inheritance

Answer

Direct Coombs test is diagnostic

Question 9

A patient with a bleeding disorder presents with increased bleeding time and normal clotting time. Which of the following conditions is the most likely diagnosis?

Accepted Answer

Idiopathic thrombocytopenic purpura

Answer

Classic hemophilia

Answer

Christmas disease

Answer

Vitamin K deficiency

Question 10

All of the following are seen in sickle cell anemia EXCEPT:

Accepted Answer

High hematocrit

Answer

Target cells

Answer

Jaundice

Answer

Reticulocytosis

Hematopathology — MCQs

Hematopathology — MCQs

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