Hematopathology Practice Questions

Q: Which of the following statements about sickle cell disease is false?

Hemolysis is predominantly intravascular.. In Sickle Cell Disease (SCD), the primary mechanism of red cell destruction is extravascular hemolysis [1]. Sickled erythrocytes are rigid and non-deformable; as they pass through the splenic sinusoids, they are recognized as abnormal and sequestered by splenic macrophages [1]. While some minor intravascular hemolysis occurs due to mechanical fragility, the predominant site of destruction is the reticuloendothelial system (spleen and liver). **Analysis of Options:** * **Option A (True):** Sickle cell trait (HbAS) provides a survival advantage against *Plasmodium falciparum* malaria. The parasite consumes oxygen, causing the cell to sickle and be cleared by the spleen, thereby reducing the parasite burden. * **Option B (True):** The molecular basis of SCD is a point mutation (GAG → GTG) in the β-globin gene, resulting in the substitution of **Glutamic acid (polar) by Valine (non-polar)** at the 6th position of the β-chain [2]. * **Option C (True):** **Hyposthenuria** (inability to concentrate urine) is a classic finding. Repeated micro-infarctions in the renal medulla (due to the hypertonic, hypoxic environment) damage the vasa recta, impairing the countercurrent exchange mechanism. **NEET-PG High-Yield Pearls:** 1. **Autosplenectomy:** Repeated splenic infarctions lead to a shrunken, fibrotic spleen by childhood, increasing susceptibility to encapsulated organisms (*S. pneumoniae*, *H. influenzae*) [1]. 2. **Howell-Jolly Bodies:** These nuclear remnants are seen on peripheral smears post-autosplenectomy. 3. **Salmonella Osteomyelitis:** While *S. aureus* is the most common cause of osteomyelitis overall, patients with SCD have a uniquely high predisposition to *Salmonella* species. 4. **Aplastic Crisis:** Most commonly triggered by **Parvovirus B19** infection [1].

Q: What are the cytogenetic abnormalities associated with lymphoplasmacytoid lymphoma?

t(9;14)(p13;q32). **Explanation:** **Lymphoplasmacytoid Lymphoma (LPL)**, often associated with **Waldenström Macroglobulinemia**, is a B-cell neoplasm characterized by the proliferation of small B lymphocytes, plasmacytoid lymphocytes, and plasma cells. [1] **Why Option A is correct:** The characteristic cytogenetic abnormality in LPL is **t(9;14)(p13;q32)**. This translocation involves the **PAX5 gene** on chromosome 9 and the **Immunoglobulin Heavy Chain (IgH) locus** on chromosome 14. This results in the overexpression of PAX5, a transcription factor essential for B-cell proliferation and differentiation, leading to the development of the lymphoma. **Analysis of Incorrect Options:** * **Option B: t(2;5)(p23;q35):** This is the hallmark of **Anaplastic Large Cell Lymphoma (ALCL)**, involving the *ALK* gene on chromosome 2 and the *NPM* gene on chromosome 5. * **Option C: t(11;14)(q13;q32):** This is diagnostic for **Mantle Cell Lymphoma**, leading to the overexpression of **Cyclin D1** (*PRAD1/BCL-1*). * **Option D: t(14;18)(q32;q21):** This is the classic translocation seen in **Follicular Lymphoma**, resulting in the overexpression of the anti-apoptotic protein **BCL-2**. **High-Yield Clinical Pearls for NEET-PG:** * **MYD88 L265P Mutation:** While t(9;14) is a known translocation, the **MYD88 L265P somatic mutation** is found in >90% of LPL cases and is a more frequent diagnostic marker in modern practice. * **Clinical Presentation:** Patients often present with **hyperviscosity syndrome** (due to monoclonal IgM paraprotein), hepatosplenomegaly, and lymphadenopathy. [1] * **Dutcher Bodies:** Look for PAS-positive intranuclear inclusions (Dutcher bodies) in malignant cells, which are highly suggestive of LPL. **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. 609-610.

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

Which of the following statements about sickle cell disease is false?

Accepted Answer

Hemolysis is predominantly intravascular.

Answer

Sickle cell trait confers a protective effect against malaria.

Answer

There is a replacement of glutamic acid by valine at the beta-6 position.

Answer

Hyposthenuria is a characteristic finding.

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

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 7

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 8

Diagnosis of Hodgkin's disease is confirmed by?

Accepted Answer

Lymph node biopsy

Answer

CT scan

Answer

Bone marrow biopsy

Answer

Lymphangiography

Question 9

Hemoglobinuria may be seen in all of the following conditions EXCEPT?

Accepted Answer

Hereditary spherocytosis

Answer

Mismatched blood transfusion

Answer

Paroxysmal cold hemoglobinuria

Answer

Thermal burns

Question 10

What are the cytogenetic abnormalities associated with lymphoplasmacytoid lymphoma?

Accepted Answer

t(9;14)(p13;q32)

Answer

t(2;5)(p23;q35)

Answer

t(11;14)(q13;q32)

Answer

t(14;18)(q32;q21)

Hematopathology — MCQs

Hematopathology — MCQs

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