Hematopathology Practice Questions

Q: Peripheral smear may show vacuolated lymphocytes in all of the following conditions EXCEPT?

Abetalipoproteinemia. **Explanation:** The presence of **vacuolated lymphocytes** on a peripheral blood smear is a classic morphological marker for several **lysosomal storage disorders (LSDs)**. These vacuoles represent enlarged lysosomes filled with undigested substrates due to specific enzyme deficiencies [1]. **1. Why Abetalipoproteinemia is the correct answer:** Abetalipoproteinemia is a disorder of lipid metabolism caused by a mutation in the microsomal triglyceride transfer protein (MTP). The hallmark peripheral smear finding in this condition is **Acanthocytes** (spur cells), not vacuolated lymphocytes. Lipid malabsorption occurs, but it does not result in the lysosomal storage patterns seen in the other options. **2. Analysis of incorrect options (Conditions showing vacuolated lymphocytes):** * **Neuronal Ceroid Lipofuscinosis (NCL):** Specifically the juvenile form (Batten disease), which is a high-yield association for vacuolated lymphocytes. * **Fucosidosis and Sialidosis:** These are glycoprotein storage diseases. Along with **Mannosidosis**, they frequently present with prominent cytoplasmic vacuolation in lymphocytes [1]. * *Other notable causes:* Pompe disease, Wolman disease, and Niemann-Pick disease [1]. **Clinical Pearls for NEET-PG:** * **Acanthocytes (Spur Cells):** Think Abetalipoproteinemia, Chronic Liver Disease, or McLeod Syndrome. * **Vacuolated Lymphocytes + Coarse Facies:** Think Mucopolysaccharidosis (MPS) or Glycoproteinosis (Fucosidosis/Sialidosis) [1]. * **Vacuolated Lymphocytes + Cherry Red Spot:** Think Sialidosis or Niemann-Pick Disease [1]. * **Jordan’s Anomaly:** This refers to vacuoles in **neutrophils** (not lymphocytes), typically seen in Chanarin-Dorfman syndrome (a lipid storage disease). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, pp. 161-164.

Q: In sickle cell trait patients, there is a reduced risk of which of the following diseases?

Malaria. **Explanation:** The correct answer is **Malaria**. This phenomenon is a classic example of **Heterozygote Advantage** (or Balanced Polymorphism), where the sickle cell trait (HbAS) provides a survival advantage against severe malaria, specifically *Plasmodium falciparum*. **Why Malaria is the correct answer:** Individuals with sickle cell trait have red blood cells (RBCs) that tend to sickle when infected by the parasite. These damaged RBCs are prematurely cleared by the spleen, reducing the overall parasite load. Furthermore, the sickling process disrupts the parasite's ability to digest hemoglobin and impairs the expression of adhesion proteins (like PfEMP-1) on the RBC surface, preventing the sequestration of infected cells in vital organs. This significantly reduces the risk of severe complications like cerebral malaria. **Analysis of Incorrect Options:** * **A. Typhoid:** Caused by *Salmonella typhi*, this is a bacterial infection of the gastrointestinal tract and bloodstream. Hemoglobinopathies do not provide protective immunity against it. * **C. G-6PD deficiency:** This is a separate genetic enzymopathy. While both G-6PD deficiency and Sickle Cell Trait are prevalent in malaria-endemic regions due to similar evolutionary pressures, one does not reduce the risk of the other. * **D. Filaria:** This is a helminthic infection involving the lymphatic system. Its pathogenesis is unrelated to hemoglobin structure or RBC survival. **High-Yield Clinical Pearls for NEET-PG:** * **Balanced Polymorphism:** This term describes how a potentially harmful allele (HbS) is maintained in a population because the heterozygote state (HbAS) offers a survival benefit. * **Other Protective Traits:** Besides Sickle Cell Trait, **G-6PD deficiency**, **Thalassemia**, and **Duffy Antigen negativity** (protective against *P. vivax*) are also associated with malaria resistance. * **Diagnosis:** On Hb electrophoresis, Sickle Cell Trait shows **HbA > HbS**.

Question 1

A 17-year-old boy presents with petechiae, sternal tenderness on examination, and a retrosternal mass on X-ray. Bone marrow biopsy shows hypercellularity with more than 20% lymphoblasts. Peripheral smear reveals very low TLC and the presence of lymphoblasts. Which of the following is NOT true about the condition the patient is suffering from?

Accepted Answer

It has a good prognosis

Answer

Notch gene mutation may be present

Answer

An immature T-cell phenotype (TdT/CD34/CD7 positive) may be seen

Answer

Thymic involvement is common

Question 2

Peripheral smear may show vacuolated lymphocytes in all of the following conditions EXCEPT?

Accepted Answer

Abetalipoproteinemia

Answer

Neuronal ceroid lipofuscinosis

Answer

Fucosidosis

Answer

Sialidosis

Question 3

Which immunoglobulin is typically implicated as a warm antibody in autoimmune hemolytic anemia?

Accepted Answer

IgG

Answer

IgM

Answer

IgE

Answer

IgA

Question 4

In blood transfusion, which blood group system is most important?

Accepted Answer

Kell

Answer

Lewis

Answer

Li

Answer

Lutheran

Question 5

Which of the following statements regarding hematological malignancies is true?

Accepted Answer

Acute lymphoid leukemia can occur in individuals less than 1 year of age.

Answer

Chronic myeloid leukemia typically occurs in individuals over 50 years of age.

Answer

Hairy cell leukemia in patients younger than 50 years generally has a good prognosis.

Answer

Chronic lymphocytic leukemia is primarily seen in individuals younger than 50 years of age.

Question 6

Microangiopathic hemolytic anemia is associated with which of the following conditions?

Accepted Answer

Hemolytic uremic syndrome

Answer

Diabetes Mellitus

Answer

Vitamin B12 deficiency

Answer

All of the above

Question 7

In sickle cell trait patients, there is a reduced risk of which of the following diseases?

Accepted Answer

Malaria

Answer

Typhoid

Answer

G-6PD deficiency

Answer

Filaria

Question 8

Which of the following is not an example of a malignant disorder causing myelofibrosis?

Accepted Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Chronic myelogenous leukemia

Answer

Hairy cell leukemia

Answer

Hodgkin's disease

Question 9

What does a low serum ferritin level indicate?

Accepted Answer

Absent body iron stores

Answer

Decreased capacity to absorb iron

Answer

Microcytic hypochromic anemia

Answer

Sideroblastic anemia

Question 10

A 30-year-old woman complains of recent easy fatigability, bruising, and recurrent throat infections. Physical examination reveals numerous petechiae over her body and mouth. Abnormal laboratory findings include hemoglobin of 6 g/dL, WBC of 1,500/mL, and platelets of 20,000/mL. The bone marrow is hypocellular and displays increased fat. What is the appropriate diagnosis?

Accepted Answer

Aplastic anemia

Answer

Iron-deficiency anemia

Answer

Megaloblastic anemia

Answer

Leukemia

Hematopathology — MCQs

Hematopathology — MCQs

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