Hematopathology Practice Questions

Q: What is characteristically found in the bone marrow in lead poisoning?

Ringed siderocytes. **Explanation:** Lead poisoning (Plumbism) interferes with the biosynthesis of heme by inhibiting two key enzymes: **delta-aminolevulinic acid dehydratase (ALAD)** and **Ferrochelatase** [1]. The inhibition of Ferrochelatase prevents the incorporation of iron into the protoporphyrin ring. Consequently, iron accumulates within the mitochondria of developing erythroblasts in the bone marrow. When these cells are stained with **Prussian Blue (Perls' stain)**, the iron-laden mitochondria appear as a necklace-like ring around the nucleus, termed **Ringed Sideroblasts** (or Siderocytes in mature forms). **Analysis of Options:** * **A. Ringed siderocytes (Correct):** These are the hallmark of sideroblastic anemias, which can be hereditary or acquired (e.g., lead poisoning, alcohol, or MDS). * **B. Giant metamyelocytes:** These are characteristic of **Megaloblastic Anemia** (Vitamin B12 or Folate deficiency) due to impaired DNA synthesis and nuclear-cytoplasmic dyssynchrony. * **C. Dwarf megakaryocytes (Micromegakaryocytes):** These are small, mononuclear megakaryocytes typically seen in **Myelodysplastic Syndromes (MDS)** or Chronic Myeloid Leukemia (CML). * **D. Fibrotic changes:** Extensive marrow fibrosis is characteristic of **Primary Myelofibrosis** or late-stage "spent phase" polycythemia vera. **High-Yield Clinical Pearls for NEET-PG:** * **Peripheral Smear:** Look for **Coarse Basophilic Stippling** (due to inhibition of 5'-nucleotidase, leading to ribosomal RNA degradation). * **X-ray:** "Lead lines" at the metaphyses of growing long bones in children [1]. * **Gums:** "Burtonian lines" (bluish-purple pigmentation at the gingival margin). * **Treatment:** Chelation therapy with **Succimer** (oral, first-line in kids) or **CaNa₂EDTA**. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Infectious Diseases, pp. 418-419.

Q: The peripheral blood smear is characteristic of which of the following hematological conditions?

Hereditary spherocytosis. ***Hereditary spherocytosis*** - Peripheral blood smear shows **spherocytes** - small, round, hyperchromatic RBCs with **absent central pallor** due to membrane loss. - **Osmotic fragility test** is positive, and patients often present with **hemolytic anemia**, **jaundice**, and **splenomegaly**. *Thalassemia* - Characteristic findings include **target cells**, **hypochromic microcytic RBCs**, and **basophilic stippling** on peripheral smear. - **Hemoglobin electrophoresis** shows elevated HbA2 and HbF levels, not spherocytes. *Sickle cell anemia* - Peripheral blood smear demonstrates **sickle-shaped** or **crescent-shaped** RBCs during sickling crises. - **Hemoglobin electrophoresis** reveals HbS, and patients experience **vaso-occlusive crises**. *G6PD deficiency* - Characteristic findings include **bite cells**, **blister cells**, and **Heinz bodies** on **supravital staining**. - Hemolysis typically occurs after exposure to **oxidative stress** from drugs or infections, not spontaneously.

Q: What is the concentration of Hb A2 in thalassemia trait?

Slightly increased. **Explanation:** In **$\beta$-thalassemia trait** (minor), there is a partial deficiency in the production of $\beta$-globin chains. To compensate for this deficit, the body increases the production of alternative hemoglobin types that do not require $\beta$-chains. 1. **Why "Slightly Increased" is correct:** In $\beta$-thalassemia trait, the excess $\alpha$-chains pair with $\delta$-chains to form **Hb A2 ($\alpha_2\delta_2$)**. In a healthy adult, Hb A2 is typically <3%. In $\beta$-thalassemia trait, it characteristically rises to **3.5% – 7%**. While this is a diagnostic increase, it is medically classified as "slight" compared to the massive shifts seen in other hemoglobinopathies. 2. **Analysis of Incorrect Options:** * **Normal:** A normal Hb A2 level (2-3%) usually rules out $\beta$-thalassemia trait (except in rare cases of co-existing iron deficiency). * **Increased/Markedly Increased:** These terms are imprecise or misleading. "Markedly increased" would describe HbF levels in $\beta$-thalassemia major (often >90%). In the context of standardized exams like NEET-PG, the specific elevation of Hb A2 in trait is classically described as "slight." **High-Yield Clinical Pearls for NEET-PG:** * **Gold Standard Diagnosis:** Hb electrophoresis showing Hb A2 >3.5% is the diagnostic hallmark of $\beta$-thalassemia trait. * **Mentzer Index:** (MCV/RBC count) 13 suggests Iron Deficiency Anemia (IDA). * **The IDA Trap:** Concomitant iron deficiency can mask $\beta$-thalassemia trait by lowering Hb A2 levels into the normal range. Always correct iron stores before testing. * **Peripheral Smear:** Shows microcytic hypochromic anemia with **target cells** and basophilic stippling [1]. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 590-591.

Q: CAR-T cells are used in the treatment of which of the following cancers?

Acute lymphoblastic leukemia. **Explanation:** **Chimeric Antigen Receptor (CAR) T-cell therapy** is a revolutionary form of adoptive immunotherapy where a patient’s T-cells are genetically engineered to express a synthetic receptor that recognizes specific tumor antigens without the need for MHC presentation. **Why Option A is Correct:** The most successful application of CAR-T therapy to date is in B-cell malignancies. The engineered T-cells typically target **CD19**, a surface marker found on almost all B-cells. **Acute Lymphoblastic Leukemia (ALL)**, specifically B-ALL, shows high expression of CD19, making it highly susceptible to this therapy. It is currently FDA-approved for relapsed or refractory B-cell ALL in children and young adults [1]. **Why Other Options are Incorrect:** * **Options B, C, and D (Soft tissue sarcoma, Medulloblastoma, Small cell lung carcinoma):** These are all **solid tumors**. CAR-T therapy faces significant challenges in solid tumors due to the lack of unique tumor-specific antigens, poor penetration into the dense tumor microenvironment, and local immunosuppressive factors. While research is ongoing, they are not currently standard indications for CAR-T therapy. **High-Yield Clinical Pearls for NEET-PG:** * **Target Antigen:** Most common target is **CD19** (used in Tisagenlecleucel and Axicabtagene ciloleucel). * **Major Side Effect:** **Cytokine Release Syndrome (CRS)**, characterized by high fever and hypotension due to massive release of IL-6. * **Management of CRS:** The IL-6 receptor antagonist **Tocilizumab** is the drug of choice. * **Neurotoxicity:** Also known as ICANS (Immune Effector Cell-Associated Neurotoxicity Syndrome), it is the second most common serious side effect. **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. 635-636.

Question 1

Absolute lymphocytosis is typically seen in which of the following conditions?

Accepted Answer

Infectious Mononucleosis (IMN)

Answer

Systemic Lupus Erythematosus (SLE)

Answer

Typhoid fever

Answer

Brucellosis

Question 2

A 35,000/mm
3

hematocrit; prothrombin time-20 sec with a control of 13 sec; partial thromboplastin time-50sec; and Fibrinogen 10mg/dL. Peripheral smear was suggestive of acute myeloblastic leukemia. Which of the following is the most likely diagnosis?

Accepted Answer

Acute myeloblastic leukemia without maturation

Answer

Acute myeloblastic leukemia with maturation

Answer

Acute promyelocytic leukemia

Answer

Acute myelomonocytic leukemia

Question 3

What is the most accurate statement regarding Disseminated Intravascular Coagulation (DIC)?

Accepted Answer

DIC is associated with increased levels of D-dimer.

Answer

Removal of the underlying cause leads to complete resolution.

Answer

It is associated with increased serum fibrinogen levels.

Answer

Normal clotting parameters excludes DIC.

Question 4

What is characteristically found in the bone marrow in lead poisoning?

Accepted Answer

Ringed siderocytes

Answer

Giant metamyelocytes

Answer

Dwarf megakaryocytes

Answer

Fibrotic changes

Question 5

Which mediastinal tumor is most likely associated with red blood cell aplasia?

Accepted Answer

Thymoma

Answer

Hodgkin's disease

Answer

Neuroblastoma

Answer

Parathyroid adenoma

Question 6

All of the following are true regarding the findings of peripheral smear in vitamin B12 deficiency except?

Accepted Answer

Microcytic red cells

Answer

Hypercellular marrow

Answer

Poikilocytosis

Answer

Hypersegmented neutrophils

Question 7

Which of the following lymph nodes are commonly affected in non-Hodgkin's Lymphoma, except?

Accepted Answer

Mediastinal lymph nodes

Answer

Epitrochlear lymph nodes

Answer

Peripheral lymph nodes

Answer

Inner Waldeyer ring

Question 8

The peripheral blood smear is characteristic of which of the following hematological conditions?

Accepted Answer

Hereditary spherocytosis

Answer

Thalassemia

Answer

Sickle cell anemia

Answer

G6PD deficiency

Question 9

What is the concentration of Hb A2 in thalassemia trait?

Accepted Answer

Slightly increased

Answer

Normal

Answer

Increased

Answer

Markedly increased

Question 10

CAR-T cells are used in the treatment of which of the following cancers?

Accepted Answer

Acute lymphoblastic leukemia

Answer

Soft tissue sarcoma

Answer

Medulloblastoma

Answer

Small cell lung carcinoma

Hematopathology — MCQs

Hematopathology — MCQs

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