Hematopathology Practice Questions

Q: Which of the following is the only form of Hodgkin disease (HD) more common in women?

Nodular-sclerosing Hodgkin disease. **Explanation:** In Hodgkin Lymphoma (HL), there is a distinct gender distribution pattern. While the overall incidence of Hodgkin Lymphoma is higher in males, **Nodular Sclerosis Hodgkin Lymphoma (NSHL)** is the notable exception. It is the only subtype that shows a **female preponderance** (or an equal gender distribution depending on the study population) and is the most common subtype overall, accounting for approximately 60–80% of cases [1]. It typically affects adolescents and young adults and frequently presents with a mediastinal mass [2]. **Analysis of Options:** * **Nodular Sclerosis (Correct):** Characterized by broad bands of collagen fibrosis surrounding nodules of tumor cells and the presence of **Lacunar cells** (a variant of Reed-Sternberg cells). It is the most common subtype in females and young adults [1]. * **Lymphocyte-Predominant (Incorrect):** This subtype has a strong male predominance (approx. 3:1). It is characterized by "Popcorn cells" (L&H cells) and generally carries the best prognosis. * **Mixed-Cellularity (Incorrect):** More common in males and older patients. It is strongly associated with EBV infection and presents with a diverse inflammatory background. * **Lymphocyte-Depleted (Incorrect):** The rarest subtype, seen most often in elderly or HIV-positive males. It carries the worst prognosis. **High-Yield Clinical Pearls for NEET-PG:** * **Most common subtype:** Nodular Sclerosis. * **Best prognosis:** Lymphocyte-Predominant. * **Worst prognosis:** Lymphocyte-Depleted. * **EBV Association:** Highest in Mixed-Cellularity and Lymphocyte-Depleted subtypes. * **Bimodal Age Distribution:** HL peaks at 15–35 years and again after 50 years. * **Reed-Sternberg (RS) Cells:** The hallmark of HL; they are CD15+ and CD30+ (except in the Nodular Lymphocyte Predominant type, which is CD20+).

Q: Wrinkled paper appearance of macrophage in bone marrow aspirate is characteristic of which condition?

Gaucher disease. **Explanation** **Gaucher disease** is the correct answer because it is characterized by the accumulation of glucocerebroside within macrophages due to a deficiency of the enzyme **glucocerebrosidase** (β-glucosidase). These lipid-laden macrophages are known as **Gaucher cells**. Under light microscopy, the cytoplasm of these cells has a classic **"wrinkled paper"** or **"crumpled silk"** appearance [1]. This unique morphology is caused by the intralysosomal accumulation of tubular bimolecular leaflets of glucocerebroside [1]. **Analysis of Incorrect Options:** * **A. Niemann-Pick disease:** This condition involves a deficiency of sphingomyelinase. The characteristic cells are **"foamy macrophages"** (vacuolated cytoplasm) rather than wrinkled ones [2]. * **C. Hemophagocytic lymphohistiocytosis (HLH):** This is characterized by activated macrophages engulfing erythrocytes, leukocytes, and platelets (**hemophagocytosis**), not lipid accumulation. * **D. Langerhans cell histiocytosis:** This features Birbeck granules (tennis-racket shaped) on electron microscopy and cells with grooved, **"coffee-bean"** nuclei, not wrinkled cytoplasm. **High-Yield Clinical Pearls for NEET-PG:** * **Gaucher Disease** is the most common lysosomal storage disorder. * **Biomarker:** Serum **ACE levels** and **TRAP** (Tartrate-resistant acid phosphatase) are often elevated. * **Clinical Triad:** Hepatosplenomegaly, bone involvement (Erlenmeyer flask deformity of the femur), and pancytopenia [1]. * **Pseudo-Gaucher cells:** These can be seen in conditions with high cell turnover, such as **Chronic Myeloid Leukemia (CML)** and Multiple Myeloma. **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, pp. 162-163. [2] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Genetic Disorders, pp. 161-162.

Q: A patient with anemia presents with peripheral blood smear showing features suggestive of thalassemia. Family history is positive. Which investigation is most appropriate to establish the diagnosis?

Hemoglobin (Hb) electrophoresis. **Explanation:** The clinical presentation of anemia, a positive family history, and a peripheral smear showing microcytic hypochromic cells with target cells strongly suggests a **Thalassemia syndrome**. **1. Why Hemoglobin (Hb) Electrophoresis is correct:** Hb electrophoresis is the **gold standard diagnostic test** for thalassemia. It works by separating different hemoglobin fractions (HbA, HbA2, and HbF) based on their electrical charge. * In **Beta-thalassemia trait**, there is a characteristic elevation of **HbA2 (>3.5%)** and sometimes HbF. [1] * In **Beta-thalassemia major**, HbA is nearly absent, replaced predominantly by HbF. * High-Performance Liquid Chromatography (HPLC) is an even more modern, automated alternative often used in clinical practice. **2. Why other options are incorrect:** * **ESR estimation:** This is a non-specific marker of inflammation and has no diagnostic value in hemoglobinopathies. * **Blood spherocyte estimation:** Spherocytes are characteristic of Hereditary Spherocytosis or Autoimmune Hemolytic Anemia, not Thalassemia (which shows target cells and leptocytes). [2] * **Bone marrow aspiration:** While it would show erythroid hyperplasia, it is an invasive procedure and cannot differentiate between various causes of microcytic anemia or confirm a genetic hemoglobin defect. [1] **Clinical Pearls for NEET-PG:** * **Mentzer Index:** (MCV/RBC count) 13 suggests Iron Deficiency Anemia. * **Peripheral Smear:** Look for "Target cells" (Codocytes) and "Basophilic stippling." [1] * **Confirmatory Test:** While electrophoresis is diagnostic for Beta-thalassemia, **Genetic testing (PCR)** is required to definitively diagnose Alpha-thalassemia.

Q: In the investigation of a patient with suspected essential thrombocythaemia, in which of the following genes may you find abnormalities?

CAL-R. **Explanation:** Essential Thrombocythaemia (ET) is a chronic myeloproliferative neoplasm (MPN) characterized by the autonomous overproduction of platelets [1]. The pathogenesis involves mutations that lead to constitutive activation of the **JAK-STAT signaling pathway**, driving megakaryopoiesis [1]. **1. Why CAL-R is correct:** Approximately 90% of ET cases are driven by one of three "driver mutations": * **JAK2 (V617F):** Present in ~50-60% of cases [1]. * **CALR (Calreticulin):** Found in ~25-30% of cases (specifically in those who are JAK2 negative) [1]. * **MPL:** Found in ~3-5% of cases [1]. **CALR** mutations (typically insertions or deletions in Exon 9) result in a mutant protein that activates the thrombopoietin receptor (MPL), leading to uncontrolled platelet production [1]. **2. Why the other options are incorrect:** * **BCL-2:** An anti-apoptotic protein associated with **Follicular Lymphoma** due to the t(14;18) translocation. * **BCR:** Part of the **BCR-ABL1** fusion gene (Philadelphia chromosome) seen in **Chronic Myeloid Leukemia (CML)** [1]. ET is specifically defined by the *absence* of the BCR-ABL1 rearrangement. * **c-MYC:** A proto-oncogene associated with **Burkitt Lymphoma** due to the t(8;14) translocation. **Clinical Pearls for NEET-PG:** * **Triple Negative ET:** Refers to the ~10% of patients who lack JAK2, CALR, and MPL mutations. * **Morphology:** Bone marrow shows increased numbers of enlarged, mature megakaryocytes with "staghorn" or deeply lobulated nuclei [1]. * **Clinical Feature:** Erythromelalgia (burning pain and erythema of hands/feet) is a classic symptom due to microvascular occlusion. * **Prognosis:** CALR-mutated ET generally has a lower risk of thrombosis compared to JAK2-mutated ET. **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. 627-628.

Q: Pappenheimer bodies are composed of:

Iron. **Explanation:** **Pappenheimer bodies** are abnormal basophilic granules found in red blood cells. They are composed of **ferritin (iron) aggregates** that have not been incorporated into hemoglobin [1]. 1. **Why Iron is Correct:** Under normal conditions, iron is transported to the mitochondria for heme synthesis. When there is iron overload or impaired utilization (as seen in Sideroblastic anemia), non-heme iron accumulates in the mitochondria. On a peripheral smear stained with **Wright-Giemsa**, these appear as small, irregular, dark-blue granules usually located at the periphery of the cell. Their identity as iron is confirmed using the **Prussian Blue (Perls') stain**, where they are referred to as **Siderotic granules**. 2. **Why Other Options are Incorrect:** * **Copper:** While copper deficiency can lead to sideroblastic-like changes, the bodies themselves are not made of copper. * **Lead:** Lead poisoning inhibits heme synthesis and causes **Basophilic Stippling**, which consists of **ribosomal precipitates (RNA)**, not iron. * **Zinc:** Excess zinc can cause a secondary copper deficiency, but it does not form intra-erythrocytic inclusions. **Clinical Pearls for NEET-PG:** * **Differential Diagnosis:** Pappenheimer bodies are most commonly seen in **Sideroblastic anemia**, **Splenectomy** (due to loss of splenic "pitting"), and **Hemolytic anemias**. * **Staining Distinction:** * **Wright-Giemsa:** Called Pappenheimer bodies. * **Prussian Blue:** Called Siderotic granules. * **Comparison:** Do not confuse with **Howell-Jolly bodies** (DNA remnants) or **Heinz bodies** (Denatured hemoglobin, seen in G6PD deficiency). **References:** [1] Kumar V, Abbas AK, et al.. Robbins and Cotran Pathologic Basis of Disease. 9th ed. Red Blood Cell and Bleeding Disorders, pp. 658-659.

Question 1

Which of the following is the only form of Hodgkin disease (HD) more common in women?

Accepted Answer

Nodular-sclerosing Hodgkin disease

Answer

Lymphocyte-predominant Hodgkin disease

Answer

Mixed-cellularity Hodgkin disease

Answer

Lymphocyte-depleted Hodgkin disease, reticular type

Question 2

In hemolytic anemias, what happens to the urobilinogen level in the blood?

Accepted Answer

Increase

Answer

Be absent

Answer

Decrease

Answer

Mildly decrease

Question 3

Hereditary spherocytosis is due to deficiency of which protein?

Accepted Answer

Ankyrin

Answer

Actin

Answer

Selectin

Answer

Integrin

Question 4

Wrinkled paper appearance of macrophage in bone marrow aspirate is characteristic of which condition?

Accepted Answer

Gaucher disease

Answer

Niemann-Pick disease

Answer

Hemophagocytic lymphohistiocytosis

Answer

Langerhans cell histiocytosis

Question 5

A patient with anemia presents with peripheral blood smear showing features suggestive of thalassemia. Family history is positive. Which investigation is most appropriate to establish the diagnosis?

Accepted Answer

Hemoglobin (Hb) electrophoresis

Answer

Erythrocyte Sedimentation Rate (ESR) estimation

Answer

Blood spherocyte estimation

Answer

Bone marrow aspiration

Question 6

In the investigation of a patient with suspected essential thrombocythaemia, in which of the following genes may you find abnormalities?

Accepted Answer

CAL-R

Answer

BCL-2

Answer

BCR

Answer

c-MYC

Question 7

A 40-year-old male presented with massive splenomegaly and mild hepatic enlargement. Peripheral blood film shows cells with hair-like projections that are positive for CD25 and CD103, and negative for CD23. What is the likely diagnosis?

Accepted Answer

Peripheral B-cell neoplasm

Answer

Precursor B-cell neoplasm

Answer

Precursor T-cell neoplasm

Answer

Peripheral T-cell neoplasm

Question 8

Pappenheimer bodies are composed of:

Accepted Answer

Iron

Answer

Copper

Answer

Lead

Answer

Zinc

Question 9

All of the following infections may be transmitted by blood transfusion, except?

Accepted Answer

Dengue virus

Answer

Parvovirus B-19

Answer

Cytomegalovirus

Answer

Hepatitis G virus

Question 10

Which of the following is not a feature of megaloblastic anemia?

Accepted Answer

Reticulocytosis

Answer

Thrombocytopenia

Answer

Megakaryocytes

Answer

Howell-Jolly bodies

Hematopathology — MCQs

Hematopathology — MCQs

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