Hematopathology Practice Questions

Q: Poorest prognosis in Acute Myeloid Leukemia (AML) is seen in which cytogenetic abnormality?

Monosomy 7. **Explanation:** In Acute Myeloid Leukemia (AML), cytogenetics is the single most important predictor of treatment response and overall survival [1]. **Why Monosomy 7 is the Correct Answer:** Monosomy 7 (-7) or a deletion of the long arm of chromosome 7 (7q-) is categorized under the **Adverse/Poor Risk group** [1]. It is frequently associated with complex karyotypes, prior exposure to chemotherapy (therapy-related AML), or evolution from Myelodysplastic Syndrome (MDS). These cases are notorious for multi-drug resistance and a very high rate of relapse, often requiring Allogeneic Stem Cell Transplant as the only curative option [1]. **Analysis of Incorrect Options:** * **B. No cytogenetic abnormality:** This represents the **Intermediate Risk group** [1]. While not as favorable as specific translocations, it has a significantly better prognosis than Monosomy 7. * **C. t(15;17):** This is the hallmark of Acute Promyelocytic Leukemia (APL). It carries a **Favorable prognosis** due to its high sensitivity to All-trans Retinoic Acid (ATRA) and Arsenic Trioxide [1]. * **D. inv(16):** Along with t(8;21), this belongs to the Core Binding Factor (CBF) leukemias. It is categorized as a **Favorable prognosis** with high rates of complete remission following high-dose Cytarabine [1]. **High-Yield NEET-PG Pearls:** * **Best Prognosis:** t(15;17) followed by t(8;21) and inv(16) [1]. * **Worst Prognosis:** Monosomy 7, Monosomy 5, 11q23 abnormalities, and complex karyotypes (≥3 abnormalities) [1]. * **FLT3-ITD Mutation:** The most common molecular abnormality associated with a poor prognosis in otherwise normal cytogenetics. * **NPM1 and CEBPA Mutations:** Generally associated with a favorable prognosis in cytogenetically normal AML.

Q: Histopathological examination reveals the diagnosis as:

Hodgkin lymphoma. ***Hodgkin lymphoma*** - Characterized by the presence of **Reed-Sternberg cells** with **owl-eye nucleoli** and **binucleated giant cells**. - The classic histopathological appearance includes a **mixed inflammatory infiltrate** surrounding the characteristic malignant cells. *Ewing sarcoma* - Shows **small round blue cells** with **uniform nuclear morphology** and **high nuclear-to-cytoplasmic ratio**. - Typically positive for **CD99** immunostain and lacks the characteristic giant cells seen in lymphomas. *Kaposi Sarcoma* - Demonstrates **spindle cells** arranged in **fascicles** with **slit-like vascular spaces**. - Associated with **HHV-8 infection** and shows **extravasated red blood cells** with **hemosiderin deposits**. *Burkitt lymphoma* - Exhibits a **starry-sky pattern** due to **scattered tingible body macrophages** among uniform lymphoid cells. - Composed of **monomorphic medium-sized B cells** with **multiple nucleoli** and high mitotic activity.

Q: A 65-year-old patient presented with weakness, fatigue for 6 months, and mild abdominal discomfort. Examination revealed moderate splenomegaly. Lab findings showed severe normocytic normochromic anemia, neutropenia with monocytopenia, and thrombocytopenia. Bone marrow aspiration resulted in a 'dry' tap. A bone marrow biopsy was performed. Which of the following CD markers can be positive in this condition EXCEPT?

CD117. The clinical presentation of an elderly patient with pancytopenia (including characteristic **monocytopenia**), splenomegaly, and a **"dry tap"** on bone marrow aspiration strongly suggests **Hairy Cell Leukemia (HCL)**. HCL is a rare B-cell lymphoproliferative disorder where the bone marrow becomes fibrotic, necessitating a biopsy which typically shows "fried-egg" appearance cells. **Why CD117 is the Correct Answer:** * **CD117 (c-kit)** is a marker for hematopoietic stem cells and myeloid precursors. It is typically positive in **Acute Myeloid Leukemia (AML)** and Mastocytosis. It is **not** expressed in Hairy Cell Leukemia, making it the correct "except" choice. **Analysis of Incorrect Options (Markers positive in HCL):** * **CD11c:** An adhesion molecule (integrin) highly expressed in HCL. * **CD103:** Considered the most specific marker for HCL among the B-cell disorders. * **CD25:** The IL-2 receptor alpha chain, characteristically positive in HCL (unlike most other B-cell lymphomas). * *Note: Other positive markers include CD19, CD20, CD22, and Annexin A1 (most specific).* **Clinical Pearls for NEET-PG:** 1. **TRAP Positive:** Hairy cells show Tartrate-Resistant Acid Phosphatase (TRAP) activity. 2. **BRAF V600E Mutation:** Present in nearly 100% of classic HCL cases. 3. **Monocytopenia:** A high-yield diagnostic clue; HCL is one of the few conditions where pancytopenia specifically includes a lack of monocytes. 4. **Treatment:** Cladribine (2-CdA) is the drug of choice.

Q: What is the most common subtype of acute lymphoblastic leukemia (ALL) in children?

Pre-B cell ALL. **Explanation:** Acute Lymphoblastic Leukemia (ALL) is the most common pediatric malignancy. It is classified based on the lineage and maturity of the malignant lymphoblasts. **1. Why Pre-B cell ALL is Correct:** Approximately **80–85%** of all pediatric ALL cases are of **B-cell lineage**. Within this group, the **Early Pre-B (Pro-B)** and **Pre-B cell** subtypes are the most frequent. These cells are characterized by the expression of CD19, CD20, and CD10 (Common ALL Antigen or CALLA). The high prevalence of these immature B-cell precursors in the bone marrow during childhood development explains why this subtype is the most common. **2. Why Incorrect Options are Wrong:** * **Mature B cell ALL (Burkitt-type):** This accounts for only **2–3%** of cases. It is characterized by surface immunoglobulin expression and specific translocations like t(8;14). * **Pre-T cell ALL:** This accounts for about **12–15%** of cases. It typically presents in older adolescent males with a high white cell count and a mediastinal mass. * **Mature T cell ALL:** This is a rare subtype and significantly less common than the precursor (Pre-T) stage. **Clinical Pearls for NEET-PG:** * **Peak Age:** 2 to 5 years. * **Best Prognostic Marker:** Hyperdiploidy (>50 chromosomes) and t(12;21) [ETV6-RUNX1]. * **Poor Prognostic Marker:** Hypodiploidy and t(9;22) [Philadelphia Chromosome]. * **Common Site of Relapse:** Central Nervous System (CNS) and Testes (sanctuary sites). * **Immunophenotype:** CD10 (CALLA) positivity is a marker for a better prognosis compared to CD10 negative cases.

Q: What is the characteristic finding in the peripheral smear of a patient with hemolytic uremic syndrome?

Burr cells with fragmented red blood cells. **Explanation:** Hemolytic Uremic Syndrome (HUS) is characterized by the classic triad of **Microangiopathic Hemolytic Anemia (MAHA)**, thrombocytopenia, and acute kidney injury. The primary pathology involves endothelial injury, leading to the formation of microthrombi in small vessels (predominantly glomerular capillaries). As Red Blood Cells (RBCs) attempt to pass through these partially occluded vessels, they are mechanically shredded by fibrin strands. This process results in the hallmark finding on a peripheral smear: **Fragmented RBCs (Schistocytes/Helmet cells)** and **Burr cells (Echinocytes)**. **Analysis of Options:** * **Option D (Correct):** The combination of fragmented RBCs (schistocytes) and Burr cells is the diagnostic morphological feature of MAHA seen in HUS. * **Option A:** While Burr cells are seen in uremia (renal failure), they are not the *only* finding. In HUS, the mechanical fragmentation (schistocytes) is equally critical for diagnosis. * **Option B:** Anisopoikilocytosis refers to variation in size and shape; while present, it is a non-specific finding seen in many anemias (like Iron Deficiency) and is not diagnostic of HUS. * **Option C:** A leukemoid reaction (high WBC count) may occur in severe infections but is not a characteristic feature of the RBC morphology required to diagnose HUS. **High-Yield Clinical Pearls for NEET-PG:** * **Most common cause:** Shiga toxin-producing *E. coli* (STEC), specifically serotype **O157:H7**. * **Triad:** Microangiopathic Hemolytic Anemia + Thrombocytopenia + Acute Renal Failure. * **Lab findings:** Increased LDH, decreased haptoglobin, and a **Negative Direct Coombs Test** (distinguishes it from autoimmune hemolysis). * **Management:** Primarily supportive; **Antibiotics and Platelet transfusions are generally avoided** as they may worsen the toxin release and microthrombi formation.

Question 1

Poorest prognosis in Acute Myeloid Leukemia (AML) is seen in which cytogenetic abnormality?

Accepted Answer

Monosomy 7

Answer

No cytogenetic abnormality

Answer

t(15;17)

Answer

inv(16)

Question 2

What is the characteristic CD marker for Langerhans cells?

Accepted Answer

CD1

Answer

CD2

Answer

CD4

Answer

CD56

Question 3

Histopathological examination reveals the diagnosis as:

Accepted Answer

Hodgkin lymphoma

Answer

Ewing sarcoma

Answer

Kaposi Sarcoma

Answer

Burkitt lymphoma

Question 4

A 65-year-old patient presented with weakness, fatigue for 6 months, and mild abdominal discomfort. Examination revealed moderate splenomegaly. Lab findings showed severe normocytic normochromic anemia, neutropenia with monocytopenia, and thrombocytopenia. Bone marrow aspiration resulted in a 'dry' tap. A bone marrow biopsy was performed. Which of the following CD markers can be positive in this condition EXCEPT?

Accepted Answer

CD117

Answer

CD11c

Answer

CD103

Answer

CD25

Question 5

What is the most common subtype of acute lymphoblastic leukemia (ALL) in children?

Accepted Answer

Pre-B cell ALL

Answer

Mature B cell ALL

Answer

Pre-T cell ALL

Answer

Mature T cell ALL

Question 6

What happens to the haptoglobin level in hemolytic anemia?

Accepted Answer

Haptoglobin levels decrease

Answer

Haptoglobin levels increase

Answer

Remains the same

Answer

May increase or decrease depending on chronicity of the disease

Question 7

All of the following precipitate sickling of HBS except?

Accepted Answer

Increased pH

Answer

Dehydration

Answer

Hypoxia

Answer

Infections

Question 8

A 22-year-old male presents with fatigue, recurrent fever, and enlarged cervical lymph nodes. Peripheral blood smear shows numerous atypical lymphocytes. A biopsy of an enlarged lymph node reveals expansion of lymphoid follicles with preserved underlying architecture, and numerous atypical lymphocytes in the paracortical areas. What is the most likely diagnosis?

Accepted Answer

Infectious mononucleosis

Answer

AIDS

Answer

Burkitt's lymphoma

Answer

Hodgkin's Disease

Question 9

What is the characteristic finding in the peripheral smear of a patient with hemolytic uremic syndrome?

Accepted Answer

Burr cells with fragmented red blood cells

Answer

Burr cell

Answer

Anisopoikilocytosis

Answer

Leukemoid reaction

Question 10

A 40-year-old woman complains of fatigue and nausea of 3 months in duration. Physical examination reveals numerous pustules on the face, as well as splenomegaly and hepatomegaly. Laboratory studies show hemoglobin of 6.3 g/dL and platelets of 50,000/mL. A peripheral smear shows malignant cells with Auer rods. The patient develops diffuse purpura, bleeding from the gums, and laboratory features of disseminated intravascular coagulation (DIC). Which of the following is the appropriate diagnosis?

Accepted Answer

Acute promyelocytic leukemia

Answer

Acute lymphoblastic leukemia

Answer

Acute megakaryocytic leukemia

Answer

Chronic myelogenous leukemia

Hematopathology — MCQs

Hematopathology — MCQs

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