Hematopathology Practice Questions

Q: Hairy cell leukemia is which type of tumor?

B cell tumor. **Explanation:** **Hairy Cell Leukemia (HCL)** is a rare, chronic lymphoproliferative disorder characterized by the proliferation of mature, small B-lymphocytes with characteristic "hairy" cytoplasmic projections [1]. **Why Option A is Correct:** HCL is definitively a **B-cell neoplasm**. The cells express pan-B-cell markers such as **CD19, CD20, and CD22**. More specifically, HCL is identified by the highly specific expression of **CD11c, CD25, CD103, and CD123**. The underlying molecular hallmark in nearly 100% of cases is the **BRAF V600E mutation**, which leads to constitutive activation of the RAF-MEK-ERK pathway, driving B-cell proliferation. **Why Other Options are Incorrect:** * **Option B & C:** While T-cell and NK-cell leukemias exist (e.g., T-cell Prolymphocytic Leukemia or Large Granular Lymphocytic Leukemia), HCL is strictly a B-lineage malignancy. There is no T-cell or NK-cell variant of classic Hairy Cell Leukemia. **High-Yield Clinical Pearls for NEET-PG:** * **Clinical Triad:** Splenomegaly (often massive), Pancytopenia, and "Dry tap" on bone marrow aspiration due to associated **reticulin fibrosis** [1]. * **Morphology:** Cells show "fried egg appearance" in bone marrow biopsies. * **Cytochemistry:** Historically diagnosed using **TRAP (Tartrate-Resistant Acid Phosphatase)** positivity, though flow cytometry is now the gold standard. * **Treatment:** Highly sensitive to purine analogs like **Cladribine** (Drug of Choice) and Pentostatin. * **Key Association:** Unlike most leukemias, HCL is notably associated with **monocytopenia**. **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, p. 612.

Q: The histological feature shown is seen in which of the following conditions?

Burkitt’s lymphoma. ***Burkitt's lymphoma*** - The **"starry sky" pattern** is pathognomonic for Burkitt's lymphoma, characterized by **tingible body macrophages** scattered among sheets of **monomorphic lymphoid cells**. - This high-grade B-cell lymphoma shows **rapid cell turnover** with macrophages phagocytosing apoptotic debris, creating the distinctive starry appearance. *Hodgkin's lymphoma* - Distinguished by the presence of **Reed-Sternberg cells** (large, multinucleated giant cells) which are absent in the starry sky pattern. - Shows **mixed inflammatory infiltrate** including eosinophils, plasma cells, and lymphocytes, not the monomorphic appearance of Burkitt's. *Non-Hodgkin's lymphoma* - This is a **broad category** that technically includes Burkitt's lymphoma, but the starry sky pattern is **specific to Burkitt's** subtype. - Other NHL subtypes like **follicular lymphoma** or **DLBCL** have different histological patterns without the characteristic starry sky appearance. *All of the above* - Incorrect because the **starry sky pattern** is **pathognomonic specifically for Burkitt's lymphoma**. - While Burkitt's is technically a type of NHL, the distinctive histological feature shown is **not seen** in Hodgkin's lymphoma or other NHL subtypes.

Q: Low LAP score may be seen in the following EXCEPT:

Polycythemia vera. **Explanation:** The **Leukocyte Alkaline Phosphatase (LAP) score** (also known as the Neutrophil Alkaline Phosphatase/NAP score) measures the activity of the enzyme alkaline phosphatase within the secondary granules of mature neutrophils. This score is a classic diagnostic tool used to differentiate between a **Leukemoid Reaction** (high score) and **Chronic Myeloid Leukemia** (low score). **Why Polycythemia Vera is the correct answer:** In **Polycythemia Vera (PV)**, which is a myeloproliferative neoplasm (MPN), there is typically an **increased LAP score**. The question asks for the condition where a low LAP score is *not* seen (the "EXCEPT"). Since PV is associated with an elevated score, it is the correct choice [1]. **Analysis of Incorrect Options (Conditions with Low LAP Score):** * **CML (Chronic Myeloid Leukemia):** This is the classic cause of a **low LAP score** due to the presence of functionally immature neutrophils that lack the enzyme [1]. * **Paroxysmal Nocturnal Hemoglobinuria (PNH):** This is a stem cell disorder where the GPI-anchor is missing. Since LAP is a GPI-anchored protein, its expression is significantly **decreased**. * **Autoimmune Hemolytic Anemia (AIHA):** While not a primary diagnostic marker for AIHA, certain types of anemia and marrow stress can result in a **low LAP score**. **High-Yield Clinical Pearls for NEET-PG:** * **High LAP Score:** Leukemoid reaction, Pregnancy (3rd trimester), Polycythemia Vera, and Down Syndrome [1]. * **Low LAP Score:** CML, PNH, Hypophosphatasia, and Aplastic Anemia [1]. * **Normal Range:** 40–100. * **Modern Shift:** In clinical practice, the LAP score has largely been replaced by cytogenetics (Philadelphia chromosome) and molecular testing (BCR-ABL1) for CML diagnosis [1]. **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. 624-627.

Q: Which is the most immunogenic RBC blood group system among the given options?

Kell. The immunogenicity of a blood group antigen refers to its ability to provoke an immune response (antibody production) in an individual who lacks that antigen. **1. Why Kell is Correct:** The **Kell (K)** antigen is the **most immunogenic** non-ABO/Rh red cell antigen. After the D antigen (Rh system), the K antigen is the most likely to cause sensitization. Approximately 5-10% of K-negative individuals will develop anti-K antibodies if transfused with one unit of K-positive blood. These antibodies (IgG) are clinically significant as they cause severe **Hemolytic Disease of the Fetus and Newborn (HDFN)** and delayed hemolytic transfusion reactions. **2. Why other options are incorrect:** * **Duffy (Fy) & Kidd (Jk):** While both can cause transfusion reactions and HDFN, they are significantly less immunogenic than Kell. * **Lewis (Le):** Lewis antigens are not integral to the RBC membrane (they are adsorbed from plasma). Lewis antibodies are usually naturally occurring IgM and are rarely clinically significant because they are neutralized by free Lewis substances in the recipient's plasma [1]. **High-Yield Clinical Pearls for NEET-PG:** * **Hierarchy of Immunogenicity:** Rh (D) > Kell (K) > others (c, E, Fya, Jka) [1]. * **Kell & Erythropoiesis:** Unlike Rh-HDFN, Kell-HDFN causes anemia not just by hemolysis, but also by **suppressing erythropoiesis** (anti-K destroys Kell-expressing erythroid precursors in the bone marrow) [2]. * **McLeod Phenotype:** A rare condition associated with the lack of Kx protein (linked to Kell), characterized by acanthocytosis and late-onset muscular dystrophy. **References:** [1] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 627-628. [2] Cross SS. Underwood's Pathology: A Clinical Approach. 6th ed. Common Clinical Problems From Blood And Bone Marrow Disease, pp. 603-604.

Question 1

Hairy cell leukemia is which type of tumor?

Accepted Answer

B cell tumor

Answer

T cell tumor

Answer

NK cell tumor

Answer

All of the above

Question 2

The histological feature shown is seen in which of the following conditions?

Accepted Answer

Burkitt’s lymphoma

Answer

Hodgkin’s lymphoma

Answer

Non-Hodgkin’s lymphoma

Answer

All of the above

Question 3

Which of the following statements regarding leukemias is true?

Accepted Answer

Acute lymphoid leukemia in individuals less than 1 year old has a poor prognosis.

Answer

Acute lymphoid leukemia commonly occurs beyond 50 years of age.

Answer

Hairy cell leukemia in individuals less than 50 years old has a good prognosis.

Answer

Chronic lymphocytic leukemia commonly occurs in individuals less than 50 years of age.

Question 4

Low LAP score may be seen in the following EXCEPT:

Accepted Answer

Polycythemia vera

Answer

CML

Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Autoimmune hemolytic anemia

Question 5

Which of the following toxic states is not associated with anemia?

Accepted Answer

Listerosis

Answer

Clostridial sepsis

Answer

Snake venom

Answer

Lead poisoning

Question 6

Which is the most immunogenic RBC blood group system among the given options?

Accepted Answer

Kell

Answer

Duffy

Answer

Kidd

Answer

Lewis antigen

Question 7

Pancytopenia with hypercellular marrow may be seen due to all of the following except:

Accepted Answer

Dyskeratosis congenita

Answer

Myelodysplasia

Answer

Paroxysmal Nocturnal Hemoglobinuria

Answer

Sarcoidosis

Question 8

Which of the following are types of Hodgkin's lymphoma?

Accepted Answer

Lymphocyte depletion

Answer

Nodular sclerosis

Answer

Mixed cellularity

Answer

Mantle cell lymphoma

Question 9

A 36-year-old male presents with weakness, pallor, and bleeding gums. His HB was 7 g/dL, total leukocyte count was 110,000/mm³ with many immature white blood cells positive for MPO, and platelet count was 22,000/mm³. What is the most likely diagnosis?

Accepted Answer

Acute Myeloid Leukemia (AML)

Answer

Chronic Myeloid Leukemia (CML)

Answer

Acute Lymphoid Leukemia (ALL)

Answer

Chronic Lymphoid Leukemia (CLL)

Question 10

Microangiopathic hemolytic anemia (MAHA) is a component of all of the following, except?

Accepted Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Thrombotic thrombocytopenic purpura (TTP)

Answer

Hemolytic uremic syndrome (HUS)

Answer

Hemolysis with elevated liver enzymes and low platelet (HELLP) syndrome

Hematopathology — MCQs

Hematopathology — MCQs

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