Hematopathology Practice Questions

Q: CD19 positive, CD22 positive, CD103 positive monoclonal B-cells with bright kappa positivity, comprising 60% of peripheral blood lymphoid cells, were found on flow cytometric analysis in a 55-year-old man with massive splenomegaly and a total leukocyte count of 3.3 × 10^9/L. What is the most likely diagnosis?

Hairy cell leukemia. ### Explanation The correct diagnosis is **Hairy Cell Leukemia (HCL)**. This is a rare B-cell lymphoproliferative disorder typically seen in middle-aged men, characterized by the classic triad of **splenomegaly** (often massive), **pancytopenia** (due to bone marrow fibrosis and splenic sequestration), and **monocytosis-absentia**. **Why HCL is the correct answer:** The flow cytometry profile provided is pathognomonic for HCL. While CD19 and CD22 confirm a B-cell lineage, the presence of **CD103** is a highly specific marker for Hairy Cell Leukemia. Other characteristic markers (not mentioned here but high-yield) include CD11c, CD25, and Annexin A1. The "bright" light chain (kappa) expression is also typical of the mature B-cell phenotype seen in HCL. **Why the other options are incorrect:** * **Splenic lymphoma with villous lymphocytes (SLVL):** While it presents with splenomegaly, it is typically **CD103 negative** and does not show the same degree of cytopenia as HCL. * **Mantle cell lymphoma (MCL):** MCL is characterized by **CD5 positivity** and the t(11;14) translocation leading to Cyclin D1 overexpression. It is CD103 negative. * **B-cell prolymphocytic leukemia (B-PLL):** This presents with a very high white cell count (usually >100 × 10⁹/L) and prominent nucleoli in prolymphocytes, unlike the leucopenia/pancytopenia seen in this case. **High-Yield Clinical Pearls for NEET-PG:** * **Morphology:** "Hairy" cytoplasmic projections on peripheral smear. * **Bone Marrow:** Often results in a **"Dry Tap"** due to increased reticulin fibrosis; biopsy shows a **"Fried Egg" appearance**. * **Cytochemistry:** **TRAP positive** (Tartrate-Resistant Acid Phosphatase). * **Genetic Marker:** **BRAF V600E** mutation is present in nearly 100% of classic HCL cases. * **Treatment of Choice:** Cladribine (2-CdA) or Pentostatin.

Q: Plasma cell dyscrasias include all of the following except?

Systemic lupus erythematosus. **Explanation:** **Plasma cell dyscrasias** are a group of disorders characterized by the neoplastic proliferation of a single clone of plasma cells. These cells typically secrete a monoclonal immunoglobulin or a fragment thereof (M-protein/paraprotein), which can be detected in the serum or urine. **Why Systemic Lupus Erythematosus (SLE) is the correct answer:** SLE is a chronic **autoimmune disease** characterized by the loss of self-tolerance and the production of a wide array of autoantibodies (like ANA and anti-dsDNA). While it involves B-cell hyperactivity, it is **not** a monoclonal neoplastic proliferation of plasma cells. Therefore, it does not fall under the category of plasma cell dyscrasias. **Analysis of incorrect options:** * **Waldenström’s Macroglobulinemia:** A lymphoplasmacytic lymphoma where malignant cells secrete monoclonal **IgM**, leading to hyperviscosity syndrome. * **Heavy Chain Disease:** A rare B-cell proliferative disorder where plasma cells produce only truncated heavy chains without light chains. * **Monoclonal Gammopathy (MGUS):** The most common plasma cell dyscrasia, characterized by the presence of M-protein in the absence of end-organ damage (CRAB features). It is often a precursor to Multiple Myeloma. **High-Yield Clinical Pearls for NEET-PG:** * **Multiple Myeloma** is the prototype plasma cell dyscrasia. Look for the **CRAB** mnemonic: **C**alcium elevation, **R**enal insufficiency, **A**nemia, and **B**one lesions (lytic). * **Diagnosis:** The presence of **"Clock-face" or "Cartwheel" nuclei** in plasma cells on histology is a classic descriptor. * **Bence-Jones Proteins:** These are monoclonal light chains found in the urine of patients with plasma cell dyscrasias; they precipitate at 40-60°C and redissolve at 100°C. * **Russell Bodies:** Cytoplasmic inclusions of immunoglobulins found in plasma cells.

Question 1

CD19 positive, CD22 positive, CD103 positive monoclonal B-cells with bright kappa positivity, comprising 60% of peripheral blood lymphoid cells, were found on flow cytometric analysis in a 55-year-old man with massive splenomegaly and a total leukocyte count of 3.3 × 10^9/L. What is the most likely diagnosis?

Accepted Answer

Hairy cell leukemia

Answer

Splenic lymphoma with villous lymphocytes

Answer

Mantle cell lymphoma

Answer

B-cell prolymphocytic leukemia

Question 2

Which of the following does not cause an increase in spleen size in later stages?

Accepted Answer

Sickle cell anaemia

Answer

Cirrhosis

Answer

Infectious mononucleosis

Answer

Hairy cell leukaemia

Question 3

Peripheral blood smear showing spur cells and stomatocytes is suggestive of which condition?

Accepted Answer

Chronic liver disease

Answer

Alcoholic hepatitis

Answer

Chronic kidney disease

Answer

Hemolytic uremic syndrome

Question 4

Basophilic stippling is:

Accepted Answer

Ribosomal inclusions

Answer

Iron deposits

Answer

DNA remnants

Answer

Denatured Hemoglobin

Question 5

Which disorder is most likely associated with erythroid hyperplasia in the bone marrow?

Accepted Answer

7 to 10 days after a gastrointestinal bleed

Answer

Anemia of chronic disease

Answer

Thalassemia minor

Answer

Iron deficiency

Question 6

Plasma cell dyscrasias include all of the following except?

Accepted Answer

Systemic lupus erythematosus

Answer

Waldenstrom's macroglobulinemia

Answer

Heavy chain disease

Answer

Monoclonal gammopathy

Question 7

Bence Jones proteins are derived from:

Accepted Answer

Gamma Globulins

Answer

Alpha Globulins

Answer

Beta Globulins

Answer

Delta Globulins

Question 8

Which of the following can cause thrombocytopenia?

Accepted Answer

Heparin, DIC, Hemolytic uremic syndrome (HUS)

Answer

Heparin, DIC, Henoch-schonlein purpura (HSP)

Answer

DIC, Henoch-schonlein purpura (HSP), Hemolytic uremic syndrome (HUS)

Answer

Heparin, Henoch-schonlein purpura (HSP), Hemolytic uremic syndrome (HUS)

Question 9

Which of the following conditions, characterized by spherocytes on peripheral smear, is Coombs negative?

Accepted Answer

G6PD Deficiency

Answer

Paroxysmal nocturnal hemoglobinuria

Answer

Hemolytic disease of newborn

Answer

Autoimmune Hemolytic Anemia (AIHA)

Question 10

Sickle cell anemia is usually associated with all of the following, except:

Accepted Answer

Normal reticulocyte count

Answer

Shortened RBC life span

Answer

Abnormality in Hemoglobin

Answer

Polymer formation is reversible

Hematopathology — MCQs

Hematopathology — MCQs

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