A 39-year-old woman presented with increasing fatigue, abdominal fullness, nausea, upper abdominal left quadrant pain, menometrorrhagia, and bruising. Evaluation revealed moderate pancytopenia (Hb: 9.2 gm/dL; HCT: 28%; WBC: 3,800/uL; Differential count: neutrophils: 67%; monocytes: 0%; lymphocytes: 28%; atypical lymphocytes: 5%). An enlarged spleen extending across the umbilicus was palpable. Splenectomy revealed a 1,535 g spleen measuring 27.0 x 17.0 x 9.0 cm. Grossly, there was red pulp expansion without any lesions. Immunohistochemically, the lesional cells are positive for CD20, CD10, and DBA.44 and negative for CD3 and CD43. Flow cytometry revealed a neoplastic B-cell population, monotypic kappa, positive for CD19, CD20, CD22, CD11c, CD25, and CD103.
The master list with the correct answer
• Extramedullary hematopoiesis
• Gaucher disease
• Hairy cell leukemia
• Kaposi sarcoma
• Splenic marginal zone lymphoma
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• Appropriate diagnosis
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