LQCL2407 - CMLE - Atypical Lymphocytosis With Villous Lymphocytes in a Patient With Splenic Marginal Zone Lymphoma-Atypical Lymphocytosis With Villous Lymphocytes in a Patient With Splenic Marginal Zone Lymphoma

Atypical Lymphocytosis With Villous Lymphocytes in a Patient With Splenic Marginal Zone Lymphoma

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The document discusses a case study of an 80-year-old woman with splenic marginal zone lymphoma (SMZL) presenting with lymphocytosis and thrombocytopenia, along with the morphological and immunophenotypic characteristics of the disease. The patient's diagnosis was confirmed through flow cytometry, bone marrow biopsy, and molecular studies showing mutations in NOTCH2 and IGLL5. SMZL is described as a rare, indolent B-cell neoplasm primarily affecting the spleen, with symptoms including fatigue, weight loss, and splenomegaly. Diagnosis and management involve a comprehensive approach, with treatment options ranging from watchful waiting to chemotherapy, immunotherapy, and splenectomy. Prognosis is generally favorable, with a 67% to 95% 10-year survival rate. The differential diagnosis includes other lymphoproliferative disorders like chronic lymphocytic leukemia and hairy cell leukemia. The document highlights the importance of timely recognition of atypical lymphoid cells in peripheral blood for prompt diagnosis and management. Additionally, it provides insights into the morphological features and treatment options for SMZL, emphasizing the need for clinical correlation and immunophenotypic studies for accurate diagnosis.

Keywords

splenic marginal zone lymphoma

SMZL

lymphocytosis

thrombocytopenia

NOTCH2

IGLL5

indolent B-cell neoplasm

spleen

chemotherapy

immunophenotypic studies