[Splenic marginal zone lymphoma]

Area of Science:

• Hematology
• Oncology
• Pathology

Background:

• Splenic marginal zone lymphoma (SMZL) is a rare B-cell neoplasm, defined by the 2016 WHO classification.
• SMZL accounts for less than 2% of non-Hodgkin lymphomas and typically affects individuals over 50.
• Diagnostic challenges can arise, particularly in differentiating SMZL from other lymphoid malignancies.

Observation:

• A case report of a 72-year-old patient presenting with massive splenomegaly is detailed.
• Computed tomography (CT) confirmed splenomegaly, with normal laboratory findings.
• Splenectomy was performed, revealing a widespread lymphomatous proliferation of small cells.

Findings:

• Microscopic examination showed CD20-positive cells, with negative CD5 and CD43 markers, leading to a diagnosis of SMZL.
• The diagnosis was supported by anatomopathological evaluation of the spleen, showing involvement of the white pulp.
• Tumor cells expressed B lymphoid markers (CD19, CD20, CD22, CD79) but lacked CD5, CD10, cyclin D1, and CD43.

Implications:

• SMZL is an indolent lymphoma with non-codified treatment strategies, influenced by prognostic factors.
• Accurate diagnosis is crucial for appropriate management and to mitigate the risk of transformation into large cell lymphoma.
• This case highlights the importance of pathological assessment in diagnosing SMZL, especially when presenting with significant splenomegaly.