Abstract

Approximately one-fourth of non-Hodgkin's lymphomas (NHLs) present with an extranodal origin. Primary and secondary involvements of the breast by lymphoma are rare because of the paucity of lymphoid tissue in the breast. Primary breast small lymphocytic lymphoma (SLL) typically presents as a manifestation of widespread chronic lymphocytic leukemia (CLL). A 58-year-old female presented to our clinic with a palpable breast mass. She had no cytopenias and her absolute peripheral B lymphocyte count was less than 5000/microL. The mass was biopsied and histology with immunohistochemistry showed neoplastic cells positive for CD23 and CD5 confirming the diagnosis of small B cell lymphocytic lymphoma of the breast. Further imaging revealed extensive mediastinal and retroperitoneal lymphadenopathy. Histopathology of bone marrow biopsy revealed diffuse infiltration with SLL. The patient was treated with six cycles of fludarabine, cyclophosphamide and rituximab (FCR) with excellent clinical response. To our knowledge, this is the first case of SLL infiltration of the breast without CLL treated successfully with FCR.

Highlights

  • Primary breast lymphoma is a rare disease entity

  • Small lymphocytic lymphoma (SLL) is a mature B cell neoplasm characterized by a progressive accumulation of functionally incompetent lymphocytes, which are monoclonal in origin

  • It manifested as a breast tissue infiltrate with the morphology and immunophenotype of chronic lymphocytic leukemia (CLL) with an insufficient number of circulating leukemia cells to fulfill the criteria for CLL

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Summary

Introduction

Primary breast lymphoma is a rare disease entity. They comprise only 0.5% to 1% of non-Hodgkin’s lymphoma (NHL) and of breast malignancies, respectively. We present a unique case of primary small lymphocytic lymphoma of the breast with bone marrow involvement that was successfully treated with fludarabine, cyclophosphamide and rituximab. The final pathologic diagnosis was consistent with primary small lymphocytic lymphoma of the breast rather than chronic lymphocytic leukemia. Bone marrow biopsy was done and histopathology revealed diffuse involvement with small lymphocytic lymphoma (Figure 5). The final clinicopathologic diagnosis was small lymphocytic lymphoma (Stage 4) with breast and bone marrow involvement. She was subsequently treated with the FCR regimen comprising of fludarabine, cyclophosphamide and rituximab which resulted in clinical and radiologic remission.

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