Abstract
Primary splenic lymphoma (PSL) is a rare malignancy representing about 1% of all lymphoproliferative disorders, when using a strict definition that allows only involvement of spleen and hilar lymph nodes. In contrast, secondary low-grade B-cell lymphomas in the spleen, such as follicular lymphomas (FL), lymphoplasmacytic lymphoma and chronic lymphocytic leukemia/ small lymphocytic lymphoma, particularly as part of advanced stage disease, are more common. Indolent B cell lymphomas expressing CD10 almost always represent FL, which in its primary splenic form is the focus of this review. Primary splenic follicular lymphoma (PSFL) is exceedingly infrequent. This type of lymphoproliferative disorder is understudied and, in most cases, clinically characterized by splenomegaly or cytopenias related to hypersplenism. The diagnosis requires correlation of histopathology of spleen, blood and/or bone marrow with the correct immunophenotype (determined by flow cytometry and/or immunohistochemistry) and if necessary, additional molecular profiling. Management of this incurable disease is evolving, and splenectomy remains the mainstream treatment for stage I PSFL.
Highlights
Neprilysin (CD10) is a cell surface zinc-dependent metalloendopeptidase expressed in normal and neoplastic hematopoietic and non-hematopoietic tissue [1]
Primary splenic follicular lymphoma (PSFL) accounts for a small fraction of Primary splenic lymphoma (PSL) and only a few case series are available on this entity [17,18,19]
follicular lymphomas (FL) is morphologically composed of a heterogeneous lymphoid proliferation including small/medium sized cleaved cells with inconspicuous nucleoli and larger cells with round/oval/convoluted vesicular nuclei, membrane bound nucleoli and visible cytoplasm admixed in various proportions (Figure 1)
Summary
Neprilysin (CD10) is a cell surface zinc-dependent metalloendopeptidase expressed in normal and neoplastic hematopoietic and non-hematopoietic tissue [1]. Benign and neoplastic B cells of germinal center derivation, and immature lymphoid cells are positive for CD10 Detection of this antigen (in conjunction with CD5 and CD23) is invaluable for the classification of mature and immature lymphoid neoplasms (see below) and was used (together with CD5) to organize this review series (Aggarwal, Auerbach, Gomes da Silva Cross Reference). Follicular lymphoma (FL) is a mature B-cell non-Hodgkin lymphoma derived from germinal center B-lymphocytes (centrocytes and centroblasts) that generally has at least a partially follicular/nodular histologic pattern [9]. A literature review highlighting unique characteristics and therapeutic management of PSFL will be presented
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