Composite lymphomas composed of two non-Hodgkin small B cell lymphomas are infrequent and composite mantle cell lymphoma and marginal zone lymphoma is extremely rare with 3 cases reported to date. The case presented herein is the first report of extranodal composite CD5 negative mantle cell lymphoma and partially CD5 positive marginal zone lymphoma. The identification of these two histologically indistinct and immunophenotypically unconventional lymphoma components requires a comprehensive study including careful interpretation of immunohistochemical results and the fluorescence in-situ hybridization (FISH) analysis of t(11;14). Clinically, the diagnosis of this composite lymphoma is compatible with patient’s aggressive disease course. Molecular studies for immunoglobulin gene rearrangement indicate that the mantle cell lymphoma and marginal zone lymphoma components are derived from different clones, supporting the notion that composite lymphomas of non-Hodgkin small B cell lymphomas are often biclonal.
composite lymphoma, mantle cell lymphoma, marginal zone lymphoma, CD5 negative mantle cell lymphoma
How to Cite
Yan, J. & Wu, Y., (2017) “Unusual extranodal composite CD5 negative mantle cell lymphoma and CD5 positive marginal zone lymphoma”, Hematopathology 2(1), p.50-57.