A Rare Presentation of Pancytopenia with Hemophagocytosis Lyphohistiocytosis: T cell/ Histiocyte Rich Large B cell Lymphoma
Journal Title: International Journal of Medical Science and Innovative Research (IJMSIR) - Year 2018, Vol 3, Issue 9
Abstract
T-cell/Histiocyte Rich Large B cell Lymphoma (T/HRLBCL) is a subtype of diffuse large B-cell lymphoma (DLBCL). This variety of lymphoma highlights the challenges in defining and classifying the specific subtypes of DLBCL. Predominantly, it is a nodal disease, with extranodal involvement, such as bone marrow, spleen, and liver. T/HRLBCL is characterized by paucity of large, atypical B-cells and surrounded in the background by a dense population of reactive T-cells and histiocytes. Morphologically, it is very similar to the Nodular Lymphocyte Predominant Hodgkin lymphoma (NLPHL). Hemophagocytic lymphohistiocytosis (HLH) is a clinical condition characterized by disarray of immune system. Cytotoxic T cells and natural killer cells functions are often impaired and most cases of lymphoma-associated with HLH are due to T cell related malignancies. HLH associated with B-cell lymphoma is uncommon. Here report a case of an immunocompetent individual presenting as pancytopenia, with secondary HLH and later diagnosed as a case of T/HRLBCL.
Authors and Affiliations
Dr Mathi Manoj Kumar R
Keywords
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