Syncytial Variant of Nodular Sclerosis Classical Hodgkin's Lymphoma With Hemophagocytic Lymphohistiocytosis: A Rare and Under-Characterized Entity.

Syncytial variant of nodular sclerosis classical Hodgkin's lymphoma (NSCHL) is a rare morphological subtype of classical Hodgkin's lymphoma (CHL). The Hodgkin and Reed-Sternberg (HRS) cells in syncytial variant NSCHL form confluent sheets. Few studies have reported the development of concurrent hemophagocytic lympho-histiocytosis (HLH) with Hodgkin's lymphoma; however, no case of this variant with HLH has been reported before. We present a case of a rare variant of NSCHL with secondary HLH in a 15-year-old male patient presenting with B symptoms of high-grade fever, weight loss, and left-sided cervical swelling. Hemogram revealed pancytopenia. Biochemical investigations were suggestive of HLH. Lymph node biopsy had areas of sclerosis, nodularity with atypical mitosis, and clusters of HRS cells. The bone marrow examination showed an increase in plasma cells and histiocytes, with hemophagocytosis, along with the presence of HRS cells and epithelioid cell granulomas in a reactive background comprising plasma cells and eosinophils. Immunohistochemistry was suggestive of Hodgkin's lymphoma, and a final diagnosis of syncytial variant of NSCHL with secondary HLH was made. Patients frequently exhibit B symptoms and are often diagnosed at a more advanced stage of disease.