Abstract
T-cell/histiocyte-rich B-cell lymphoma is a high-grade, morphologic variant of diffuse large B-cell lymphoma. T-cell/histiocyte-rich B-cell lymphoma. It is rare as a primary splenic involvement and is usually reported as a second malignancy after hairy cell leukemia. Here, we report the first case that describes the occurrence of primary splenic T-cell/histiocyte-rich B-cell lymphoma in a patient with a previous diagnosis of recurrent hairy cell leukemia. A 53-year-old male patient was diagnosed with hairy cell leukemia in 1996 and achieved complete remission with Pentostatin. Then, recurrence of hairy cell leukemia was diagnosed in 2015 and treated with Cladribine. In 2016, he presented with B symptoms and hypersplenism. Therapeutic and diagnostic splenectomy was performed. Histopathological study with immunohistochemistry evaluation revealed the presence of T-cell/histiocyte-rich B-cell lymphoma. Therefore, second malignancies should be considered in patients with a previous neoplasm when symptoms recur or develop.