Keyword: Hodgkin's Lymphoma

ABSTRACT: Richter's syndrome (RS) is a malignant transformation of chronic lymphocytic leukemia (CLL) or small lymphocytic lymphoma (SLL) into a more aggressive lymphoid neoplasm. Typically, this term refers to the development of diffuse large B-cell lymphoma (DLBCL), which accounts for 90-95% of transformations. Much more rarely (in less than 5% of cases), the so-called Hodgkin’s variant of Richter’s syndrome occurs—transformation into classical Hodgkin’s lymphoma (HL). [1] The Hodgkin’s variant is characterized by the appearance of Reed-Sternberg cells or their variants in the infiltrate, surrounded by an inflammatory background typical of HL. It most often develops in patients with a long history of CLL/SLL, sometimes in the context of immunosuppression or after treatment with purine analogs and monoclonal antibodies. [2] The clinical picture of the Hodgkin’s variant of Richter’s syndrome typically includes pronounced B-symptoms (fever, night sweats, weight loss), rapidly progressing enlargement of lymph nodes and/or splenomegaly. Unlike the DLBCL variant, the course may be somewhat less aggressive; however, the prognosis remains unfavorable compared to primary Hodgkin’s lymphoma. [3]