Chronic lymphocytic leukemia/small lymphocytic lymphoma with Hodgkin Reed-Sternberg-like cells and subsequent transformation to EBV-positive diffuse large B-cell lymphoma – A case report and literature review

Abstract

A subset of chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) patients develop Richter transformation (RT), classically resulting in diffuse large B-cell lymphoma (RT-DLBCL); however, a minority of cases result in Hodgkin lymphoma (RT-HL). RT-HL can be of type 1 or 2. RT-HL1 consists of Hodgkin-Reed Sternberg (HRS) cells in a background of CLL, and RT-HL2 has HRS cells in the typical inflammatory background of classical HL (CHL). Here we describe a rare case of CLL with an increased number of HRS-like cells which evolved to Epstein–Barr virus (EBV)-positive DLBCL over 2 years. The patient was a 48-year-old man who initially presented with right inguinal lymphadenopathy and was found to have both CLL/SLL and HSV lymphadenitis, which was then treated with valacyclovir and acyclovir prophylaxis. Approximately 1 year later, he developed diffuse lymphadenopathy with CLL and scattered EBV-positive HRS-like cells, consistent with RT-HL1, for which he received rituximab. After 3 months, he again developed diffuse lymphadenopathy with worsening symptoms. The patient was diagnosed with EBV-positive DLBCL by lymph node biopsy and responded well to 6 cycles of R-CHOP. This is the first case of CLL with RT-HL1 predating RT-DLBCL. While RT-HL1 and RT-DLBCL have been described previously, the potential for an association between these entities is cryptic. As more cases are reported, this relationship can be studied in greater detail.

Keywords

• CLL/SLL
• Richter transformation
• EBV infection
• Hodgkin Reed-Sternberg-like cells
• Classical Hodgkin Lymphoma
• Diffuse large B-cell lymphoma