Abstract
Context Richter Transformation (RT), also known as Richter's syndrome, is defined as the development of an aggressive large-cell lymphoma in the context of underlying chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL). The incidence of RT ranges from 2 to 9% of all CLL/SLL cases and usually has a poor prognosis with an average survival of five to eight months. Hodgkin lymphoma (HL) is reported to be one of the rarest histological types of RT. Clinically, patients are presented with symptomatic deterioration after achieving disease remission. Due to data scarcity, the recommendations on therapy are limited. The response rate and clinical endpoints are suggested to be slightly inferior compared to in de novo HL but higher than other forms of RT. Objective The objective of this report is to fill the gap in the data and knowledge regarding rare subtype of Richter Transformation and its treatment efficacy. Design This is an ongoing case report with an updating follow-up. Setting This case was evaluated and managed in two independent medical centers: one in Armenia and one in the U.S. Patients or other participants A 67-year-old male with no remarkable previous medical history was diagnosed with CLL/SLL in 2015 and successfully received four cycles of fludarabine and cyclophosphamide as treatment with the elimination of leukocytosis and reduction of adenopathy as a result. After four years in a stable health condition, the patient developed regular fever once a week, as well as right-sided cervical and axillary lymphadenopathy without night sweats, chills, or weight loss. Interventions After a diagnostic workup, including blood tests and CT scans of the chest, neck, and abdomen, as well as a pathohistological examination of a right cervical lymph node biopsy, the diagnosis of Hodgkin variant of RT was established. The decision was made to proceed with the six-course treatment of ABVD regimen (doxorubicin, bleomycin, vinblastine, and dacarbazine). The patient is currently under the supervision of specialized medical team care and demonstrates high tolerability to the treatment.
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