Diagnostic and Therapeutic Difficulties in Diffuse Large B-cell Lymphoma Arising From HHV8 Positive Castleman Disease

Abstract

Diffuse large B-cell lymphoma arising from multicentric Castleman disease is an aggressive disorder with short survival. In 2001 our 46-year old patient presented cervical lymphadenopathy. The third bioptic sample proved HHV8 positive Castleman disease in 2002. Chemoterapy (steroid, cyclophosphamide, CVP, interferon-alpha, thalidomide) and radiotherapy was given with themporary success. In 2009 compression of the respiratory tract needed mantle irradiation, and rapid progression developed, inguinal biopsy revealed DLBCL. R-CHOP was started, but the interim PET/CT showed no respons, thus therapy was changed to R-DHAP, autologous stem cell transplantation was planned. The stem cell collection was made after 2 cycles of chemotherapy. However, further progression could be detected which seemed to be chemoresistant (R-IGEV, R-ICE). Palliative irradiation was performed but serious myelosuppression was developed which led to febrile neutropenia with pneumonia and sepsis. Despite of targeted antibiotic treatment the infection caused our patient's death six months after the diagnosis of DLBCL. We would like to highlite the importance of the repeated biopsies if the behavior of a disease has changed and the rare disorders needed personalised treatment.




doi:10.4021/jh27w