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Reactivation of Behçet's disease in the course of multicentric HHV8‐positive Castleman's disease: long‐term complete remission by a combined chemo/radiation and interferon‐α therapy regimen
Author(s) -
Strohal Robert,
Tschachler Erwin,
Breyer Stefan,
Uthman Aumed,
Simonitsch Ingrid,
Tratting Sigfried,
Scheithauer Werner,
Stingl Georg,
Kornek Gabriela V.
Publication year - 1998
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1046/j.1365-2141.1998.01038.x
Subject(s) - medicine , discontinuation , regimen , behcet's disease , gastroenterology , abvd , radiation therapy , disease , immunology , chemotherapy , cyclophosphamide , vincristine
We used a new combined chemo‐ (COP/ABVD), radiation and interferon‐α (10 × 10 6 IU s.c. 3× per week/12 months) therapy regimen to treat severe multicentric Castleman's disease (CD) complicated by relapsing Behçet's disease (BD) manifestations. More than 16 years after diagnosis of CD the patient remains in very good clinical condition, with remission of all CD and BD manifestations 13 months after discontinuation of the interferon‐α treatment. In addition, our clinicopathological, immunohistological and virological data suggest a pathogenetic link between CD and BD via activation of pre‐existing BD‐specific plasma cells due to CD‐related HHV8‐induced overexpression of interleukin‐6.