Retroperitoneal fibrosis as manifestation of chronic GVHD after allogeneic hematopoietic SCT

摘要

GVHD comprises a spectrum of diseases following hematopoietic SCT (HSCT), caused by the graft recognition of allogeneic Ag on host tissue cells with subsequent inflammation and structural tissue damage. The host target cells are mostly epithelial in nature, with only rare cases of manifestations in mesenchymal tissues. GVHD may, however, mimic any and even rare forms of autoimmune disease. Here we report two cases of retroperitoneal fibrosis (RPF) in allogeneic transplant recipients, in both cases associated with extensive chronic GVHD, suggesting that RPF may be considered as a manifestation of GVHD.The first patient was a 52-year-old man with anaplastic lymphoma kinase-negative anaplastic large T-cell lymphoma, who experienced relapse 14 months after dose-dense CHOEP primary chemotherapy. PBSCT from a matched unrelated donor was performed following conditioning treatment with fludarabine, CY and BU. The post-transplant course was complicated by severe chronic GVHD, with significant manifestations in more than two organs starting 8 months after transplantation. Symptoms included initial maculopapular rash of the whole integument, lichenoid lesions in the buccal mucosa and keratoconjunctivitis sicca of both eyes. Immunosuppressive treatment with systemic prednisolone and CsA with doses adjusted to maintain levels of 200ng/ml_ did not achieve an adequate therapeutic response.