Factor XIII replacement in stem-cell transplant recipients with severe hemorrhagic cystitis: a report of four cases.

BACKGROUND: Hemorrhagic cystitis (HC) is an important cause of morbidity in patients undergoing allogeneic stem-cell transplantation (SCT). Various causes have been identified, such as the use of high-dose cyclophosphamide or busulfan and the occurrence of acute graft-versus-host disease or viral infections (cytomegalovirus, adenovirus, polyomavirus).

METHODS: The clinical course of four patients treated with factor XIII (FXIII) concentrate for severe HC after allogeneic SCT is described.

RESULTS: Four patients were treated with one or two infusions of 50 IU/kg of FXIII concentrate. Only one patient showed a plasmatic FXIII decrease before treatment. Three of the four patients responded to this treatment, and HC completely resolved in two of them. No adverse event was observed.

CONCLUSION: The use of FXIII concentrate can improve the major symptoms of HC in patients with decreased or normal FXIII plasma level after allogeneic SCT.