Fatal hemophagocytic syndrome after living-related liver transplantation: a report of two cases
Chisuwa, H.; Hashikura, Y.; Nakazawa, Y.; Kamijo, T.; Nakazawa, K.; Nakayama, J.; Oh-Ishi, T.; Ikegami, T.; Terada, M.; Kawasaki, S.
Transplantation 72(11): 1843-1846
2001
ISSN/ISBN: 0041-1337 PMID: 11740400 Document Number: 526337
Background: Hemophagocytic syndrome (HPS) is a serious hematological disorder caused by activated T lymphocytes in immunologically compromised patients. There is no report of HPS in liver transplant recipients. Methods: Among 135 patients who underwent living-related liver transplantation between June 1990 and October 2000, HPS developed in two pediatric patients (1.5%) on the 15th and 134th postoperative day, respectively. The courses of these patients were evaluated. Results: The cause of HPS was unknown in patient 1 and suspected to be Epstein-Barr virus infection in patient 2. The course of patient 2 was also complicated by posttransplant lymphoproliferative disorder. Both patients had high fever, pancytopenia, coagulopathy, and marked elevation of serum-soluble interleukin 2 receptor, serum ferritin, and urine beta-microglobulin levels. The diagnosis was established based on clinical findings, laboratory data, and bone marrow biopsy. Both patients died in an acute course despite intensive care. Conclusions: HPS should be recognized as a severe hematological complication in liver transplant patients. Prompt institution of adequate treatment is necessary to prevent fatality.