Fatal hepatic failure associated with graft rejection following reduced-intensity stem-cell transplantation for chronic idiopathic myelofibrosis (CIMF).
A 54-year-old man with chronic idiopathic myelofibrosis (CIMF) underwent RIST. His clinical course had been uneventful until day 60, when splenomegaly reappeared. Hepatic dysfunction developed on day 75. Recipient-type hematopoiesis increased to 51% on day 90. After rapid tapering of cyclosporin, serum levels of AST and ALP normalized in parallel with recovery of complete chimerism on day 134. Yet, jaundice progressed. He died of liver failure on day 176. Postmortem examination revealed neither GVHD nor VOD. Graft rejection following RIST for CIMF may lead to fatal hepatic damage through extramedullary hematopoiesis in the liver or cytokine-mediated immune dysregulations.
Miyakoshi
Shigesaburo
S
Department of Hematology Toranomon Hospital Tokyo Japan.
Kami
Masahiro
M
Kishi
Yukiko
Y
Murashige
Naoko
N
Yuji
Koichiro
K
Kusumi
Eiji
E
Matsumura
Tomoko
T
Onishi
Yasushi
Y
Kobayashi
Kazuhiko
K
Kim
Sung-Won
SW
Hamaki
Tamae
T
Takaue
Yoichi
Y
Taniguchi
Shuichi
S
D002908:Chronic Disease; D017809:Fatal Outcome; D005355:Fibrosis / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D006084:Graft Rejection / Q000150:complications* / Q000473:pathology; D006410:Hematopoiesis; D006801:Humans; D017093:Liver Failure / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D013163:Splenomegaly / Q000150:complications / Q000473:pathology / Q000601:surgery; D033581:Stem Cell Transplantation; D015898:Tomography Scanners, X-Ray Computed