Thrombotic microangiopathy following intestinal transplantation: a single center experience.
BACKGROUND
Transplant-related thrombotic microangiopathy (TMA) is a well-recognized complication of all types of transplantations. Despite its known relationship with immunosuppressive therapy, only a few cases have been reported following intestinal transplantation.


METHODS
We retrospectively reviewed the medical files of nine consecutive intestinal transplant patients between 2000 and 2008.


RESULTS
The diagnosis of TMA was established in 3 patients (33%). At diagnosis the immunosuppressive therapy consisted of tacrolimus (n = 3), combined with azathioprine (n = 1) or sirolimus (n = 2) and steroids (n = 2). The median time between transplantation and TMA was 104 days (range, 55-167 days). Levels of ADAMTS13, a von Willebrand protease, were within normal ranges in all 3 patients. Treatment consisted of stopping/tapering of tacrolimus, together with initiation of plasma therapy, leading to complete remission in all 3 patients. During further follow-up, all 3 patients showed severe graft rejection necessitating more profound immunosuppressive therapy, leading to graft loss in 1 patient and infection-related death in the 2 others. At a median follow-up of 52 months (range, 9-100 months) all remaining TMA-free patients (n = 6) were alive with functioning grafts under minimal immunosuppression.


CONCLUSIONS
Herein we have described 3 intestinal transplant patients who were diagnosed with transplantation-related TMA. Despite excellent disease control the final outcomes were dismal, which clearly contrasts with the outcome among TMA-free patients, who were all well with functioning grafts at last follow-up.
Dierickx
D
D
Department of Hematology, University Hospitals Leuven, Leuven, Belgium. daan.dierickx@uzleuven.be
Monbaliu
D
D
De Rycke
A
A
Wisanto
E
E
Lerut
E
E
Devos
T
T
Meers
S
S
Darius
T
T
Ferdinande
P
P
Pirenne
J
J
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D016736:Antiphospholipid Syndrome / Q000175:diagnosis; D001379:Azathioprine / Q000627:therapeutic use; D015267:Churg-Strauss Syndrome / Q000175:diagnosis; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007422:Intestines / Q000637:transplantation*; D008297:Male; D011183:Postoperative Complications / Q000473:pathology; D012189:Retrospective Studies; D020123:Sirolimus / Q000627:therapeutic use; D016559:Tacrolimus / Q000627:therapeutic use; D013927:Thrombosis / Q000175:diagnosis; D057049:Thrombotic Microangiopathies / Q000453:epidemiology*
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents; D001379:Azathioprine; D020123:Sirolimus; D016559:Tacrolimus