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Fatal Hemorrhagic Gastrointestinal Angioectasia after Bone Marrow Transplantation for Dyskeratosis Congenita.

Abstract
Dyskeratosis congenita (DC) is a rare inherited disease in which the telomere complex cannot be maintained. Shortened telomeres can cause a number of clinical conditions. We herein report a case of unrelated bone marrow transplantation due to aplastic anemia associated with DC. The patient died of uncontrollable refractory intestinal bleeding. Three cases of DC with life-threatening hemorrhaging after transplantation have been reported; however, the bleeding origin could not be determined. Our case is the only patient in which a gastrointestinal bleeding point, jejunal multiple angioectasia, was determined.
AuthorsJin Imai, Takayoshi Suzuki, Marie Yoshikawa, Makiko Dekiden, Hirohiko Nakae, Fumio Nakahara, Shingo Tsuda, Hajime Mizukami, Jun Koike, Muneki Igarashi, Hiromasa Yabe, Tetsuya Mine
JournalInternal medicine (Tokyo, Japan) (Intern Med) 2016 Vol. 55 Issue 23 Pg. 3441-3444 ISSN: 1349-7235 [Electronic] Japan
PMID27904106 (Publication Type: Case Reports, Journal Article)
Topics
  • Anemia, Aplastic (etiology, therapy)
  • Bone Marrow Transplantation (adverse effects)
  • Dilatation, Pathologic (etiology)
  • Dyskeratosis Congenita (complications)
  • Female
  • Gastrointestinal Hemorrhage (etiology)
  • Humans
  • Young Adult

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