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Long-term Survival, Organ Function, and Malignancy after Hematopoietic Stem Cell Transplantation for Fanconi Anemia

Författare och institution:
C. Bonfim (-); L. Ribeiro (-); S. Nichele (-); M. Bitencourt (-); G. Loth (-); A. Koliski (-); V. A. M. Funke (-); D. V. Pilonetto (-); N. F. Pereira (-); M. E. D. Flowers (-); E. Velleuer (-); R. Dietrich (-); Anders Fasth (Institutionen för kliniska vetenskaper, sektionen för kvinnors och barns hälsa, Avdelningen för pediatrik); C. C. Torres-Pereira (-); P. Pedruzzi (-); M. Eapen (-); R. Pasquini (-)
Publicerad i:
Biology of Blood and Marrow Transplantation, 22 ( 7 ) s. 1257-1263
ISSN:
1083-8791
Publikationstyp:
Artikel, refereegranskad vetenskaplig
Publiceringsår:
2016
Språk:
engelska
Fulltextlänk:
Sammanfattning (abstract):
We report on long-term survival in 157 patients with Fanconi anemia (FA) who survived 2 years or longer after their first transplantation with a median follow-up of 9 years. Marrow failure (80%) was the most common indication for transplantation. There were 20 deaths beyond 2 years after transplantation, with 12 of the deaths occurring beyond 5 years after transplantation. Donor chimerism was available for 149 patients: 112 (76%) reported > 95% chimerism, 27 (18%) reported 90% to 95% chimerism, and 8 (5%) reported 20% to 89% donor chimerism. Two patients have < 20% donor chimerism. The 10- and 15-year probabilities of survival were 90% and 79%, respectively. Results of multivariate analysis showed higher mortality risks for transplantations before 2003 (hazard ratio [HR], 7.87; P =.001), chronic graft-versus-host disease (GVHD) (HR, 3.80; P =.004) and squamous cell carcinoma after transplantation (HR, 38.17; P <.0001). The predominant cause of late mortality was squamous cell carcinoma, with an incidence of 8% and 14% at 10 and 15 years after transplantation, respectively, and was more likely to occur in those with chronic GVHD. Other causes of late mortality included chronic GVHD, infection, graft failure, other cancers, and hemorrhage. Although most patients are disease free and functional long term, our data support aggressive surveillance for long periods to identify those at risk for late mortality.
Ämne (baseras på Högskoleverkets indelning av forskningsämnen):
MEDICIN OCH HÄLSOVETENSKAP ->
Klinisk medicin ->
Pediatrik
Nyckelord:
Fanconi anemia, Survival, Late effects, Allogeneic transplantation, versus-host-disease, bone-marrow-transplantation, human-papillomavirus, failure syndromes, european group, follow-up, head, neck, carcinoma, blood, Hematology, Immunology, Transplantation
Postens nummer:
239466
Posten skapad:
2016-07-25 15:38

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