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Titolo:
UNUSUAL COMPLICATIONS AFTER BONE-MARROW TRANSPLANTATION FOR DYSKERATOSIS-CONGENITA
Autore:
ROCHA V; DEVERGIE A; SOCIE G; RIBAUD P; ESPEROU H; PARQUET N; GLUCKMAN E;
Indirizzi:
HOP ST LOUIS,BONE MARROW TRANSPLANT UNIT,UNITE RECH CLIN,PAVILLON LALLIER,1 AV CLAUDE VELLEFAUX PARIS FRANCE
Titolo Testata:
British Journal of Haematology
fascicolo: 1, volume: 103, anno: 1998,
pagine: 243 - 248
SICI:
0007-1048(1998)103:1<243:UCABTF>2.0.ZU;2-1
Fonte:
ISI
Lingua:
ENG
Soggetto:
COLONY-STIMULATING FACTOR; APLASTIC-ANEMIA; HOST DISEASE; NEUTROPENIA;
Keywords:
DYSKERATOSIS CONGENITA; BONE MARROW TRANSPLANTATION; ENDOTHELIAL CELL DAMAGE SYNDROME; PULMONARY FIBROSIS; EVANS SYNDROME;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
26
Recensione:
Indirizzi per estratti:
Citazione:
V. Rocha et al., "UNUSUAL COMPLICATIONS AFTER BONE-MARROW TRANSPLANTATION FOR DYSKERATOSIS-CONGENITA", British Journal of Haematology, 103(1), 1998, pp. 243-248

Abstract

Dyskeratosis congenita (DC) is a rare inherited disorder often associated with aplastic anaemia, We report the cases of five boys transplanted with an HLA-identical related donor for severe aplastic anaemia (SAA) associated to DC; in all cases successful engraftment was observed. Three patients died 2-8 years after bone marrow transplantation (BMT) with signs of endothelial cell damage syndrome (kidney microangiopathy and liver veno-occlusive disease). Another boy died 1 year after BMT from Evans syndrome and invasive aspergillosis, One boy currently presents anaemia, polyarthritis of unknown origin, pulmonary fibrosis and gut malabsorption 7.5 years after BMT. SAA associated with DC can besuccessfully treated by allogeneic BMT. However, these early and latecomplications observed are very unusual after BMT and probably reflect the association of transplanted-related factors, evolution of the underlying disease, and increased sensitivity of endothelial cells. Modified conditioning approaches, advances in supportive care and surveillance of these unusual complications offer the possibility of improved outcome for these patients.

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Documento generato il 07/07/20 alle ore 11:47:54