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Titolo:
Bone marrow transplantation from matched siblings in patients with Fanconianemia utilizing low-dose cyclophosphamide, thoracoabdominal radiation andantithymocyte globulin
Autore:
Ayas, M; Solh, H; Mustafa, MM; Al-Mahr, M; Al-Fawaz, I; Al-Jefri, A; Shalaby, L; Al-Nasser, A; Al-Sedairy, R;
Indirizzi:
King Faisal Specialist Hosp & Res Ctr, Dept Oncol, Riyadh 11211, Saudi Arabia King Faisal Specialist Hosp & Res Ctr Riyadh Saudi Arabia 11211 i Arabia
Titolo Testata:
BONE MARROW TRANSPLANTATION
fascicolo: 2, volume: 27, anno: 2001,
pagine: 139 - 143
SICI:
0268-3369(200101)27:2<139:BMTFMS>2.0.ZU;2-O
Fonte:
ISI
Lingua:
ENG
Soggetto:
SEVERE APLASTIC-ANEMIA; TERM FOLLOW-UP; CONTROLLED TRIAL; THERAPY; IRRADIATION; EXPERIENCE; REGISTRY; SEATTLE; DISEASE; DONOR;
Keywords:
bone marrow transplantation; Fanconi anemia; matched donors; antithymocyte globulins;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
31
Recensione:
Indirizzi per estratti:
Indirizzo: Ayas, M King Faisal Specialist Hosp & Res Ctr, Dept Oncol, MBC 64,POB 3354, Riyadh11211, Saudi Arabia King Faisal Specialist Hosp & Res Ctr MBC 64,POB 3354 Riyadh Saudi Arabia 11211
Citazione:
M. Ayas et al., "Bone marrow transplantation from matched siblings in patients with Fanconianemia utilizing low-dose cyclophosphamide, thoracoabdominal radiation andantithymocyte globulin", BONE MAR TR, 27(2), 2001, pp. 139-143

Abstract

Nineteen patients with Fanconi anemia (FA) and bone marrow failure underwent bone marrow transplantation (BMT) from matched siblings. Median age at BMT was 8.7 years, Conditioning consisted of low-dose cyclophosphamide (CY 5mg/kg x 4 days) and thoracoabdominal irradiation (TAI 400 cGy), Graft-versus-host disease (GVHD) prophylaxis was cyclosporin A (CsA) in 13 patients and CsA plus methotrexate in 6 patients. Antithymocyte globulin (ATG) was added in the pretransplant as well as the post-transplant period. All patients received high-dose acyclovir from day 2 pre-BMT to day 28 post BMT, and intravenous immunoglobulins (IVIG), 500 mg/kg weekly from day 7 pre-BMT to day 90 post BMT, No fungal prophylaxis was given. All patients engrafted, (median, 14 days for an absolute neutrophil count greater than or equal to0.5x 10(9)/l; median, 37 days for platelet count greater than or equal to 20 x 10(9)/1), Fourteen (74%) patients are alive with sustained engraftment and are transfusion independent. Three (16.6%) patients developed acute GVHD;none developed chronic GVHD, Five (26%) patients developed invasive fungalinfections, and two (10%) developed fatal CMV disease. We believe the addition of ATG may have contributed to the increased incidence of severe life-threatening fungal and viral infections in our series.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 04/04/20 alle ore 14:08:21