ABSTRACT
We transplanted 47 patients with Fanconi anemia using an alternative source of hematopoietic cells. The patients were assigned to the following groups: group 1, unrelated bone marrow (N = 15); group 2, unrelated cord blood (N = 17), and group 3, related non-sibling bone marrow (N = 15). Twenty-four patients (51 percent) had complete engraftment, which was not influenced by gender (P = 0.87), age (P = 0.45), dose of cyclophosphamide (P = 0.80), nucleated cell dose infused (P = 0.60), or use of anti-T serotherapy (P = 0.20). Favorable factors for superior engraftment were full HLA compatibility (independent of the source of cells; P = 0.007) and use of a fludarabine-based conditioning regimen (P = 0.046). Unfavorable factors were > or = 25 transfusions pre-transplant (P = 0.011) and degree of HLA disparity (P = 0.007). Intensity of mucositis (P = 0.50) and use of androgen prior to transplant had no influence on survival (P = 0.80). Acute graft-versus-host disease (GVHD) grade II-IV and chronic GVHD were diagnosed in 47 and 23 percent of available patients, respectively, and infections prevailed as the main cause of death, associated or not with GVHD. Eighteen patients are alive, the Kaplan-Meyer overall survival is 38 percent at ~8 years, and the best results were obtained with related non-sibling bone marrow patients. Three recommendations emerged from the present study: fludarabine as part of conditioning, transplant in patients with <25 transfusions and avoidance of HLA disparity. In addition, an extended family search (even when consanguinity is not present) seeking for a related non-sibling donor is highly recommended.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Adolescent , Adult , Fanconi Anemia/therapy , Hematopoietic Stem Cell Transplantation/methods , Transplantation Conditioning/methods , Acute Disease , Chronic Disease , Cyclophosphamide/therapeutic use , Graft vs Host Disease/diagnosis , Graft vs Host Disease/prevention & control , Histocompatibility Testing , HLA Antigens/analysis , Immunosuppressive Agents/therapeutic use , Multivariate Analysis , Retrospective Studies , Severity of Illness Index , Time Factors , Treatment Outcome , Transplantation, Homologous/immunology , Transplantation, Homologous/methodsABSTRACT
A microbiologia das amígdalas continua sendo assunto de muita discussäo. Este trabalho investiga a flora amigdaliana abrangendo-a em praticamente todas as situaçöes patogênicas e também normais. Foram colhidos swabs da superfície de amígdalas de 132 crianças normais (sem AR) em meses quentes (Q> (61 crianças) e frios (F) (71 crianças), de 64 crianças na fase aguda de amigdalite (AA) e de 76 com amigdalite recorrente (com AR, fora da fase aguda). Neste último grupo ainda foi realizado o estudo do core (interior) das amígdalas. Imediatamente após a coleta, o material era introduzido em meio específico e a cultura era realizada no máximo em 8 horas, seguindo padröes da microbiologia clínica. Como resultados, obtivemos uma alta prevalência de Streptococcus viridans nos três grupos. Entre o grupo Q e F foi notado que realmente existem diferenças sazonais, sendo Streptococcus viridans, Neisseria sp e enterobactérias mais freqüentes no grupo F. Comparando-se o grupo com AR e o sem AR vimos que Neisseria sp e enterobactérias säo mais freqüentes no sem AR. No grupo AA, Neisseria sp foi mais freqüente que no grupo normal (sem AR). Este trabalho é importante, pois traz a microbiologia das amígdaIas estudada de forma ampla, melhorando assim a interpretaçäo dos swabs e direcionando a um correto tratamento das afecçöes amigdalianas