RESUMO
We report the successful HLA-identical sibling bone marrow transplantation (BMT) of a 12-year-old boy with chronic mucocutaneous candidiasis (CMC) syndrome who also had Coomb's positive haemolytic anaemia and recurrent bronchopneumonia with resulting bronchiectasis. Conditioning was with busulfan and cyclophosphamide. Engraftment was slow but complete. Selective red cell haemopoietic failure was encountered soon after transplantation but resolved spontaneously. The patient remains alive 3 years after transplantation and now has normal immune, haemopoeitic and pulmonary functions. No manifestation of CMC has been evident since transplantation.
Assuntos
Transplante de Medula Óssea , Candidíase Mucocutânea Crônica/terapia , Sistema ABO de Grupos Sanguíneos , Anemia Hemolítica Autoimune/complicações , Transplante de Medula Óssea/efeitos adversos , Transplante de Medula Óssea/patologia , Bronquiectasia/complicações , Broncopneumonia/complicações , Candidíase Mucocutânea Crônica/complicações , Candidíase Mucocutânea Crônica/imunologia , Criança , Antígenos HLA , Hematopoese , Humanos , Masculino , Transplante HomólogoRESUMO
Haemophagocytic Lymphohistiocytosis (HLH) is a rare clinical illness with a high mortality. There are reported effective treatment and a favourable outcome if diagnosed early. Five cases of childhood non-familial HLH seen over a 3 year period in our hospital are presented. The diagnosis was not suspected in the referring hospitals even after a bone marrow biopsy examination in two cases. Delay in referral was between 2 weeks to 2 months. A viral trigger was detected in only two cases. There were two deaths. Cause of death in both cases were cytomegalovirus pneumonitis and disseminated intravascular coagulopathy. Respond to treatment was better if started earlier. One case spontaneously resolved. Earlier diagnosis will lead to prompt treatment and a better outcome.