Telangiectasia hemorrágica hereditária: melhoria dramática após terapêutica com talidomida

EnglishHereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disease that is characterized by mucocutaneous and visceral telangiectasia, involving several organs with vascular malformations. We present a patient with HHT and severe iron deficiency anemia, requiring blood transfusions every 3 weeks, despite treatment with intravenous iron. There was no satisfactory haematological response after hormone therapy with estradiol, so we started treatment with thalidomide 200 mg / day, with dramatic improvement, with no need for transfusions, even after dose reduction to 100 mg / day. Despite the long treatment with thalidomide, side effects did not occurred portuguesA telangiectasia hemorragica hereditaria (THH) e uma doenca autossomica dominante, caracterizada por telangiectasias mucocutâneas e viscerais, envolvendo varios orgaos com malformacoes vasculares. Apresentamos o caso de um paciente com THH e anemia grave por deficiencia de ferro, dependente de transfusoes de sangue a cada 3 semanas, apesar da terapeutica com ferro endovenoso. Iniciou terapeutica hormonal com estradiol, sem resposta hematologica satisfatoria, pelo que comecou tratamento com talidomida 200 mg/dia, com melhoria evidente, sem necessidade de transfusoes adicionais, mesmo apos reducao da dose para 100 mg/dia. Apesar do tratamento prolongado, nao acorreram efeitos colaterais.