FAMILIAL RELAPSING THROMBOTIC MICRO-ANGIOPATHY IN 2 SIBLINGS

Two siblings, a 12-year-old girl and a 7-year-old boy, had recurrent e pisodes of haemolyticuraemic syndrome/thrombotic thrombocytopenic purp ura, manifested mainly by thrombocytopenia and micro-angiopathic haemo lytic anaemia. During 11 years of follow up the girl responded only to steroids, whereas many other therapeutic modalities were ineffective. Following treatment with low dose danazol relapses became fewer and o f diminished severity and completely subsided after 6 months. The boy started his illness with signs of haemolytic uraemic syndrome and late r developed neurological manifestations. During a 6 year follow up he responded only to plasma exchange. Although chronic thrombocytopenia p ersisted during the past 3 years, the boy's clinical condition improve d. Conclusion A family with two children with recurrent episodes of th rombotic thrombocytopenic purpura is described, In one child danazol c ould have had a beneficial effect.