Successful management of secondary cryofibrinogenaemia using bosentan therapy.

The authors report the first case of cryofibrinogenaemia secondary to cyclophoshamide, thalidomide, and dexamethasone refractory to conventional treatment in a 76-year-old white female, but markedly responsive to vasodilator therapy with bosentan. The patient's phenotype had elements of a systemic sclerosis overlap syndrome. Examination for active autoimmune, inflammatory, and hypercoagulable conditions was negative. The most sensitive techniques have yielded 3 percent in healthy patients.