Possible role of hypercoagulability in calciphylaxis: review of the literature.

Abstract

The role of a hypercoagulable state in the pathogenesis of calciphylaxis has yet to be determined. We sought to find evidence of an association between hypercoagulability and calciphylaxis. We reviewed the primary literature for review articles, studies, and case reports that discussed or demonstrated a possible relationship between calciphylaxis and a hypercoagulable state. Review of the primary literature showed that in cases of calciphylaxis with reported levels of protein C and S, 38% of the patients had decreased protein C levels and 43% had decreased levels of protein S. From review of case reports, 3 cases of improvement of skin lesions with low molecular weight heparin treatment, and a fourth case of healing of skin lesions with tissue plasminogen activator treatment, were found. Calciphylaxis was also found in a patient with antiphospholipid antibody syndrome, and a patient with cryofibrinogenemia had clinical and histologic findings consistent with possible calciphylaxis. A limited number of reports were available for review. Our review of the literature found sufficient evidence to suggest a possible role of a hypercoagulable state in the pathogenesis of calciphylaxis. A prospective study with serial testing of all relevant clotting factors in patients with calciphylaxis is needed to more definitively establish this role.