Possible role of hypercoagulability in calciphylaxis: review of the literature.
BACKGROUND
The role of a hypercoagulable state in the pathogenesis of calciphylaxis has yet to be determined.


OBJECTIVE
We sought to find evidence of an association between hypercoagulability and calciphylaxis.


METHODS
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.


RESULTS
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.


CONCLUSIONS
A limited number of reports were available for review.


CONCLUSIONS
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.
Harris
Ryan Jeffrey
RJ
School of Medicine, University of Virginia, Charlottesville, VA, USA.
Cropley
Thomas George
TG
D002115:Calciphylaxis / Q000188:drug therapy / Q000209:etiology*; D006495:Heparin, Low-Molecular-Weight / Q000627:therapeutic use; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications; D020151:Protein C Deficiency / Q000150:complications; D018455:Protein S Deficiency / Q000150:complications; D019851:Thrombophilia / Q000150:complications*
D006495:Heparin, Low-Molecular-Weight