Calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K

Douglas L. Riegert-Johnson, Judith S. Kaur, Eric A. Pfeifer

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39 Scopus citations


Calciphylaxis is a rare disorder of small-vessel calcification and cutaneous infarction associated with chronic renal failure. Rare cases of calciphylaxis not associated with chronic renal failure have been reported with breast cancer, hyperparathyroidism, and alcoholic cirrhosis. To our knowledge, we report the first case of calciphylaxis without chronic renal failure associated with cholangiocarcinoma and the first attempt to treat calciphylaxis with vitamin K. A 56-year-old woman presented with necrotic leg ulceration. She was treated initially with low-molecular-weight heparin, with no effect. A coagulation work-up showed vitamin K deficiency. During vitamin K therapy, the patient had fulminant progression of the calciphylaxis. She died, and an autopsy showed metastatic cholangiocarcinoma. Thrombosis and protein C deficiency have been implicated in the pathophysiology of calciphylaxis. Functional protein C deficiency may be one of several factors contributing to the development of calciphylaxis. Vitamin K therapy was ineffective in our patient and may have been detrimental.

Original languageEnglish (US)
Article number65007
Pages (from-to)749-752
Number of pages4
JournalMayo Clinic proceedings
Issue number7
StatePublished - 2001

ASJC Scopus subject areas

  • General Medicine


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