Cryofibrinogenemia is a rare disorder in which plasma, not serum, forms a cryoprecipitate. Patients with
cryofibrinogenemia may be asymptomatic, or they may have painful
ulcers,
purpura,
livedo reticularis,
Raynaud phenomenon,
perniosis of the extremities,
thrombosis, and
arthralgia.
Cryofibrinogenemia may be primary or secondary to an underlying disorder such as
connective tissue disease,
malignancy,
infection, drugs, or thromboembolic disease. Here, we present a 41-year-old woman with a pancreatic
neuroendocrine tumor who underwent a Whipple procedure in 2003 followed by 2
liver transplants for hepatic
metastases. Three years posttransplant, we discovered a biopsy-proven metastatic lesion in her femur. Five years posttransplant, she developed acute, severe
pain in both feet, and was found to have
cryofibrinogenemia despite immunosuppression post-transplant. Testing for
connective tissue diseases and
hematologic malignancy were negative. She was treated with high-dose
prednisone, which completely resolved her symptoms. We also conducted a review of the literature via a PubMed search to summarize the association of
cryofibrinogenemia with
malignancy and treating
cryofibrinogenemia with
corticosteroids. Our study is the first reported case of
cryofibrinogenemia that developed secondary to a
neuroendocrine tumor posttransplant. Our report suggests that
cryofibrinogenemia may occur despite immunosuppression adequate to prevent graft rejection, and that high-dose
corticosteroids are an effective treatment for posttransplant
cryofibrinogenemia.