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Cryofibrinogenemia After a Liver Transplant: First Reported Case Posttransplant and a Case-Based Review of the Nontransplant Literature.
MedLine Citation:
PMID:  24679054     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
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 posttransplant. 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.
Authors:
Yuanyuan Chen; Gayatri M Sreenivasan; Kam Shojania; Eric M Yoshida
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Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2014-3-28
Journal Detail:
Title:  Experimental and clinical transplantation : official journal of the Middle East Society for Organ Transplantation     Volume:  -     ISSN:  2146-8427     ISO Abbreviation:  Exp Clin Transplant     Publication Date:  2014 Mar 
Date Detail:
Created Date:  2014-3-31     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101207333     Medline TA:  Exp Clin Transplant     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
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