Cryofibrinogenemia

A rare disorder characterized by the precipitation of protein complexes containing FIBRIN; FIBRINOGEN; and FIBRONECTIN as well as IMMUNOGLOBULINS and plasma proteins in the blood upon exposure to cold temperatures. Patients may experience skin symptoms such as RAYNAUD PHENOMENON or PURPURA, as well as systemic effects including THROMBOSIS; transient NEPHROTIC SYNDROME; or HEMATURIA. OMIM: 123540

Networked: 56 relevant articles (5 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Ulcer
2.	Disseminated Intravascular Coagulation
3.	Cryoglobulinemia
4.	Vasculitis (Vasculitides)
5.	Neoplasms (Cancer)

Experts

1.	Michaud, Martin: 3 articles (08/2022 - 04/2013)
2.	Gaches, Francis: 2 articles (08/2022 - 08/2015)
3.	Huart, Antoine: 2 articles (08/2022 - 08/2015)
4.	Puissant, Bénédicte: 2 articles (08/2022 - 08/2015)
5.	Sailler, Laurent: 2 articles (08/2022 - 08/2015)
6.	Santiago, Mittermayer B: 2 articles (01/2022 - 03/2021)
7.	Pourrat, Jacques: 2 articles (08/2015 - 04/2013)
8.	Callen, Jeffrey P: 2 articles (01/2006 - 11/2004)
9.	Adoue, Daniel: 1 article (08/2022)
10.	Alric, Laurent: 1 article (08/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Cryofibrinogenemia:

1.	Fibrinogen (Factor I)FDA Link 04/01/1991 - "[Cryofibrinogenemia--successful therapy by decreasing fibrinogen]." 09/06/1996 - "[Cryofibrinogenemia caused by monoclonal anti-fibrinogen antibodies]." 01/01/1977 - "Other abnormalities included prolonged prothrombin time, increased fibrinogen-fibrin degradation products, decreased levels of factors V and VIII, cryofibrinogenemia, and microangiopathic hemolytic anemia. " 01/01/2009 - "Cryofibrinogenemia is due to the presence of reversibly cold-precipitating plasma proteins and material, consisting mostly of fibrinogen, fibronectin, and fibrin. " 01/01/1983 - "Hematologic studies revealed platelet dysfunction, cryofibrinogenemia and mild chronic disseminated intravascular coagulation with prolonged PT and PTT, hypofibrinogenemia, a high turnover rate of 125I-fibrinogen and mild elevation of fibrinogen-fibrin degradation products, beta-thromboglobulin and platelet factor 4. A decrease in clot retraction and a marked reduction in maximal amplitude of thrombelastogram were also found. "
2.	Stanozolol (Winstrol)FDA Link 01/01/1993 - "We conclude that stanozolol is a safe and effective treatment of the cutaneous manifestations of cryofibrinogenemia." 07/15/2010 - "Stanozolol treatment for successful prevention of attacks of severe primary cryofibrinogenemia." 01/01/1993 - "Eight patients with cutaneous ulcerations from cryofibrinogenemia were treated with stanozolol. " 09/01/1998 - "Treatment of cryofibrinogenemia may include Stanozolol, plasmapheresis, and fibrinolytics. " 01/01/1993 - "Our purpose was to test the efficacy of stanozolol, a drug capable of fibrinolytic enhancement, in treating cutaneous ulcers caused by cryofibrinogenemia. "
3.	Adrenal Cortex Hormones (Corticosteroids)IBA 06/01/2015 - "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. " 01/01/2007 - "Cryofibrinogenemia associated with Sjögren's syndrome: a case of successful treatment with high-dose corticosteroid." 06/01/2015 - "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. " 12/01/2011 - "Successful treatment of leg ulcer caused by cryofibrinogenemia by using a systemic corticosteroid and warfarin." 08/01/2015 - "Presence of cryofibrinogenemia was associated with use of corticosteroids, immunosuppressants, or plasmapheresis in cryoglobulinemic vasculitis patients (OR 22.7; 95% CI, 2.02-256.44). "
4.	Immunosuppressive Agents (Immunosuppressants)IBA 08/01/2015 - "Presence of cryofibrinogenemia was associated with use of corticosteroids, immunosuppressants, or plasmapheresis in cryoglobulinemic vasculitis patients (OR 22.7; 95% CI, 2.02-256.44). " 12/01/2009 - "Complete remission of cryofibrinogenemia was achieved in 78% of patients receiving antithrombotic agents, steroids, or immunosuppressants, whereas 41.6% of patients experienced a relapse after a median time of 9 months (range 7-42 months). "
5.	ColchicineFDA LinkGeneric 04/01/1982 - "We found a higher incidence of cryofibrinogenemia in subjects with mild to moderately severe disease not being treated with colchicine (six of eight) as compared with colchicine-treated subjects who were in partial or complete clinical remission (four or 16; p less than 0.02). " 01/01/2009 - "To our knowledge, this is the first report documenting use of the combination of colchicine and high-dose pentoxifylline to successfully treat ulcers due to cryofibrinogenemia." 01/01/2009 - "Healing of ulcers due to cryofibrinogenemia with colchicine and high-dose pentoxifylline."
6.	SteroidsIBA 12/01/2009 - "Complete remission of cryofibrinogenemia was achieved in 78% of patients receiving antithrombotic agents, steroids, or immunosuppressants, whereas 41.6% of patients experienced a relapse after a median time of 9 months (range 7-42 months). "
7.	Fibrinolytic Agents (Antithrombotic Agents)IBA 12/01/2009 - "Complete remission of cryofibrinogenemia was achieved in 78% of patients receiving antithrombotic agents, steroids, or immunosuppressants, whereas 41.6% of patients experienced a relapse after a median time of 9 months (range 7-42 months). "
8.	CryoglobulinsIBA 12/01/2009 - "Sixty patients (11.7%) with persistent cryofibrinogenemia and without cryoglobulin were included in the study. " 02/01/2017 - "People with cryofibrinogenemia may be asymptomatic, but this cryoprotein can be associated with thromboembolic disease, particularly affecting the skin. " 04/01/2013 - "Cryofibrinogenemia is a cryoprotein that was first identified in 1955 by Korst and Kratochvil. " 12/01/2009 - "Essential cryofibrinogenemia represents 12% of all the cryoproteins at Pitie-Salpêtriere Hospital. " 12/01/2009 - "Cryofibrinogenemia is an under-recognized cryoprotein that can be life-threatening when untreated. "
9.	Fibrin Fibrinogen Degradation ProductsIBA 01/01/1977 - "Other abnormalities included prolonged prothrombin time, increased fibrinogen-fibrin degradation products, decreased levels of factors V and VIII, cryofibrinogenemia, and microangiopathic hemolytic anemia. " 01/01/1983 - "Hematologic studies revealed platelet dysfunction, cryofibrinogenemia and mild chronic disseminated intravascular coagulation with prolonged PT and PTT, hypofibrinogenemia, a high turnover rate of 125I-fibrinogen and mild elevation of fibrinogen-fibrin degradation products, beta-thromboglobulin and platelet factor 4. A decrease in clot retraction and a marked reduction in maximal amplitude of thrombelastogram were also found. "
10.	Platelet Factor 4IBA 01/01/1983 - "Hematologic studies revealed platelet dysfunction, cryofibrinogenemia and mild chronic disseminated intravascular coagulation with prolonged PT and PTT, hypofibrinogenemia, a high turnover rate of 125I-fibrinogen and mild elevation of fibrinogen-fibrin degradation products, beta-thromboglobulin and platelet factor 4. A decrease in clot retraction and a marked reduction in maximal amplitude of thrombelastogram were also found. "

Therapies and Procedures

1.	Therapeutics 07/01/1996 - "In patients with spontaneous ulceration of the extremities, particularly when they do not respond appropriately to standard therapy, the possibility of cryoglobulinemia or cryofibrinogenemia should be considered." 06/01/1997 - "In the present case, we cannot exclude the possibility that methimazole therapy contributed to the cryofibrinogenemia." 06/01/1997 - "Cryofibrinogenemia developed in an 18-year-old Japanese female patient during methimazole therapy for Graves' disease. " 01/01/2006 - "Warfarin therapy for livedoid vasculopathy associated with cryofibrinogenemia and hyperhomocysteinemia."
2.	Plasmapheresis 04/01/2013 - "Immunosuppressive therapies, plasmapheresis, and/or intravenous fibrinolysis are useful at treating severe forms of cryofibrinogenemia. " 09/01/1998 - "Treatment of cryofibrinogenemia may include Stanozolol, plasmapheresis, and fibrinolytics. " 08/01/2015 - "Presence of cryofibrinogenemia was associated with use of corticosteroids, immunosuppressants, or plasmapheresis in cryoglobulinemic vasculitis patients (OR 22.7; 95% CI, 2.02-256.44). "
3.	Cryosurgery 10/01/1978 - "Cryo corner: a complication of cryosurgery in a patient with cryofibrinogenemia." 12/15/2012 - "Contraindications to cryosurgery include cryofibrinogenemia, cryoglobulinemia, Raynaud disease, agammaglobulinemia, and multiple myeloma. "
4.	Stem Cell Transplantation 12/01/2000 - "Cryofibrinogenemia and skin necrosis in a patient with diffuse large cell lymphoma after high-dose chemotherapy and autologous stem cell transplantation."
5.	Liver Transplantation 06/01/2015 - "Cryofibrinogenemia After a Liver Transplant: First Reported Case Posttransplant and a Case-Based Review of the Nontransplant Literature."