Clinical Significance of Cryofibrinogenemia: Possible Pathophysiological Link with Raynaud’s Phenomenon

Soyfoo, Muhammad Shahnawaz; Goubella, Ahmed; Cogan, Elie; Wautrecht, Jean-Claude; Ocmant, Annick; Stordeur, Patrick

doi:10.3899/jrheum.110793

Cited by 22 publications

(19 citation statements)

References 19 publications

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“…This is consistent with a previous study by Soyfoo and associates, 9 which suggests that treating patients with CF results in progressive normalization of CF.…”

Section: Discussionsupporting

confidence: 82%

“…6,7,9,15 In addition to being the first reported case after a liver transplant, we also believe this is the first reported association between neuroendocrine tumor and CF. A previous study by Belizna and associates 14 recommended a systematic search for lymphoma be performed in patients with an established diagnosis of essential CF, as 27% of patients diagnosed with essential CF are found to have lymphoma in a few years.…”

Section: Discussionmentioning

confidence: 89%

“…The most common symptoms of CF are caused by cutaneous ischemia including ulcerations, purpura, livedo reticularis, ecchymosis, Raynaud phenomenon, perniosis of the extremities, ischemic necrosis, and gangrene. [5][6][7]9,10 Cutaneous lesions typically occur at distal extremities such as the hands, feet, ears, nose, and buttocks, and are often aggravated by exposure to cold. 11 Other symptoms include thrombosis (which occurs in 25% to 40% of patients), arthralgia, and glomerulonephritis.…”

Section: Introductionmentioning

confidence: 99%

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Chen

Sreenivasan²,

Shojania³

et al. 2015

Exp Clin Transplant

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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.

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“…This is consistent with a previous study by Soyfoo and associates, 9 which suggests that treating patients with CF results in progressive normalization of CF.…”

Section: Discussionsupporting

confidence: 82%

Section: Discussionmentioning

confidence: 89%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Untitled

Chen

Sreenivasan²,

Shojania³

et al. 2015

Exp Clin Transplant

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“…However, no study has been done to assess this association. Moreover, it is possible that some RP considered as PRP could be SRP, if future studies confi rm the possible role of cryofi brinogenaemia [15].…”

Section: Associated Conditions In Srpmentioning

confidence: 99%

ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon

Belch

Carlizza

Carpentier

et al. 2017

Vasa

Self Cite

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Abstract. Regarding the clinical diagnosis of Raynaud’s phenomenon and its associated conditions, investigations and treatment are substantial, and yet no international consensus has been published regarding the medical management of patients presenting with this condition. Most knowledge on this topic derives from epidemiological surveys and observational studies; few randomized studies are available, almost all relating to drug treatment, and thus these guidelines were developed as an expert consensus document to aid in the diagnosis and management of Raynaud’s phenomenon. This consensus document starts with a clarification about the definition and terminology of Raynaud’s phenomenon and covers the differential and aetiological diagnoses as well as the symptomatic treatment.

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“…This cryogel, cold-insoluble complex is related with various diseases such as thromboembolic disorder [8], Raynaud's disease [9], rheumatoid arthritis [10], and so on. In a few decades, therapeutic plasmapheresis techniques called "cyrofiltration" have been developed to remove the cryogel from patient's plasma [11].…”

Section: Introductionmentioning

confidence: 99%

Effects of urea and NaCl on the fibrinogen cryogelation

et al. 2017

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Fibrinogen is converted to fibrin monomer and forms a three-dimensional gel network in a step wise manner by the enzymatic action of thrombin (thrombin-induced fibrin gelation). On the other hand, when a fibrinogen solution is incubated at low temperatures (e.g. below 4°C), it is converted to a gel without thrombin. The fibrinogen gel induced by lowering temperature is called "cryogel" and is closely related to some diseases. However, the mechanism of fibrinogen cryogelation has not been understood yet. In this study, the effects of urea and NaCl on fibrinogen cryogelation and thrombin-induced fibrin gelation have been studied turbidimetrically. The addition of urea retarded both gelation, and the extent of retardation increased with increasing the concentration of urea. The effects of urea on fibrinogen cryogelation were much larger than those on thrombin-induced fibrin gelation, and fibrinogen cryogelation was completely inhibited by the addition of 150 mM urea. The addition of NaCl retarded fibrinogen cryogelation with increasing the concentration of NaCl. By contrast, the effects of NaCl on thrombin-induced fibrin gelation were only limited. Based on the experimental results, the contributions of hydrogen and ionic bonds to each gelation are discussed.

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Clinical Significance of Cryofibrinogenemia: Possible Pathophysiological Link with Raynaud’s Phenomenon

Abstract: There seems to be a significant correlation between cryofibrinogen concentration and the severity of the clinical signs, particularly when cryoprecipitate is composed of fibrinogen alone. CF might have a possible pathophysiological role in primary Raynaud's phenomenon.

Cited by 22 publications

References 19 publications

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ESVM guidelines – the diagnosis and management of Raynaud’s phenomenon

Effects of urea and NaCl on the fibrinogen cryogelation

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