Outcome of essential cryofibrinogenaemia in a series of 61 patients

Bélizna, C.; Tron, F; Joly, P; Godin, Matthieu; Hamidou, M.; Lévesque, H.

doi:10.1093/rheumatology/kem341

Cited by 34 publications

(21 citation statements)

References 6 publications

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“…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: Discussionsupporting

confidence: 52%

“…6 The prevalence of CF has been estimated to be 3.4% to 13%. 4,[6][7][8] Patients with CF may be asymptomatic. 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.…”

Section: Introductionmentioning

confidence: 99%

“…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%

“…11 Other symptoms include thrombosis (which occurs in 25% to 40% of patients), arthralgia, and glomerulonephritis. 3,[5][6][7] The most specific finding of a skin biopsy is plugging of superficial and deep small-sized and medium-sized blood vessels with thrombi that contain cryofibrinogen. Nonspecific findings of leukocytoclastic vasculitis and necrosis of the dermis and epidermis are commonly observed.…”

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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Section: Discussionsupporting

confidence: 52%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Untitled

Chen

Sreenivasan²,

Shojania³

et al. 2015

Exp Clin Transplant

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“…At high concentrations, cryofibrinogenemia can induce a severe necrotizing vasculopathy. Most studies concerning cryofibrinogenemia excluded patients with concomitant cryoglobulinemia [2][3][4][5][6]. Therefore, the clinical significance of cryofibrinogenemia detection in cryoglobulinemia vasculitis is unknown, except in associated chronic hepatitis C virus (HCV) infection [7].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

Cryofibrinogenemia: A Marker of Severity of Cryoglobulinemic Vasculitis

Michaud¹,

Moulis²,

Puissant³

et al. 2015

The American Journal of Medicine

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Stanozolol treatment for successful prevention of attacks of severe primary cryofibrinogenemia

Ünal

Kara

Özen

et al. 2010

Pediatric Blood & Cancer

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Severe essential cryofibrinogenemia is rare in childhood, and both the diagnosis and the management are challenging for pediatricians. An 11-year-old male, who had already lost two digits following cold exposure, was referred after multiple visits to various hospitals and subsequently diagnosed as primary cryofibrinogenemia. His history revealed unresponsiveness to calcium channel blockers, acetyl salicylic acid, pentoxifylline, dextran, and steroids. Stanozolol (2 mg/day, orally) prophylaxis was initiated and no new skin lesions developed following starting this treatment. Some of the newly formed lesions at the onset of stanozolol healed.

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Outcome of essential cryofibrinogenaemia in a series of 61 patients

Cited by 34 publications

References 6 publications

Untitled

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Cryofibrinogenemia: A Marker of Severity of Cryoglobulinemic Vasculitis

Stanozolol treatment for successful prevention of attacks of severe primary cryofibrinogenemia

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