Cryofibrinogenemia is an uncommon cause of intravascular coagulation necrosis of the skin and occurs as a result of vascular occlusion from cryoproteins, which reversibly precipitate in cold temperatures. The disease is associated with various conditions, most commonly neoplastic and thromboembolic diseases, and produces cutaneous manifestations such as purpura, ecchymoses, gangrene, and ulcerations. Diagnosis is based on clinical cutaneous manifestations, histopathology, and the laboratory detection of cryofibrinogen precipitation. Treatment is based upon resolution of the underlying disease process or condition, although some interventions have been reported to have therapeutic efficacy. We discuss the presentation, diagnosis, and treatment of a case of cryofibrinogenemia in a patient with underlying B-cell lymphoma.
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Cryoglobulinemic Vasculitis in Disguise: Cryofibrinogenemia as Variant of Monoclonal Gammopathy of Renal Significance.
Am J Kidney Dis
March 2024
Internal Medicine/Nephrology, Meander Medical Centre, Amersfoort, The Netherlands.
Article Synopsis
- A 39-year-old woman diagnosed with relapsing nephrotic syndrome was found to have cryofibrinogen and a monoclonal immunoglobulin (M-protein) in her blood, indicating monoclonal gammopathy related to kidney issues.
- Initial immunosuppressive treatment led to temporary improvement, but after her condition relapsed, she underwent more intensive therapy, including stem cell transplantation, which again provided temporary relief.
- Subsequent experiments revealed that the cryoactivity was dependent on the M-protein, suggesting that cryofibrinogenemia is linked to this protein and should be associated with monoclonal gammopathy of renal significance.
Monoclonal Gammopathy of Thrombotic Significance.
Cancers (Basel)
January 2023
Department of Clinical Therapeutics, School of Medicine, National and Kapodistrian University of Athens, 15772 Athens, Greece.
The current review provides an overview of the thrombotic risk observed in patients with MG who do not otherwise require treatment. We discuss clinical and biomarker studies that highlight the heterogenous hemostatic profile observed in these patients and how knowledge has evolved over the past 20 years. Biomarker studies suggest shared biologic features between multiple myeloma and monoclonal gammopathy of undetermined significance (MGUS), which involves both hypercoagulability and platelet activation.
View Article and Find Full Text PDFAnalysis of cryoproteins with a focus on cryofibrinogen: a study on 103 patients.
Clin Chem Lab Med
October 2022
Chair of Rheumatology - Department of Maternal, Child and Adult Medical and Surgical Sciences, Università degli Studi di Modena e Reggio Emilia, Modena, Italy.
Article Synopsis
- The study focuses on the objectives and methodology for testing Cryofibrinogen (CF), a protein that behaves abnormally at different temperatures, highlighting its relevance in clinical diagnostics, especially in rheumatology.
- A retrospective analysis from 2019 to 2021 indicates that most CF test requests came from the rheumatology department, with a significant number of patients testing positive for CF, while isolated CF cases were notably high among those with systemic sclerosis.
- The findings suggest that the established testing protocols can help create standardized recommendations for CF analysis, emphasizing the need to include CF testing in assessments for conditions that may lead to cryofibrinogenemia.
Clinical impact and prognosis of cryoglobulinemia and cryofibrinogenemia in systemic sclerosis.
Autoimmun Rev
August 2022
Centre Hospitalier Universitaire, Medecine Interne, Toulouse, France; Centre Hospitalier Universitaire, Laboratoire d'Immunologie, Toulouse, France; Clinique Saint-Exupery, Medecine Interne, Toulouse, France; Centre Hospitalier Universitaire, Néphrologie, Toulouse, France; Centre Hospitalier Universitaire, Cardiologie, Toulouse, France; Clinique Ambroise-Paré, Medecine Interne, Toulouse, France; Centre Hospitalier Universitaire, Pneumologie, Toulouse, France; Hopital Joseph Ducuing, Medecine Interne, Toulouse, France; Centre D'investigation Clinique (CIC), 1436 PEPSS Team, Toulouse, France.
Introduction: An association of systemic sclerosis (SSc) with cryoglobulin and/or cryofibrinogenemia has been described. However, clinical, biological, morphological and prognostic implications are unknown. The objective of this study was to describe the phenotype and evaluate the prognosis of cryoglobulinemia and/or cryofibrinogenemia in the progression of SSc.
View Article and Find Full Text PDFHigh prevalence of cryofibrinogenemia in patients with chilblains during the COVID-19 outbreak.
Int J Dermatol
December 2020
Division of Dermatology, Hospital Universitario Marqués de Valdecilla, University of Cantabria, IDIVAL, Santander, Spain.
Background: Many cutaneous manifestations have been described in possible association with the COVID-19 pandemic, including acral lesions resembling chilblains. The underlying pathomechanisms of COVID-19 chilblains are not fully understood. The aim of this study was to describe the clinical, pathological, and laboratory findings of a series of patients who developed chilblains during the COVID-19 outbreak and to investigate the possible factors that could be involved in the pathogenesis of these lesions.
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