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Unusual Ulcers of the Extremities: Cryofibrinogenemia and Cryoglobulinemia

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Uncommon Ulcers of the Extremities

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Abstract

Cryoproteins are blood protein that can be precipitated by cooling and redissolved by warming. We distinguish cryoglobulins that precipitate in serum’s patient and cryofibrinogenemia that precipitate in plasma with cold (Table 9.1). The prevalence of cryoglobulinemia vasculitis has been reported as approximately 10 per million inhabitants [1]. However, the prevalence and incidence of cryoglobulinemia vasculitis is difficult to estimate because of the numerous cause and clinical presentation. Cryofibrinogenemia represents 10% of cryoproteins. Essential cryofibrinogenemia is rare but its prevalence remains unknown. A study in 36,000 hospitalized patients reported a prevalence of 3.4% of cryofibrinogenemia [2].

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References

  1. Gorevic PD, Kassab HJ, Levo Y, Kohn R, Meltzer M, Prose P, et al. Mixed cryoglobulinemia: clinical aspects and long-term follow-up of 40 patients. Am J Med. 1980;69(2):287–308.

    Article  CAS  PubMed  Google Scholar 

  2. Smith SB, Arkin C. Cryofibrinogenemia: incidence, clinical correlations, and a review of the literature. Am J Clin Pathol. 1972;58(5):524–30.

    Article  CAS  PubMed  Google Scholar 

  3. Saadoun D, Elalamy I, Ghillani-Dalbin P, Sene D, Delluc A, Cacoub P. Cryofibrinogenemia: new insights into clinical and pathogenic features. Am J Med. 2009;122(12):1128–35.

    Article  CAS  PubMed  Google Scholar 

  4. Korst DR, Kratochvil CH. Cryofibrinogen in a case of lung neoplasm associated with thrombophlebitis migrans. Blood. 1955;10(9):945–53.

    Article  CAS  PubMed  Google Scholar 

  5. Stathakis NE, Karamanolis D, Koukoulis G, Tsianos E. Characterization of cryofibrinogen isolated from patients plasma. Haemostasis. 1981;10(4):195–202.

    CAS  PubMed  Google Scholar 

  6. Amdo TD, Welker JA. An approach to the diagnosis and treatment of cryofibrinogenemia. Am J Med. 2004;116(5):332–7.

    Article  PubMed  Google Scholar 

  7. Brouet JC, Clauvel JP, Danon F, Klein M, Seligmann M. Biologic and clinical significance of cryoglobulins. A report of 86 cases. Am J Med. 1974;57(5):775–88.

    Article  CAS  PubMed  Google Scholar 

  8. Blain H, Cacoub P, Musset L, Costedoat-Chalumeau N, Silberstein C, Chosidow O, et al. Cryofibrinogenaemia: a study of 49 patients. Clin Exp Immunol. 2000;120(2):253–60.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Kalbfleisch JM, Bird RM. Cryofibrinogenemia. N Engl J Med. 1960;263:881–6.

    Article  CAS  PubMed  Google Scholar 

  10. Belizna CC, Tron F, Joly P, Godin M, Hamidou M, Lévesque H. Outcome of essential cryofibrinogenaemia in a series of 61 patients. Rheumatology (Oxford). 2008;47(2):205–7.

    Article  CAS  PubMed  Google Scholar 

  11. Rachmilewitz EA, Sacks MI, Zlotnick A. Essential cryofibrinogenemia. Clinical, pathological and immunological studies. Isr J Med Sci. 1970;6(1):32–43.

    CAS  PubMed  Google Scholar 

  12. Euler HH, Zeuner RA, Béress R, Gutschmidt HJ, Christophers E, Schroeder JO. Monoclonal cryo-antifibrinogenemia. Arthritis Rheum. 1996;39(6):1066–9.

    Article  CAS  PubMed  Google Scholar 

  13. Copeman PW. Cryofibrinogenaemia and skin ulcers: treatment with plasmapheresis. Br J Dermatol. 1979;101(Suppl 17):57–8.

    PubMed  Google Scholar 

  14. Terrier B, Krastinova E, Marie I, Launay D, Lacraz A, Belenotti P, et al. Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey. Blood. 2012;119(25):5996–6004.

    Article  CAS  PubMed  Google Scholar 

  15. Terrier B, Karras A, Kahn J-E, Le Guenno G, Marie I, Benarous L, et al. The Spectrum of type I Cryoglobulinemia vasculitis: new insights based on 64 cases. Medicine. 2013;92(2):61–8.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Fermand J-P, Bridoux F, Kyle RA, Kastritis E, Weiss BM, Cook MA, et al. How I treat monoclonal gammopathy of renal significance (MGRS). Blood. 2013;122(22):3583–90.

    Article  CAS  PubMed  Google Scholar 

  17. Harel S, Mohr M, Jahn I, Aucouturier F, Galicier L, Asli B, et al. Clinico-biological characteristics and treatment of type I monoclonal cryoglobulinaemia: a study of 64 cases. Br J Haematol. 2015;168(5):671–8.

    Article  CAS  PubMed  Google Scholar 

  18. Sidana S, Rajkumar SV, Dispenzieri A, Lacy MQ, Gertz MA, Buadi FK, et al. Clinical presentation and outcomes of patients with type 1 monoclonal cryoglobulinemia. Am J Hematol. 2017;92(7):668–73.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  19. Cacoub P, Desbois AC, Comarmond C, Saadoun D. Impact of sustained virological response on the extrahepatic manifestations of chronic hepatitis C: a meta-analysis. Gut. 2018;67(11):2025–34.

    Article  CAS  PubMed  Google Scholar 

  20. Cacoub P, Vautier M, Desbois AC, Lafuma A, Saadoun D. Effectiveness and cost of hepatitis C virus cryoglobulinaemia vasculitis treatment: from interferon-based to direct-acting antivirals era. Liver Int. 2017;37(12):1805–13.

    Article  CAS  PubMed  Google Scholar 

  21. Cacoub P, Delluc A, Saadoun D, Landau DA, Sene D. Anti-CD20 monoclonal antibody (rituximab) treatment for cryoglobulinemic vasculitis: where do we stand? Ann Rheum Dis. 2008;67(3):283–7.

    Article  CAS  PubMed  Google Scholar 

  22. Saadoun D, Resche Rigon M, Sene D, Terrier B, Karras A, Perard L, et al. Rituximab plus peg-interferon-alpha/ribavirin compared with peg-interferon-alpha/ribavirin in hepatitis C-related mixed cryoglobulinemia. Blood. 2010;116(3):326–34; quiz 504–5.

    Article  CAS  PubMed  Google Scholar 

  23. Terrier B, Marie I, Launay D, Lacraz A, Belenotti P, de Saint-Martin L, et al. Predictors of early relapse in patients with non-infectious mixed cryoglobulinemia vasculitis: results from the French nationwide CryoVas survey. Autoimmun Rev. 2014;13(6):630–4.

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Internal Medicine and Clinical Immunology, Sorbonne Universités, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France

    M. Vautier & D. Saadoun

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  1. M. Vautier
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  2. D. Saadoun
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Correspondence to D. Saadoun .

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Editors and Affiliations

  1. Institute of Medical Sciences, Department of Surgery, Banaras Hindu University, Varanasi, India

    Ajay K. Khanna

  2. Institute of Medical Sciences, Department of General Surgery, Banaras Hindu University, Varanasi, India

    Satyendra Kumar Tiwary

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© 2023 The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd.

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Vautier, M., Saadoun, D. (2023). Unusual Ulcers of the Extremities: Cryofibrinogenemia and Cryoglobulinemia. In: Khanna, A.K., Tiwary, S.K. (eds) Uncommon Ulcers of the Extremities. Springer, Singapore. https://doi.org/10.1007/978-981-99-1782-2_9

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  • DOI: https://doi.org/10.1007/978-981-99-1782-2_9

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  • Online ISBN: 978-981-99-1782-2

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