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Cryoglobulinemic Syndromes: Diagnosis and Management

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Biology and Management of Unusual Plasma Cell Dyscrasias

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Abstract

The cryoglobulinemic syndromes are a collection of syndromes in which a cryoglobulin, made up of immunoglobulin and complement component precipitates upon cooling. While the majority of the patients with a cryoglobulin are asymptomatic, unique clinical syndromes may occur often resulting in life-threatening complications. The Brouet classification uses the clonality of the cryoglobulin to define the subtype, and the clinical manifestations vary upon the type of cryoglobulinemia Type I versus mixed cryoglobulinemias (Type II or III). While immediate management is dependent upon controlling the cryoglobulin, the management is in large part contingent upon treating the underlying condition that gave rise to the cryoglobulin. Immediate management of the organ-specific complications is necessary if required, but management of the underlying condition, if identified, is crucial to long-term control. In Type I cryoglobulinemia, treatment of the underlying lymphoproliferative disorder remains the focus of the primary management, while with the mixed cryoglobulinemia, the management is dependent upon treating the infectious or non-infectious etiology. For patients with infectious mixed cryoglobulinemia, the combination of antiviral therapy and rituximab appears to result in improved outcomes while those patients with non-infectious, or essential cryoglobulinemia, the combination of corticosteroids and rituximab should be considered.

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Authors and Affiliations

  1. Section of Hematology/Oncology, The University of Chicago, 55841 S. Maryland Avenue, MC 2115, Chicago, IL, 60637, USA

    Todd M. Zimmerman MD

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  1. Todd M. Zimmerman MD
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Correspondence to Todd M. Zimmerman MD .

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Editors and Affiliations

  1. Section of Hematology/Oncology MC2115, University of Chicago, Chicago, USA

    Todd M. Zimmerman

  2. Division of Hematology, Mayo Clinic, Rochester, Minnesota, USA

    Shaji K. Kumar

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Zimmerman, T.M. (2017). Cryoglobulinemic Syndromes: Diagnosis and Management. In: Zimmerman, T., Kumar, S. (eds) Biology and Management of Unusual Plasma Cell Dyscrasias. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-6848-7_7

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  • DOI: https://doi.org/10.1007/978-1-4419-6848-7_7

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