Symptomatic cryoglobulinemia.

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Curr Treat Options Oncol 2000 Jun;1(2):105-18

Symptomatic cryoglobulinemia.

Dispenzieri A.

Mayo Clinic, Division of Hematology, 200 First Street SW, Rochester, MN 55905,

USA.

Treatment of symptomatic cryoglobulinemia is exceedingly challenging due to the

multisystemic nature of its presentation, the variability of its course, and the

paucity of effective therapeutic options supported by randomized controlled

clinical trials. Patients with mild, relatively asymptomatic disease should be

observed without introduction of systemic therapy. Patients with symptomatic

type I secondary cryoglobulinemia should be treated as appropriate for their

underlying lymphoproliferative or plasmaproliferative disorder. Patients with

secondary type II or type III cryoglobulinemia should be treated for their

underlying connective tissue, lymphoproliferative, or liver disorder or

infection. First line treatment for symptomatic, essential type II or type III

cryoglobulinemia associated with hepatitis C should include interferon

(IFN)-alpha. Depending on the severity of clinical presentation, adjuvant

corticosteroids or plasmapheresis should be considered. Life-threatening or

acute organ-threatening presentations should be managed with combined modality

therapy, which should include high-dose corticosteroid, plasmapheresis, or

alkylator-based therapy. Many chemotherapeutic and immunosuppressive strategies

have been tried but not systematically tested; their value is therefore unknown.

Challenges for the future of treating symptomatic cryoglobulinemia include

devising better therapies and more systematic evaluation of existing therapies.

PMID: 12057048 [PubMed - in process]