Severe, refractory type II essential mixed cryoglobulinemia treated with 2-chlorodeoxyadenosine and mycophenolate mofetil

Excerpt

Mediated by cold-precipitating immunoglobulins, necrotizing vasculitis can result in multiple organ system dysfunction and failure. Classified into three subtypes in 1974 by Brouet et al. [1] these cryoglobulins are associated with malignancy, autoimmunity, and viral infection. Eight years earlier, Meltzer et al. [2] described essential mixed cryoglobulinemia to denote autonomous disease. The mixed cryoglobulinemia syndrome characterized by purpura, weakness, and arthralgias, may result pathologically in chronic hepatitis, membranoproliferative glomerulonephritis, peripheral neuropathy, and skin ulceration. Initial interventions for symptomatic essential mixed cryoglobulinemia are immunosuppression with steroids and cyclophosphamide with or without plasmapheresis/plasma exchange [3]. …