| Literature DB >> 30628015 |
Milena Tocut1, Ziv Rozman2, Alexander Biro3, Asher Winder4,5, Amir Tanay5,6, Gisele Zandman-Goddard7,8.
Abstract
Type I cryoglobulinemia is associated with B cell proliferative diseases, whereas essential mixed cryoglobulinemia is classically associated with infections, malignancy, and autoimmune diseases, but may be idiopathic. Prognosis in patients with grave manifestations and renal involvement is often poor. We report a case of a 40-year-old woman, 2 weeks post-partum for pre-eclampsia who was hospitalized with nephritic syndrome and acute renal failure. The patient harbored type I and type II cryoglobulinemia. Renal and cutaneous biopsies confirmed the diagnosis; however, an underlying etiology was not established. A bone marrow biopsy suggested monoclonal gammopathy of undetermined source (MGUS). Despite therapy with intravenous cyclophosphamide, rituximab, plasmapheresis, dialysis, and bortezomib, the patient succumbed after 8 months of hospitalization. We suggest that an overlap entity of types I and II cryoglobulinemia with severe multi-organ involvement not only is rare but also may be resistant to conventional therapy and fatal.Entities:
Keywords: Bortezomib; Cryoglobulinemia type I; Cyclophosphamide (CYC); Mixed cryoglobulinemia; Monoclonal gammopathy of unclear significance (MGUS); Plasma exchange; Rituximab (RTX); Vasculitis
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Year: 2019 PMID: 30628015 DOI: 10.1007/s10067-018-04423-y
Source DB: PubMed Journal: Clin Rheumatol ISSN: 0770-3198 Impact factor: 2.980