The complexity of an overlap type resistant cryoglobulinemia: a case report and review of the literature

• Published in: Clinical rheumatology Vol. 38; no. 5; pp. 1257 - 1262
• Main Authors: Tocut, Milena, Rozman, Ziv, Biro, Alexander, Winder, Asher, Tanay, Amir, Zandman-Goddard, Gisele
• Format: Journal Article
• Language: English
• Published: London Springer London 01.05.2019; Springer Nature B.V
• Subjects: Adult; Apheresis; Autoimmune Collection 2018; Autoimmune diseases; Benign monoclonal gammopathy; Biopsy; Blood diseases; Bone marrow; Bortezomib; Case Based Review; Case reports; Cryoglobulinemia; Cryoglobulinemia - complications; Cryoglobulinemia - therapy; Cyclophosphamide; Dialysis; Etiology; Fatal Outcome; Female; Humans; Immunoglobulin kappa-Chains - analysis; Immunotherapy; Intravenous administration; Kidney - pathology; Kidneys; Literature reviews; Lymphocytes B; Malignancy; Medicine; Medicine & Public Health; Monoclonal antibodies; Monoclonal Gammopathy of Undetermined Significance - complications; Monoclonal Gammopathy of Undetermined Significance - therapy; Plasmapheresis; Pre-eclampsia; Renal failure; Rheumatology; Rituximab; Skin - pathology; Targeted cancer therapy; Plasma exchange; Bortezomib; Vasculitis; Cryoglobulinemia type I; Mixed cryoglobulinemia; Monoclonal gammopathy of unclear significance (MGUS); Cyclophosphamide (CYC); Rituximab (RTX)
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-018-04423-y

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.