TY - JOUR
T1 - The complexity of an overlap type resistant cryoglobulinemia
T2 - a case report and review of the literature
AU - Tocut, Milena
AU - Rozman, Ziv
AU - Biro, Alexander
AU - Winder, Asher
AU - Tanay, Amir
AU - Zandman-Goddard, Gisele
N1 - Publisher Copyright:
© 2019, International League of Associations for Rheumatology (ILAR).
PY - 2019/5/1
Y1 - 2019/5/1
N2 - 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.
AB - 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.
KW - Bortezomib
KW - Cryoglobulinemia type I
KW - Cyclophosphamide (CYC)
KW - Mixed cryoglobulinemia
KW - Monoclonal gammopathy of unclear significance (MGUS)
KW - Plasma exchange
KW - Rituximab (RTX)
KW - Vasculitis
UR - http://www.scopus.com/inward/record.url?scp=85059704424&partnerID=8YFLogxK
U2 - 10.1007/s10067-018-04423-y
DO - 10.1007/s10067-018-04423-y
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C2 - 30628015
AN - SCOPUS:85059704424
VL - 38
SP - 1257
EP - 1262
JO - Clinical Rheumatology
JF - Clinical Rheumatology
SN - 0770-3198
IS - 5
ER -