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

  • Milena Tocut
  • Ziv Rozman
  • Alexander Biro
  • Asher Winder
  • Amir Tanay
  • Gisele Zandman-GoddardEmail author
Case Based Review
  • 78 Downloads
Part of the following topical collections:
  1. Autoimmune Collection 2018

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.

Keywords

Bortezomib Cryoglobulinemia type I Cyclophosphamide (CYC) Mixed cryoglobulinemia Monoclonal gammopathy of unclear significance (MGUS) Plasma exchange Rituximab (RTX) Vasculitis 

Notes

Compliance with ethical standards

Disclosures

None.

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Copyright information

© International League of Associations for Rheumatology (ILAR) 2019

Authors and Affiliations

  1. 1.Department of Internal Medicine CWolfson Medical CenterHolonIsrael
  2. 2.Department of Intensive CareWolfson Medical CenterHolonIsrael
  3. 3.Department of NephrologyWolfson Medical CenterHolonIsrael
  4. 4.Department of HematologyWolfson Medical CenterHolonIsrael
  5. 5.Sackler Faculty of MedicineTel-Aviv UniversityTel AvivIsrael
  6. 6.Department of RheumatologyWolfson Medical CenterHolonIsrael

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