title: Recurrent Mixed Cryoglobulinemia (MCS): A Case Report and Literature Review.
abstract: We report a case of recurrent mixed type II cryoglobulinemia with difficult diagnosis and treatment dilemma and uncertain prognosis in view of limited studies. A 60-year-old male with history of essential mixed cryoglobulinemia 12 years ago treated successfully with six months of cyclophosphamide and prednisone presented with bilateral lower extremity pupuric rash and swelling. He was found to have proteinuria, hematuria, RBC casts, low serum complement levels, and acute kidney injury (AKI). Initial therapy with methylprednisone and oral cyclophosphamide was ineffective (patient developed respiratory failure due to alveolar hemorrhage). Additional labs revealed positive type II cryoglobulins, high free Kappa/Lambda, UPEP with minimal urine protein, SPEP with marked hypogammaglobulinemia, and negative tests for HIV, HCV, ANA, and ANCA. More aggressive therapy with daily plasmapheresis and rituximab was instituted with very good clinical response. He achieved clinical remission but developed another flare 8 months later. Kidney biopsy showed membranoproliferatve glomerulonephritis with cryoglobulin deposits. Flow cytometry and biopsy of bone marrow was consistent with lymphoplasmacytic lymphoma. His diagnosis was eventually confirmed and responded clinically to another course of rituximab and plasmapheresis, but prognosis is yet to be seen.
authors.affiliation: Geriatrics Division, Department of Medicine, Rhode Island Hospital, Alpert Medical School of Brown University, Providence, RI.
authors.forename: Manoj
authors.initials: M
authors.lastname: Bhattarai
authors.affiliation: Division of Hematology and Oncology, Hillman Cancer Center, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Donald V
authors.initials: DV
authors.lastname: Woytowitz
authors.affiliation: Renal-Electrolyte Division, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Hoda
authors.initials: H
authors.lastname: Kaldash
authors.affiliation: Department of Pathology, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Sheldon
authors.initials: S
authors.lastname: Bastacky
authors.affiliation: Renal-Electrolyte Division, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Shan Shan
authors.initials: SS
authors.lastname: Chen
authors.affiliation: Electrolyte Division, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: James R
authors.initials: JR
authors.lastname: Johnston
authors.affiliation: Electrolyte Division, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania.
authors.forename: Jose F
authors.initials: JF
authors.lastname: Bernardo
mesh_terms: D003449:Cryoglobulinemia / Q000175:diagnosis*; D003450:Cryoglobulins / Q000032:analysis; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010956:Plasmapheresis; D012008:Recurrence; D000069283:Rituximab / Q000627:therapeutic use; D008258:Waldenstrom Macroglobulinemia / Q000175:diagnosis* / Q000628:therapy
chemical_list: D003450:Cryoglobulins; D007155:Immunologic Factors; D000069283:Rituximab
keywords: Cryoglobulinemia; Cyclophosphamide; Hepatitis C; Lymphoplasmacytic Lymphoma; Plasmapheresis; Rituximab