title: Clinical presentation and outcomes of patients with type 1 monoclonal cryoglobulinemia.
abstract: We describe a series of 102 patients diagnosed from January 1, 1990 to December 31, 2015 with Type 1 monoclonal cryoglobulinemia (MoC). Symptoms were seen in 89 (87%) patients, including: cutaneous symptoms in 64 (63%) patients, with purpura (n = 43, 42%) and ulcers/gangrene (n = 35, 34%) being most common; neurological findings in 33 (32%) patients, most frequently sensory neuropathy (n = 24, 24%); vasomotor symptoms, mainly Raynaud's phenomenon in 25 (25%); arthralgias in 24 (24%); and renal manifestations, primarily glomerulonephritis in 14 (14%) patients. An underlying lymphoproliferative disorder was identified in 94 (92%) subjects; MGUS-39, myeloma-20, lymphoplasmacytic lymphoma-21 and others-14. Treatment was initiated in 73 (72%) patients, primarily for cryoglobulinemia-related symptoms in 57. Treatment regimens consisted of: steroids ± alkylating agents in 29 (40%), novel myeloma therapies in 16 (22%), rituximab with alkylating agents in 12 (16%) and rituximab ± steroids in 11 (15%) patients; 22 patients received plasmapheresis. Six patients underwent autologous stem cell transplant. Cryocrit at treatment initiation, change in cryocrit and time to nadir cryocrit were predictive of symptom improvement. Treatment directed toward the underlying clonal disorder resulted in improvement (n = 47) or stabilization (n = 16) of symptoms in the majority of patients and disappearance of cryoglobulin in over one-half.
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Surbhi
authors.identifier: http://orcid.org/0000-0003-3288-7614
authors.initials: S
authors.lastname: Sidana
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: S Vincent
authors.initials: SV
authors.lastname: Rajkumar
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Angela
authors.initials: A
authors.lastname: Dispenzieri
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Martha Q
authors.initials: MQ
authors.lastname: Lacy
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Morie A
authors.initials: MA
authors.lastname: Gertz
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Francis K
authors.initials: FK
authors.lastname: Buadi
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Suzanne R
authors.initials: SR
authors.lastname: Hayman
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: David
authors.initials: D
authors.lastname: Dingli
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Prashant
authors.initials: P
authors.lastname: Kapoor
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Wilson I
authors.initials: WI
authors.lastname: Gonsalves
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Ronald S
authors.identifier: http://orcid.org/0000-0002-8284-3495
authors.initials: RS
authors.lastname: Go
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Yi Lisa
authors.initials: YL
authors.lastname: Hwa
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.|Division of Nephrology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Nelson
authors.identifier: http://orcid.org/0000-0002-5651-1411
authors.initials: N
authors.lastname: Leung
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Amie L
authors.initials: AL
authors.lastname: Fonder
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Miriam A
authors.initials: MA
authors.lastname: Hobbs
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Steven R
authors.initials: SR
authors.lastname: Zeldenrust
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Stephen J
authors.initials: SJ
authors.lastname: Russell
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: John A
authors.initials: JA
authors.lastname: Lust
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Robert A
authors.initials: RA
authors.lastname: Kyle
authors.affiliation: Division of Hematology, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Shaji K
authors.initials: SK
authors.lastname: Kumar
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001706:Biopsy; D003131:Combined Modality Therapy; D003449:Cryoglobulinemia / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy*; D003450:Cryoglobulins; D003937:Diagnosis, Differential; D005260:Female; D018380:Hematopoietic Stem Cell Transplantation / Q000379:methods; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007075:Immunoglobulin M / Q000097:blood; D053208:Kaplan-Meier Estimate; D008297:Male; D008875:Middle Aged; D010641:Phenotype; D014182:Transplantation, Autologous; D016896:Treatment Outcome
chemical_list: D003450:Cryoglobulins; D007074:Immunoglobulin G; D007075:Immunoglobulin M