title: Long-term outcome of monoclonal (type 1) cryoglobulinemia.
abstract: The aim of this study is to investigate long-term outcome of symptomatic type 1 cryoglobulinemia (CG) and its determinants. Retrospective cohort study was conducted in two French University Hospitals. Patients with type 1 CG were identified using laboratory databases. Inclusion criterion was the presence of persistent symptoms of CG. Among 227 screened patients, 36 were included. Skin or vasomotor symptoms were the most frequent features (75%). Nephropathy and neuropathy occurred in 30% and 47% of cases, respectively. The underlying B cell disease (BCD) was a nonmalignant monoclonal gammopathy (NMMG) in 13 (36%) and a hematologic malignancy (HM) in 23 (64%; Waldenstrom macroglobulinemia (WM) in 12, low-grade non-Hodgkin lymphoma (NHL) in 6, multiple myeloma (MM) in 4, and chronic lymphocytic leukemia in 1 patient. Severe manifestations affected half the patients and were more frequent with IgG (82 vs. 30% (P = 0.006)). At last follow-up, 64% of patients had suffered no hematologic manifestation. Potent chemotherapeutic regimens were mainly used in HM. For patients with NMMG, WM, or NHL, fludarabine or rituximab-based regimens appeared to yield better responses. Five-year survival rate was 82%. In multivariate analysis, mortality was significantly higher in older patients (HR: 1.17 per year [95% CI: 1.06-1.28], P = 0.001) and those with nephropathy (HR: 8.9 [95% CI: 1.9-43], P = 0.006). Kidney disease, infections, Richter's transformation, and second malignancies were important sources of morbi-mortality. Despite its limitations, this series provide novel information regarding type 1 CG. Further studies are needed to improve its management. To date, therapeutic strategy should be tailored according to patient's characteristics (age, comorbidities, underlying BCD), and therapeutic target.
authors: CHU Nantes, Service de Médecine Interne, F 44093, Nantes, France.
authors: Antoine
authors: A
authors: Néel
authors: François
authors: F
authors: Perrin
authors: Olivier
authors: O
authors: Decaux
authors: Thomas
authors: T
authors: Dejoie
authors: Benoit
authors: B
authors: Tessoulin
authors: Maxime
authors: M
authors: Halliez
authors: Béatrice
authors: B
authors: Mahé
authors: Thierry
authors: T
authors: Lamy
authors: Fadi
authors: F
authors: Fakhouri
authors: Patrick
authors: P
authors: Jego
authors: Christian
authors: C
authors: Agard
authors: Cécile
authors: C
authors: Vigneau
authors: Lucienne
authors: L
authors: Guenet
authors: Bernard
authors: B
authors: Grosbois
authors: Philippe
authors: P
authors: Moreau
authors: Mohamed
authors: M
authors: Hamidou
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000009:adverse effects / Q000627:therapeutic use; D015331:Cohort Studies; D003449:Cryoglobulinemia / Q000150:complications / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D005260:Female; D005500:Follow-Up Studies; D005602:France; D006801:Humans; D008297:Male; D008875:Middle Aged; D063868:Patient Outcome Assessment; D010951:Plasma Exchange; D016896:Treatment Outcome