%0 Journal Article %T Long-term outcome of monoclonal (type 1) cryoglobulinemia. %+ Service de médecine interne [Nantes] %+ Service de Médecine interne et immunologie clinique [Rennes] = internal medicine and clinical immunology [Rennes] %+ Laboratoire de Biochimie [Nantes] %+ Service d'Hématologie [Nantes] %+ Service d'hématologie clinique %+ Service de Néphrologie [Nantes] %+ Unité de recherche de l'institut du thorax (ITX-lab) %+ KYCA %+ Laboratoire de Biochimie [Rennes] %A Néel, Antoine %A Perrin, François %A Decaux, Olivier %A Dejoie, Thomas %A Tessoulin, Benoit %A Halliez, Maxime %A Mahé, Béatrice %A Lamy, Thierry %A Fakhouri, Fadi %A Jego, Patrick %A Agard, Christian %A Vigneau, Cécile %A Guenet, Lucienne %A Grosbois, Bernard %A Moreau, Philippe %A Hamidou, Mohamed %Z Groupama Loire Bretagne - Fondation Groupama pour la santé (A.N.) %< avec comité de lecture %@ 0361-8609 %J American Journal of Hematology %I Wiley %V 89 %N 2 %P 156-61 %8 2014-02 %D 2014 %R 10.1002/ajh.23608 %M 24532335 %Z Life Sciences [q-bio]Journal articles %X 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.Am. J. Hematol. 89:156-161, 2014. © 2013 Wiley Periodicals, Inc. %G English %L hal-00966891 %U https://univ-rennes.hal.science/hal-00966891 %~ UNIV-NANTES %~ UNIV-RENNES1 %~ CNRS %~ IFR140 %~ HL %~ IGDR %~ STATS-UR1 %~ IGDR-TR %~ UR1-UFR-SVE %~ UR1-HAL %~ UR1-SDV %~ TEST-UNIV-RENNES %~ TEST-UR-CSS %~ UNIV-RENNES %~ THORAX-UMR %~ UR1-BIO-SA %~ NANTES-UNIVERSITE %~ UNIV-NANTES-AV2022 %~ TEST3-HALCNRS %~ UFR-MEDECINE-NANTES