ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 2639

Long-Term Outcome of Monoclonal (type 1) Cryoglobulinemia

Antoine Néel1, François Perrin1, Olivier Decaux2, Thomas Dejoie3, Maxime Halliez3, Béatrice Mahé4, Thierry Lamy5, Fadi Fakhouri6, Patrick Jego2, Christian Agard1, Cécile Vigneau7, Lucienne Guenet8, Bernard Grosbois9, Philippe Moreau4 and Mohamed Hamidou1, 1Internal Medicine Department, Nantes University Hospital, Nantes, France, 2Department of Internal Medicine, Rennes University Hospital, Rennes, France, 3Biochemistry Laboratory, Nantes University Hospital, Nantes, France, 4Hematology, Nantes University Hospital, Nantes, France, 5Hematology, Rennes University Hospital, Rennes, France, 6Nephrology, Nantes University Hospital, Nantes, France, 7Nephrology, Rennes University Hospital, Rennes, France, 8Biochemistry Laboratory, Rennes University Hospital, Rennes, France, 9Internal Medicine Department, Rennes University Hospital, Rennes, France

Meeting: 2013 ACR/ARHP Annual Meeting

Keywords: , ,

Session Information

Title: Vasculitis III

Session Type: Abstract Submissions (ACR)

Background/Purpose: To investigate long-term outcome of symptomatic type 1 cryoglobulinemia and its determinants.

Methods: Retrospective analysis of a prospective cohort from 2 French university hospitals. Patients with type 1 cryoglobulinemia were identified using laboratory databases. Inclusion criterion was the presence of persistent symptoms of cryoglobulinemia.

Results: 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 lymphoproliferative disorder (LPD) was a gammopathy of unknown significance (MGUS) 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 (CLL) in 1). Severe manifestations affected half the patients and were more frequent with IgG cryoglobulins (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 MGUS, WM or NHL, fludarabine or rituximab-based regimens appeared to yield better therapeutic responses. Five-year actuarial survival rate was 82 %. Older age and hemoglobin level < 12g/dL at diagnosis correlated with a poorer survival (p<0.05, Log-rank test). Nephropathy, infections, Richter’s transformation and second malignancies were important sources of morbi-mortality.

Conclusion: Despite its limitations, this series provide novel information regarding type 1 cryoglobulinemia. Further studies are needed to improve its management. To date, therapeutic strategy should be tailored according to patient’s characteristics (age, comorbidities, underlying LPD), and therapeutic target.

Disclosure:

A. Néel,
None;

F. Perrin,
None;

O. Decaux,
None;

T. Dejoie,
None;

M. Halliez,
None;

B. Mahé,
None;

T. Lamy,
None;

F. Fakhouri,
None;

P. Jego,
None;

C. Agard,
None;

C. Vigneau,
None;

L. Guenet,
None;

B. Grosbois,
None;

P. Moreau,
None;

M. Hamidou,
None.

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