Vasculitis associated with large granular lymphocyte (LGL) leukemia: Presentation and treatment outcomes of 11 cases

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• 作者：Alex ; ra Audemard ; Thierry Lamy ; Beno卯t Bareau ; Flore Sicre ; Felipe Suarez ; Florence Truquet ; V茅ronique Salaun ; Magaret Macro ; Laurence Verneuil ; Thierry Lobbedez ; Cindy Castrale ; Jonathan Boutemy ; St茅phane Cheze ; Lo茂k Geffray ; Nicolas Schleinitz ; J茅r么me Rey ; Estibaliz Lazaro ; Lo茂c Guillevin ; Boris Bienvenu
• 关键词：Large granular lymphocyte leukemia ; LGL leukemia ; Vasculitis
• 刊名：Seminars in Arthritis and Rheumatism
• 出版年：December, 2013
• 年：2013
• 卷：43
• 期：3
• 页码：362-366
• 全文大小：198 K

文摘

Objective

The association between vasculitis and large granular lymphocyte (LGL) leukemia has rarely been reported or investigated. Thus, we assessed the clinical and biological phenotypes of LGL leukemia associated with vasculitis.

Results

We studied a series of 11 patients displaying LGL leukemia associated with vasculitis (LAV). The mean age at diagnosis of LGL leukemia was 60.3 years; there were nine women and two men. The mean follow-up period was 45 months. The main LGL lineage was T-LGL (10 patients), and only one NK-LGL was identified. Clinical and biological features of T-LGL leukemia were compared with those from the 2009 French T-LGL registry. We did not find any relevant differences except that patients with LAV were predominantly female (p < 0.05). The most frequently observed vasculitis was cryoglobulinemia (n = 5). Three patients presented with cutaneous leukocytoclastic angiitis, two patients had ANCA-negative microscopic polyangiitis, and one patient had giant cell arteritis. The main clinical features involved the skin, e.g., purpura (91%), arthralgia (37%), peripheral neuritis (27%), and renal glomerulonephritis (18%). The most frequent histologic finding was leucocytoclastic vasculitis (54%). The rate of complete remission was high; i.e., 80%. A minority of patients had a vasculitis relapse (27%). Three patients (27%) died; one death was related to LGL leukemia (acute infection) and the two other deaths were related to vasculitis (both with heart failure).

Conclusion

We conclude that vasculitis is overrepresented in the population of LGL patients, LAV predominantly affects women, vasculitis preferentially affects the small vessels, and LAV has high rate of complete response.