title: Vasculitis associated with large granular lymphocyte (LGL) leukemia: presentation and treatment outcomes of 11 cases.
abstract: 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).
  
  
  CONCLUSIONS
  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.
authors: Department of Internal Medicine, CHU Caen, France.
authors: Alexandra
authors: A
authors: Audemard
authors: Thierry
authors: T
authors: Lamy
authors: Benoît
authors: B
authors: Bareau
authors: Flore
authors: F
authors: Sicre
authors: Felipe
authors: F
authors: Suarez
authors: Florence
authors: F
authors: Truquet
authors: Véronique
authors: V
authors: Salaun
authors: Magaret
authors: M
authors: Macro
authors: Laurence
authors: L
authors: Verneuil
authors: Thierry
authors: T
authors: Lobbedez
authors: Cindy
authors: C
authors: Castrale
authors: Jonathan
authors: J
authors: Boutemy
authors: Stéphane
authors: S
authors: Cheze
authors: Loïk
authors: L
authors: Geffray
authors: Nicolas
authors: N
authors: Schleinitz
authors: Jérôme
authors: J
authors: Rey
authors: Estibaliz
authors: E
authors: Lazaro
authors: Loïc
authors: L
authors: Guillevin
authors: Boris
authors: B
authors: Bienvenu
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D054066:Leukemia, Large Granular Lymphocytic / Q000150:complications* / Q000188:drug therapy; D008297:Male; D008875:Middle Aged; D016896:Treatment Outcome; D014657:Vasculitis / Q000150:complications* / Q000188:drug therapy
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents
keywords: LGL leukemia; Large granular lymphocyte leukemia; Vasculitis