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Abstract 5109 Background: Large granular leukemia (LGL) leukemia is derived from either T-cell or NK-cell lineages. Clinical presentation of LGL leukemia is dominated by recurrent infections associated with neutropenia, anemia, splenomegaly and auto-immune diseases. The association of vasculitis and LGL leukemia has been rarely reported and investigated. Objective: To improve knowledge about this association, we retrospectively describe the clinical and biological characteristics of patients, their response to therapy and their prognosis. Methods: We performed a retrospective study based on 229 patients included in the French national LGL proliferation registry and a national mailing survey to 1350 internists in order to collect medical characteristics of patients displaying both vasculitis and LGL leukemia. We retrospectively collected medical charts from 11 patients between September 1999 and February 2012. Medical history, hematologic laboratory tests and response to therapy were compiled at first presentation of LGL leukemia associated vasculitis (LAV) and at each visit. Results: At the time of diagnosis of LGL leukemia the mean age was 60. 3 years (range, 28–74 years). They were 9 women and two men. The mean of follow up was 45 months (range, 1–108). The main LGL lineage was T-LGL (10 patients, 91%) and only one NK-LGL was identified. Hematological laboratory findings revealed that 5 patients (45%) patients presented hyperlymphocytosis. The mean absolute circulating LGL count for the series was 1. 10 9/L (range, 0. 45–4. 59 x. 10 9/L). Three patient (27%) had neutropenia ( Conclusion: Association of LGL leukemia and vasculitis is rare but not fortuitous. Based on this retrospective analysis, it seems that LAV preferentially affect female patients, with a low LGL count. LAV present with frequent cutaneous involvement and have a good response to therapy. Pathophysiological link between LGL leukemia and vasculitis remains to be investigated. Disclosures: No relevant conflicts of interest to declare.

Problematique Fin 2007, 25 cas de LEMP chez des patients traites par rituximab, anticorps monoclonal anti CD20, pour une hemopathie (23 cas) ou un lupus (2 cas) ont ete declares a la Food and Drug Administration (FDA). Le role causal du rituximab est cependant debattu. En effet, tous ces patients avaient au prealable recu une chimiotherapie, et parfois une allogreffe, ou differents immunosuppresseurs. Nous rapportons deux cas de LEMP chez des patients suivis et traites pour une maladie de Wegener mais n’ayant jamais recu de rituximab. Cas cliniques Le 1 er patient a ete traite pour une maladie de Wegener avec atteinte ORL, pulmonaire, renale, ANCA anti-PR3 par corticotherapie, cyclophosphamide, immunoglobulines IV puis azathioprine de 1997 a fin 2005. La LEMP a ete diagnostiquee fin 2005 devant une aphasie de Broca, une apraxie et une IRM cerebrale evocatrice. Ses lymphocytes CD4 etaient a 180/mm 3 . La ponction lombaire ne retrouvait pas le virus JC par PCR, mais la presence du virus a ete confirmee par hybridation in situ sur la biopsie cerebrale stereotaxique. Le 2 e patient a ete traite de 1992 a 1993 pour une maladie de Wegener avec atteinte pulmonaire, ORL, ANCA anti-PR3 par bolus de cyclophosphamide associes a une corticotherapie. A l’issue du 13 e bolus, il a developpe une LEMP diagnostiquee sur l’IRM cerebrale (lymphopenie a 900/mm 3 ). Il est decede 4 mois apres le diagnostic de LEMP. Conclusion Ces deux cas rappellent que la reactivation du virus JC peut etre une complication, qui reste toutefois rare, des traitements immunosuppresseurs classiques. L’imputabilite du rituximab dans la survenue des cas de LEMP rapportes par la FDA reste tres incertaine.

Objective:To characterise major infectious complications and analyse potential risk factors in patients with Wegener granulomatosis (WG).Methods:Data from 113 patients with WG (69 male) followed at least once between January 1984 and March 2006 in our internal medicine department, were analysed retrospectively.Results:A total of 35 patients (mean (SD) age at WG diagnosis: 50.2 (13.05) years) developed 53 major infections. Infections were: bronchopneumonias (n = 19), herpes zoster recurrences (n = 9), cellulitis (n = 4), prostatitis (n = 4), spondylodiscitis and septic arthritis (n = 3), digestive tract infections (n = 2), Enterococcus faecalis or Staphylococcus aureus septicaemia (n = 2), viral hepatitis B reactivations (n = 2), post transfusion HIV infection with fatal cerebral toxoplasmosis, oesophageal candidiasis, disseminated herpes simplex and cytomegalovirus infection, cytomegalovirus retinitis, herpetic keratitis, herpetic stomatitis, Serratia sp. node suppuration and fever resolving under broad spectrum antibiotics (n = 1 each). Half of the major infectious episodes occurred within 3 years after WG diagnosis. Eight (7%) patients died, with two (2%) infection-related deaths. Patients diagnosed with WG before 1996 had a significantly higher rate of infection than those diagnosed later (48% vs 24%, p = 0.02). Cyclophosphamide and corticosteroids were independently associated with significantly higher risk of major infection (pConclusion:Cyclophosphamide and corticosteroids were associated with higher risk of infection. Despite systematic cotrimoxazole prophylaxis, major infections, mostly bronchopneumonias and herpes zoster recurrences, were still common in the course of WG.

The aim of this National Diagnostic and Care Protocol (PNDS) is to explain to the professionals involved the current optimal diagnosis and therapeutic management and care approach for a patient with Takayasu’s arteritis. Its purpose is to optimize and harmonize the management and follow-up of this rare disease throughout the country. It also identifies pharmaceutical specialties used in an indication not provided for in the Marketing Authorization, as well as the specialties, products or services necessary for the care of patients but not usually paid for or reimbursed.