Evans’ Syndrome (ES) is defined as the combination of immune thrombocytopenia (ITP) and autoimmune haemol-ytic anemia (AIHA), in the absence of any known underlying etiology. Pathophysiology, epidemiology and outcome remain unclear. Population. - Thirty-six children (20 male, 16 female), who were diagnosed in the SHIP french centres (Socié té d’hé matologie et d’immunologie pé diatri-que) between 1990 and 2002 with ES, were included in this retrospective study. Results. - Median age at diagnosis was 4 years. In 21 children, ES occurred in the setting of consanguinity, family history of autoimmune/ inflammatory disease, associated autoimmune disorder or immunoregulatory abnormalities (serum imunoglobulins, peripheral blood lymphocytes subsets, low level of the C3- C4 complement components, nuclear antibodies). Several successive treatments were used in this serie (med-ian: 3, range: 0- 10) including corticosteroid therapy (35/36), intravenous immunoglobulins (32/36), immu-nosuppressive agents (14/36), splenectomy (9/36) and anti CD 20 monoclonal antibodies (6/36). Patients with a low level of serum immunoglobulins were more often non-responders to corticosteroid therapy/intravenous immunoglobulins and required more frequently further therapy (P=0.03). Three patients died (intracranial bleeding, N=2, Guillain-Barre syndrome; N=1). Conclusion. - ES was a severe, life-threatening disease, requiring aggressive immunosuppressive therapy in as many as half the patients. Our forthcoming study aims to (i) describe homogeneously-studied and prospectively-analysed cohort of childhood ES, (ii) separate ES from specific immune deficiency (especially fas gene mutations), generalised autoi-mmune/inflammatory disorders and genetic diseases, (iii) identify well-defined ES subsets, (iv) establish prognostic factors and optimal treatment within these subsets.
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