[Diagnosis and management of acquired immune haemolytic anaemia excluding neoplasia. Adequacy with the current guidelines published in 2009]. / Diagnostic et prise en charge de l'anémie hémolytique auto-immune à l'exclusion des formes secondaires à une cause néoplasique. Adéquation de la prise en charge au PNDS octobre 2009.

OBJECTIVES: Describe the management of Acquired Immune Haemolytic Anaemia (AIHA) and correlate with the current guidelines published in 2009. The secondary objective was to calculate the positive predictive value of the Direct Antiglobulin Test (DAT) for the diagnosis of AIHA. METHODS: A retrospective and monocentric study was performed from 2010 to 2015 based on positive DATs, identified in the French Blood Agency database or in medical files. All patients managed for initial diagnosis or relapse of AIHA were included, excluding neoplasia. RESULTS: Six hundred and twenty-three patients had a positive DAT, 42 had non-neoplastic AIHA. Thirty-nine patients were included, 32 had warm antibodies, 5 had a negative DAT and 2 had cold antibodies. No cause was found for 46% (17/37) of the warm antibody and negative DATs AIHAs. Autoimmune disease was found in 11 cases (30%), infection in 4 cases (11%). The etiologic investigations were consistent with the guidelines in 49% of cases. Corticosteroids were first prescribed, as recommended. Second-line treatments were rituximab in 9 cases, splenectomy in 4 cases and azathioprine in 3 cases. The management of cold antibody AIHA complied with the guidelines. The positive predictive value of DATs in hospitalized population was of 14% (85/610). CONCLUSION: AIHA guidelines seem insufficiently applied in our center.

[Infectious events during the course of systemic necrotizing vasculitis: a retrospective study of 82 cases]. / Événements infectieux au cours des vascularites nécrosantes systémiques: étude rétrospective de 82 cas.

PURPOSE: The aim of this study was to assess the infections occurring in a series of 82 patients followed for a systemic necrotizing vasculitis and to determine potential risk factors. METHODS: We studied retrospectively the medical files of 23 Churg and Strauss syndrome, 18 periarteritis nodosa, 14 microscopic polyangiitis, and 27 granulomatosis with polyangiitis, over a 15-year period. Infection delay corresponded to the period from treatment to first infection or between two infections. RESULTS: A total of 61 patients developed 147 infections. Causal agent was identified in 70 cases, 42 were bacterial, 20 viral and 8 fungal. Bronchopneumonia was the most frequent infection (43 %). Sixty-two percent of infections occurred within 2 years after vasculitis diagnosis. Seven infections were major, requiring intensive care, with one infection-death related. Pneumocystis prophylaxis concerned 75 % of patients on cyclophosphamide. Significant factors reducing infection delay were initial hypergammaglobulinemia, hypoalbuminemia, lymphopenia, as well as cyclophosphamide and methotrexate treatment. Large quantities of corticosteroids, cyclophosphamide or azathioprine increased infection delay. This result underlines the early occurrence of infectious complications during vasculitis course. CONCLUSION: Infectious events occurring in systemic necrotizing vasculitis are frequent and occurs early in disease course, and could be prevented with simple prophylactic measures. Vasculitis relapse and infection share similarities and this require permanent clinical vigilance.

[Association of systemic diseases and myelodysplastic syndromes. A retrospective study of 14 cases]. / Association maladies systémiques et syndromes myélodysplasiques. Etude rétrospective portant sur 14 observations.

CONTEXT: The association of a systemic disease (SD) and a myelodysplastic syndrome (MDS) may not be a coincidence. We report 14 cases. METHODS: A retrospective study was conducted in patients presenting with an MDS, hospitalised between 1989 and 1999, in the search for a concomitant systemic disease. RESULTS: Ninety-seven patients, 61 men and 36 women, with a mean age of 74 +/- 11 years suffered from an MDS and 14 of them a concomitant SD: one nodular periateritis, 2 systemic vascularitis, 2 cutaneous vascularitis, 2 atrophic polychondritis, 4 Gougerot-Sjogrën syndrome, 2 systemic lupus and one cutaneous lupus. The systemic disease did not appear to influence survival. CONCLUSION: It is possible that the association is not a coincidence and therefore an MDS should be searched for when confronted with an SD, so that treatment may be adapted appropriately.