[Diagnostic performance of κ/λ serum free light chain ratio (Freelite® assay) and IgGκ/IgGλ ratio (Hevylite® assay) as prognostic biomarkers of the evolution of immune thrombocytopenia into a chronic disease in adult patients]. / Performance diagnostique des rapports κ/λ des chaines légères libres sériques (test Freelite®) et IgGκ/IgGλ (test Hevylite®) comme marqueurs pronostiques de chronicisation du purpura thrombopénique immunologique de l'adulte.

INTRODUCTION: Immune thrombocytopenia (ITP) is an acquired hemorrhagic disease due to antiplatelet antibodies, that will become a chronic disease in 70% of adults. Most of chronic ITP patients display clonal restriction of antiplatelet antibodies. To date, there is no biomarker able to predict the evolution of the disease. The objective of the study is to determine whether Hevylite® and/or Freelite® assays are prognostic factors for progression to chronic ITP. METHODS: This is a retrospective, monocentric, prognostic study of a biomarker, performed using frozen samples stored in a serum library. Freelite® and a Hevylite® assays were performed on the samples collected at diagnosis for adult patients with newly diagnosed ITP at the University Hospital of Poitiers between 2014/01/01 and 2017/05/01. To predict the evolution into a chronic disease, a ROC curve analysis was performed on four variables: IgGκ, IgGκ/IgGλ ratio, IgGκ - IgGλ, and κ/λ ratio. RESULTS: Thirty-two patients were included and analyzed. No patient had an abnormal κ/λ ratio. Three patients had an abnormal IgGκ/IgGλ ratio. The following variables IgGκ, IgGκ/IgGλ, IgGκ - IgGλ, and κ/λ ratio were not able to predict progression to chronic ITP in our study. CONCLUSION: This study did not reveal any prognostic value of the Freelite® and Hevylite® tests on the evolution of ITP into a chronic disease.

[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.

[Acute intermittent porphyria associated with hyperaldosteronism and inappropriate antidiuretic hormone secretion syndrome]. / Porphyrie aiguë intermittente associée à un hyperaldostéronisme et à un syndrome de sécrétion inappropriée d'hormone anti-diurétique.

Acute intermittent porphyria, the most commun acute porphyria in France, is an autosomal dominant disorder of heme biosynthesis. The basic biochemical defect is reduced activity of the enzyme porphobilinogen deaminase. Clinical evolution is characterized by acute attacks, with a severe prognosis due to acute abdominal pain and risk of neurological complications, induced by drug intake, infection, alcohol intake or unknown factors. We report the case of a patient with an inappropriate antidiuretic secretion syndrome and secondary hyperaldosteronism associated with acute intermittent porphyria and polyradiculoneuritis syndrome. This syndrome was found to be induced a delayed reaction to thiopental. A favorable response was achieved with heme-arginate treatment.

[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.

[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.

[Chronic idiopathic thrombocytopenia outcome during pregnancy (62 cases)]. / Évolution d'une thrombopénie chronique idiopathique en cours de grossesse (62 grossesses).

PURPOSE: The aim of this study was to assess the platelet count outcome during a pregnancy occurring in a series of 62 women followed for a chronic idiopathic thrombocytopenia. METHODS: We studied the medical files of women who had a previous history of chronic idiopathic thrombocytopenia persistently below 150G/L for at least 1 year, and who became pregnant over a 14-year period. RESULTS: Sixty-two pregnancies (including 41 in women suffering from an immune thrombocytopenic purpura according to updated definition criteria) which occurred in 50 women, were analysed. At the beginning of the pregnancy, platelet count was above 150G/L in 16% of the cases and lower than 50G/L in 8%. Platelets decreased by more than 25% for 55% of the pregnancies, remained stable during pregnancy in 33% and improved in 12%. Platelet count remained above 50G/L in 70% of the pregnancies and higher than 100G/L in 27%. Mean nadir was 84G/L at 31 weeks of gestation. A treatment was started in 40% of pregnancies, among them 64% of the cases during the last month only in order to allow locoregional anaesthesia at delivery. Platelet count was below 150G/L at delivery in 82% of the women (116±56G/L). No bleeding occurred in 83% of the pregnancies. Neonatal mean platelet count was 225±87G/L, thrombocytopenia occurred in 17% of the babies (platelet count below 150G/L), without any serious bleeding. CONCLUSION: Pregnancy worsens chronic idiopathic thrombocytopenia outcome in half of the cases, most of the time without any haemorrhagic complications.