[Hemolytic disorders and venous thrombosis: An update]. / Maladies hémolytiques et thrombose veineuse : mise au point.

Many factors can contribute to the risk of venous thrombosis observed in hemolytic diseases. Some mechanisms are related to hemolysis by itself, while others seem more specific to each disease. Despite recent advances in the quantification of this risk and in understanding its physiopathology, the association of hemolysis with venous thrombosis is often unknown. The purpose of this general review is to clarify the main pro-thrombotic mechanisms during hemolysis and to synthesize the clinical data currently available. We will focus on the main types of hemolytic pathologies encountered in current practice, namely paroxysmal nocturnal hemoglobinuria, hemoglobinopathies, auto-immune hemolytic anemia and thrombotic microangiopathies.

[Validity and reproducibility of two direct observation assessment forms for evaluation of internal medicine residents' clinical skills]. / Validité et reproductibilité de deux grilles d'observation des compétences cliniques des internes en DES de médecine interne.

INTRODUCTION: The revision of the French medical studies' third cycle ought to be competency-based. In internal medicine, theoretical and practical knowledge will be assessed online with e-learning and e-portfolio. In parallel, a reflection about clinical skills assessment forms is currently ongoing. In this context, our aim was to assess the reproducibility and validity of two assessment forms based on direct clinical observation. METHOD: A prospective and multicentric study has been conducted from November 2015 to October 2016 aiming at evaluating the French translations of the MINI-Clinical Examination Exercice (MINI-CEX) and the Standardized Patient Satisfaction Questionnaire (SPSQ). Included residents have been assessed 2 times over a period of 6 months by the same binoma of judges. RESULTS: Nineteen residents have been included. The inter-judge reproducibility was satisfactory for the MINI-CEX: intraclass coefficients (ICC) between 0.4 and 0.8 and moderate for the SPSQ: ICC between 0.2 and 0.7 with a good internal coherence for both questionnaires (Cronbach between 0.92 and 0.94). Significant differences between the distributions of the scores given by the judges and a significant inter-center variability have been found. CONCLUSION: If the absolute value of the scores should not be taken into account in the evaluation process given its high variability, it could be of interest for the follow-up of the progression in the competencies. These forms could support the residents' debriefing based on the general trends given by the scores.

Venous thromboembolism related to warm autoimmune hemolytic anemia: a case-control study.

BACKGROUND: The risk of venous thromboembolism (VTE) during warm autoimmune hemolytic anemia (wAIHA) is apparent in several published series. Unlike proximate disorders (autoimmune thrombocytopenia, non-immune hemolytic diseases) little is known about the presentation and risk factors for VTE in this setting. OBJECTIVE: To determine the frequency, presentation and risk factors for VTE associated with wAIHA. METHODS: We performed a single center retrospective study of adult patients (>18years) followed for wAIHA between 2009 and 2013. VTE risk factors were systematically assessed. The characteristics of patients with or without VTE were compared. VTE presentation and precipitating factors were analyzed. The Padua VTE risk score was calculated in each case. RESULTS: Forty patients were included. wAIHA was idiopathic in 24 patients (60%). Twelve patients (30%) had Evans syndrome. Mean lowest hemoglobin level was 6.6g/dl [3.7-11.5]. Eight patients (20%) presented VTE after the appearance of wAIHA, at a mean age of 52.5years. All patients had pulmonary embolus, associated with a deep venous thrombosis in 4 cases. At the time of VTE 7/8 patients had frank hemolysis (median hemoglobin level: 7g/dL) and 6/8 were outpatients with a low Padua VTE risk score. The frequency of usual VTE risk factor was similar in cases and controls. By contrast, lowest hemoglobin level was significantly lower in patients that experienced VTE (5.3 vs 7.2g/dL, p=0.016). During the first episode of wAIHA, patients with concurrent VTE had a more pronounced anemia (5.3 vs 7.4g/dL, p=0.026). At the time of VTE, anemia was more severe when no other precipitating factor was present (6 vs 8.9g.dL, p=0.04). CONCLUSION: In our cohort, 20% of patients with wAIHA presented VTE. The vast majority of VTE occurred during severe hemolytic flares and were not attributable to usual VTE risk factors. VTE prophylaxis is advisable in any patient admitted for wAIHA, irrespective of Padua VTE risk score. Prophylaxis also seems reasonable for outpatients with marked hemolysis.

[Unilateral isolated Raynaud's phenomenon leading to diagnosis of multifocal giant-cell arteritis complicated with renal ischemia]. / Phénomène de Raynaud unilatéral isolé révélateur d'une artérite à cellules géantes multifocale (maladie de Horton) compliquée d'ischémie rénale.

We report a case of a 76-year-old woman with isolated unilateral Raynaud phenomenon revealing giant-cell arteritis with diffuse arterial lesions and bilateral renal artery stenosis. Doppler ultrasonography showed bilateral stenosis of the subclavian and axillary arteries. Angio-CT PET enlightened diffuse arterial lesions, mainly involving the aorta and the brachial and femoral arteries as well as bilateral renal ostial stenosis with right kidney ischemia. Diagnosis of giant-cell arteritis was made on the temporal artery biopsy. Corticosteroid therapy led to rapid clinical and radiological improvement. Clinical manifestations of giant-cell arteritis may be atypical. Diffuse arterial disease may exist in the absence of cephalic symptoms or significant inflammatory biological features. Ostial renal artery stenosis may induce potentially threatening renal ischemia.