Lupus anticoagulant-hypoprothrombinemia syndrome presenting with co-existing cerebral venous thrombosis and subdural hemorrhage.

The lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) - the association of acquired factor II deficiency and lupus anticoagulant - is a rare disease that may cause a predisposition not only to thrombosis but also to severe bleeding. We are reporting on a 36-year-old female patient presenting with co-existing cerebral venous thrombosis and subdural hemorrhage. The coagulation screening showed a prolonged prothrombin time (PT), activated partial thromboplastin time (aPTT), and a normal fibrinogen level and platelet count. Evaluation of the clotting factors revealed decreased levels of factors II (37%). Factors V, VIII, IX and XI were normal. Lupus anticoagulant (LA) was demonstrated by the Dilute Russell's Viper Venom Test (DRVVT). Immunological work-up was positive for IgG type anticardiolipines antibodies (aCL). Successful management consisted first of oral prednisone (60mg/d). Thus, anticoagulation was introduced once factor II had stabilized.

[Pathophysiology of Behçet's disease]. / Physiopathologie de la maladie de Behçet.

Although the precise pathogenesis and etiology of Behçet's disease (BD) still remains unknown, current evidence suggests that inflammatory reaction in BD arises from disruption of homeostasis in genetically susceptible individuals, resulting in altered innate and adaptive immunity responses, pathogenic T cell activation in the peripheral blood, and in inflammatory sites. Association with HLA-B51 is known as the strongest genetic susceptibility factor for BD. Recent GWAS (genome-wide association studies) have confirmed this relationship, and reported new susceptibility genes (IL-10, IL-23R, IL-12RB2) for the disease. A triggering infectious agent could operate through molecular mimicry, and the disease could subsequently be perpetuated by an abnormal immune response to an auto-antigen in the absence of ongoing infection. Several potential bacteria have been investigated but the most commonly implicated microorganism is Streptococcus sanguis. Recent data have showed that the T cell homeostasis perturbation consisted mainly of Th1 and Th17 expansions, while regulatory T cell response was suppressed. Cytokine such as IL-17, IL-23 and IL-21 play a significant role in the pathogenesis of BD. Inflammatory cells within BD inflammatory lesions include mostly neutrophils, CD4(+) T cells, and cytotoxic cells. Lastly, endothelium dysfunction has been clearly established. This improved understanding of the pathophysiology of BD will certainly lead to the development of new therapeutic agents, potentially more effective than current therapy. In this review, we have studied the etiopathogenesis of BD in the light of recent advances.

Chest pain in patients with undiagnosed Behçet's disease.

Behçet's disease (BD) is a systemic inflammatory disease having a chronic and prolonged course with 4 major symptoms: oral and genital ulcerations, eye disease and cutaneous manifestations, as well as other multisystem involvements. Arterial involvement is a comparatively rare complication in BD and coronary lesions are extremely rare. We report here two cases of BD presenting as myocardial infarction (MI) with coronary artery aneurysm (CAA), with good improvement after immunosuppressive therapy.