[Acquired haemophilia A: two cases]. / Deux observations d'hémophilie A acquise.

BACKGROUND: Acquired haemophilia A (AH) is an uncommon bleeding disorder that presents as multiple, disseminated spontaneous subcutaneous bleeds. Diagnosis may be made on the basis of prolonged activated partial thromboplastin time (aPTT). The severity of the disease is associated with the low risk of haemoglobin levels and with potential links with other diseases. OBSERVATIONS: Two men were hospitalized for extensive and spontaneous subcutaneous hematoma. In both cases, the International Normalized Ratio (INR) was normal, but aPTT was 3 times higher than normal. Autoantibodies against coagulation factor VIII confirmed the diagnosis of AH. The patients received immunomodulatory treatment. In one patient, diffuse large B-cell lymphoma was discovered one year after successful treatment of AH. DISCUSSION: AH may be revealed by areas of bruising, subutaneous haematomas mimicking erythema nodosum, and muscle pain. APTT results alone can prompt the biologist to screen for factor VIII inhibitors. Aside from the risk of fatal bleeding, in half of all cases, the prognosis is determined by associated disorders such as blood dyscrasias, solid tumours, autoimmune diseases, use of certain medicines and pregnancy. After treatment for bleeding complications, therapy focuses on restoring the coagulation time. The aim of immunomodulatory therapy is to stem production of autoantibodies against coagulation factor VIII. CONCLUSION: AH must be considered rapidly in order to reduce the risk of bleeding emergencies and to screen for potential related diseases.

[Efficacy of TNF-alpha antagonists for plaque-type psoriasis: a systematic review and graphical presentation]. / Efficacité des anti-TNF-alpha dans le psoriasis: revue systématique et représentation graphique.

BACKGROUND: Numerous studies investigating the efficacy of TNF-alpha antagonists in moderate-to-severe plaque-type psoriasis have been published. We present a graphic synthesis of efficacy data based on a systematic review of the published studies. METHODS: The Medline, Embase and Cochrane Library bibliographic databases were searched for studies based on the following selection criteria: 1. Original studies, 2. Using infliximab, etanercept or adalimumab as the sole systemic treatment for plaque-type cutaneous psoriasis, 3. Minimum follow-up of 10 weeks, 4. Use of the PASI75 index (improvement of at least 75% from baseline PASI score) as an endpoint. Twenty-one articles were selected and PASI75 scores and corresponding 95% confidence intervals were calculated at three, six and 12 months. RESULTS: At M3, success rates were about 80% with infliximab and adalimumab once-weekly, around 50% with etanercept 50mg twice-weekly and adalimumab fortnightly; and 30% with etanercept 25mg twice-weekly. Maintenance regimens after three months ensured success rates above 50% with all three treatments, although the long-term results were based on fewer studies. CONCLUSION: Our graphic synthesis and tolerance data for the three drugs should guide clinicians in their therapeutic choices in moderate-to-severe plaque-type psoriasis. Additional studies with better reporting of loss to follow-up are needed to better assess the long-term efficacy of these drugs.

[Primary pleural lymphoma: a late complication of pleural decortication for tuberculosis: two cases in western countries]. / Lymphome pleural primitif: une complication tardive de la collapsothérapie chez des anciens tuberculeux. A propos de deux nouveaux cas.

Pleural lymphomas after a long standing pyothorax due to pleuropulmonary tuberculosis are now well identified, but rarely observed in Europe. We report two new cases in a non-immunocompromised patients. The two cases occurred 5455 years following artificial pneumothorax for pulmonary tuberculosis. The patients presented with a localized pleural tumor mass. Histology revealed high-grade lymphomas, diffuse large B-cell lymphoma and anaplastic lymphoma. Serology for Epstein-Barr virus was positive. Pleural lymphomas are an established complication of artificial pneumothorax. Epstein-Barr virus is known to play a crucial role in the pathogenesis, but despite the large number of artificial pneumothorax operations, these lymphomas remain rare, suggesting additional oncogenic factors.