Traitement du PTI et de l’AHAI au cours du DICV : revue systématique de la littérature

INTRODUCTION: Ten to 15% of common variable immunodeficiencies (CVID) develop auto-immune hemolytic anemia (AIHA) and immune thrombocytopenia (ITP). Treatment is based on immunosuppressants, which produce blocking effects in the CVID. Our objective was to assess their risk-benefit ratio in these immunocompromised patients. METHODS: We identified 17 articles detailing the treatment of AIHA and/or ITP in patients suffering from CVID through a systematic review of the MEDLINE database. RESULTS: The increased infectious risk with corticosteroids does not call into question their place in the first line of treatment of ITP and AIHA in CVID. High-doses immunoglobulin therapy remain reserved for ITP with a high risk of bleeding. In second-line treatment, rituximab appears to be effective, with a lower infectious risk than the splenectomy. Immunosuppressants (azathioprine, methotrexate, mycophenolate, cyclophosphamide, vincristine, ciclosporine) are moderately effective and often lead to severe infections, meaning that their use is justified only in resistant cases and steroid-sparing. Dapsone, danazol and anti-D immunoglobulins have an unfavorable risk-benefit ratio. The place of TPO receptor agonists is still to be defined. The establishment of immunoglobulin replacement in the place of immunosuppressants (except for short-term corticotherapy) or splenectomy appears to be essential to limit the risk of infections, including in the absence of previous infections. CONCLUSION: The presence of CVID does not mean that it is necessary to give up on corticosteroids as a first-line treatment and rituximab as a second-line treatment for AIHA and ITP, but it should be in addition to immunoglobulin replacement. A splenectomy should be reserved as a third-line treatment

Urticaire chronique superficielle associée aux cancers solides : un cas et revue de la littérature

BACKGROUND: Chronic urticaria is common and is generally idiopathic ("spontaneous"). Links between solid cancer and chronic urticaria have been mentioned in the literature. PATIENTS AND METHODS: We report the case of a 63-year-old man presenting with superficial chronic urticaria associated with adenocarcinoma of the ethmoid sinus. We discuss the possibility of systemic origin in light of the severity of the disease and its resistance to treatment. Only recurrent ethmoidal cancer was highlighted. Curative care resulted in complete resolution of the urticaria without relapse at 32 months of follow-up. DISCUSSION: In a literature review, we collected 17 cases of superficial chronic urticaria associated with cancer. These cases were marked by synchronous progression and by the inefficacy (86%) of anti-histamines and systemic corticosteroids. Although cases of chronic superficial urticaria associated with cancer remain rare, the condition merits discussion due to its severity and significant resistance to therapy