ما الإجراء الواجب اتخاذه عند فشل العلاج بمضادات IL-6 في الخط الأول لداء كاستلمان متعدد المراكز مجهول السبب غير الحاد

DOI: 10.1182/blood-2018-07-862334

• iMCD patients who are not severely sick are typically diagnosed in the outpatient setting and have a good performance status without evidence of abnormal organ function, whereas other patients are more symptomatic and often exhibit an IL-6–driven inflammatory response that interferes significantly with their ability to function and work.
• Patients should be classified as nonsevere iMCD if the above criteria are not met.
• Second-line therapy should comprise rituximab to which immunomodulatory/immunosuppressive agents (Figure 2), and steroids may be added.
• Immunomodulatory/immunosuppressive agents for second- or third-line therapy include thalidomide, cyclosporine A, sirolimus, anakinra, or bortezomib, but we recommend consulting with an expert at this stage.