علاج داء كاستلمان متعدد المراكز مجهول السبب بدون خلل وظيفي حاد في الأعضاء

References

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.
• We recommend use of an immunomodulatory/immunosuppressive agent because these agents have less toxicity than chemotherapy and have similar efficacy (69% response), albeit in fewer case reports.
• These agents include cyclosporine A, sirolimus, thalidomide, lenalidomide, bortezomib, the IL-1b receptor antagonist anakinra, retinoic acid derivatives, and interferon-a.