9/28/2018 With the CDCN’s leadership, another major milestone has been achieved in the fight against Castleman Disease! The CDCN convened 42 experts from 10 different countries to establish the first-ever treatment guidelines for idiopathic (HHV-8-negative) multicentric Castleman Disease (iMCD) based on data from over 300 iMCD patients. CDCN co-founder, Dr. Frits van Rhee, is the Lead author of the study and CDCN co-founder, Dr. David Fajgenbaum, is the Senior author. This landmark study is pre-published in Blood, the top hematology journal in the world. Before this study, physicians had to rely on past case reports and personal experience to determine the best treatment for their patients with iMCD. Now, physicians will be able to use these treatment guidelines to help determine the best treatment for their iMCD patients. Knowing which treatment is most likely to help the sickest iMCD patients — and when to try a new treatment if the initial treatments aren’t working — can ultimately save lives.
This paper, entitled “International, evidence-based consensus treatment guidelines for idiopathic multicentric Castleman disease,” details a pathway for physicians to follow when treating iMCD patients. Determining the right treatment rests first on differentiating between severe (life-threatening organ dysfunction, see below) and non-severe iMCD patients. For patients with non-severe iMCD, the anti-IL-6 medication siltuximab with or without steroids is first-line treatment. If siltuximab is not available, tocilizumab may be used instead. For patients with severe iMCD, siltuximab with high-dose steroids is started. If a patient with severe iMCD demonstrates any worsening of organ dysfunction at any time or does not improve within one week on anti-IL-6 medications, combination chemotherapy is recommended. Recommendations are also made for patients who do not respond to anti-IL-6 medication in first-line. The full text of the paper can be found here.
Please note that these treatment guidelines are only for iMCD. Treatment information for HHV-8-positive MCD can be found here with a treatment algorithm displayed here. Treatment information for Unicentric Castleman disease can be found here with a treatment algorithm here. More information on past treatments used for patients with all subtypes of Castleman disease can be found here.
The CDCN encourages physicians and patients to use these guidelines as part of their treatment decision-making process. The CDCN supports these guidelines but does not recommend any specific treatment for patients as every treatment plan should be tailored to each patient and made with thorough discussion with their physicians. You can read an article about this major milestone in Hematology News here.