NCCN Guidelines for Castleman's Disease

Corey Casper, MD, MPH:For diseases that are very rare and that clinicians don’t see a lot, it’s really useful to have guidelines to help provide not just clues to diagnosis but also to therapy. The NCCN has come out with a series of guidelines around the treatment of multicentric Castleman’s disease, and these can be helpful for providers thinking about the treatment of the disease. But, I would really emphasize that these are guidelines and they’re guidelines writ large. So, every provider needs to keep the individual patient in front of them in mind when they consider the appropriateness of these guidelines. When you look at the NCCN Guidelines, the first branch point after you’ve made a diagnosis of Castleman’s disease is determining whether it’s idiopathic or related to human herpesvirus-8. In the NCCN Guidelines, for human herpesvirus-8—associated Castleman’s disease, they recommend one of two different options. The preferred option that they cite is the use of rituximab with liposomal doxorubicin. As an alternative, they cite a dual antiviral therapy with zidovudine and ganciclovir.

In my experience, I would say that responses to rituximab and doxorubicin are limited. In my experience, about half of patients respond to that and recurrences are common. There also can be long-term sequelae of treatment like that, like a higher incidence of Kaposi sarcoma. It’s not my practice pattern to start with that. In most of my patients with HHV-8—associated Castleman’s disease, I tend to use antiviral therapy first; either rerecommended potential options from NCCN for first-line therapy. In the other branch point for initial therapy, the guidelines recommend the use of siltuximab, as this is really the only FDA-approved drug for the treatment of Castleman’s disease. There is mentioned, in the guidelines, the use of other therapies such as cytotoxic chemotherapy or rituximab. And, again, I think that there may be different situations that influence in your individual patient which of those options you’d like to choose.

The guidelines also address relapsed or refractory disease with Castleman’s disease, and I recognize that these are terms that are commonly used with malignancies and Castleman’s is not a malignancy, maybe a premalignant condition. But, certainly, I think that those phraseologies are useful. So, many patients may not respond to first-line therapy and so, in those patients, again, the guidelines call for the use of conventional chemotherapy or monoclonal antibody biologics against CD20 as therapy for relapsed or refractory disease.

Corey Casper, MD, provides information on the diagnosis and treatment of patients with Castleman Disease (CD).