UAMS Physician Establishes Second International Guidelines for Treating Castleman Disease

LITTLE ROCK -- Frits van Rhee, MD, PhD, a myeloma researcher and clinician at the University of Arkansas for Medical Sciences (UAMS), was lead author on a recently published paper that establishes consensus diagnosis and treatment guidelines for a form of Castleman disease, a rare disorder of the lymph nodes and related tissues.

The recommendations were created to improve outcomes in patients with a form of the disease called unicentric Castleman disease (UCD) that affects a single lymph node area and can compress vital structures such a blood vessels, nerve bundles or the airways. In others, UCD can give rise to night sweats, fevers, weight loss and anemia

Castleman disease is rare so people don't know how to treat it and until recently there has not been any systematic approach to UCD according to van Rhee, an international expert on Castleman disease.

Van Rhee is a professor of medicine, clinical director of the UAMS Myeloma Center and holds the Charles and Clydene Scharlau Chair for Hematologic Malignancies Research.

The paper, "International Evidence-based Consensus Diagnostic and Treatment Guidelines for Unicentric Castleman Disease" was recently published in Blood Advances, an online publication of the American Society of Hematology. It included research and input from van Rhee and 41 other specialists, researchers, and clinicians from 10 countries.

The expert panel convened in a number of meetings organized by the Castleman Disease Collaborative Network, which van Rhee cofounded in 2012 with his patient David Fajgenbaum, M.D. The panel based the guidelines on published literature, a review of treatment in published cases of UCD, and data from an international registry from the network for patients with Castleman disease.

If it cannot be removed surgically and you have symptoms because the mass is pressing on something vital, treatment can be much more difficult. A classic example is a mass in the chest that is near one of the major airways like the windpipe or the two main branches of the windpipe. Sometimes the only way to remove the mass is to remove one of the lungs and obviously you want to try to avoid that.

In those cases, the guidelines recommend partial surgical removal or treatment with other agents, including corticosteroids, antibodies or radiation.

Castleman disease occurs when an abnormal overgrowth of cells occurs in the lymph system, which serves as the main part of the body's immune system. The disease, which affects 5,000 to 6,000 patients across the nation, was identified by Benjamin Castleman, M.D., in 1954.

Van Rhee was previously the principal investigator on a worldwide trial with siltuximab, which led to the first FDA-approved treatment for multicentric Castleman disease and led to the approval of the drug by the European Medicine Agency.