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Aaron Goodman, MD, Tells Us the 'Crux' of Castleman Disease

Aaron Goodman, MD, an Associate Professor of Medicine at the University of California San Diego, joined Chadi Nabhan, MD, MBA, FACP, on “The HemOnc Pulse” to discuss Castleman disease and how each subtype is diagnosed and treated. The following excerpt is taken from this week’s episode. Dr. Nabhan: We wanted to discuss today Castleman disease, because it’s not really a very common disease. Dr. Goodman: The crux of Castleman, and why it’s horribly understood and mismanaged, is that it’s incredibly rare. It’s many different diseases, and it falls across many different specialties. Patients with unicentric Castleman disease are asymptomatic other than an isolated or regional group of lymph nodes that are enlarged. When you resect [the lymph node], it’s gone and the patient’s done with the disease. They don’t have any B symptoms, inflammatory symptoms, or other lab abnormalities. Dr. Nabhan: Is it a diagnosis of exclusion? Dr. Goodman: Basically, it has to present. In unicentric Castleman disease, the pathologist has to have Castleman-like findings with one lymph node. Usually, it’s in the mediastinum or in the cervical chain. I’ve seen patients that presented with large intraabdominal masses. Most cases of unicentric Castleman that I’ve seen are incidental findings on imaging. What causes this? We have no idea. The suspicion is that there is a not cancer, but a clonal population of cells within the lymph node, particularly within the follicular dendritic meshwork. Dr. Nabhan: Is Castleman cancer? Dr. Goodman: No. Dr. Nabhan: Why would you treat it then? Dr. Goodman: Unicentric is asymptomatic other than the regional node. The more confusing clinical scenario is the classic patient presents with fevers, B symptoms and fluid retention, and there’s a concern for an inflammatory disorder, bad infection, or even a malignancy. On that imaging study, they see modest lymphadenopathy. They’re referred for an excisional biopsy, which comes back with findings consistent with Castleman disease. They don’t see any Hodgkin lymphoma or follicular lymphoma. This patient has multicentric Castleman disease. There are three flavors of multicentric Castleman disease. One is human herpesvirus-8 (HHV-8) associated multicentric Castleman disease, which is almost always found in the setting of a concurrent HIV or AIDS diagnosis. If they’re HHV-8 negative, then they have idiopathic multicentric Castleman disease, otherwise known as David Fajgenbaum disease. Like and subscribe to @bloodcancerstoday2935 for all the latest news in hematology oncology.