Castleman disease

Castleman disease is a benign lymphoproliferative disorder that affects the lymphatic system and can present in either a localized or multicentric form. In the localized type, individuals may have a discrete group of enlarged lymph nodes, while the multicentric form is characterized by widespread lymphadenopathy along with systemic symptoms. The exact prevalence is not well established, and the condition is considered uncommon. Its cause is not linked to any known genetic mutations and appears to result from complex immune and inflammatory processes.

Individuals with Castleman disease often present with enlarged lymph nodes, which can be found in the mediastinum, abdomen, or other regions. Many experience systemic features such as weight loss, fatigue, and anemia, as well as laboratory indications of inflammation like an elevated erythrocyte sedimentation rate and increased circulating C-reactive protein. Other symptoms, including abdominal pain, nausea, vomiting, and jaundice, may develop and vary in severity between patients. Not all individuals experience all features, and severity varies considerably.

The precise genetic basis of Castleman disease is under investigation, and no specific genes have been implicated in its development. As such, there is no defined inheritance pattern associated with this condition. Castleman disease is thought to result from complex interactions within the immune system that lead to abnormal lymph node proliferation and an inflammatory response. Given the current understanding, family history does not appear to play a significant role in the risk for developing this condition.

Currently, management focuses on supportive care and symptom management since there are no treatments specifically approved for Castleman disease. Treatment is tailored to the individual and typically involves a multidisciplinary team to address both systemic symptoms and local manifestations. Regular surveillance is an essential part of long-term care, helping to identify potential complications before they become serious. Patients should discuss treatment options with their healthcare team to determine which supportive strategies may be most appropriate for their specific situation.

The clinical course of Castleman disease is highly variable, with some individuals experiencing only mild symptoms while others may develop significant systemic involvement. Outcomes described in medical literature continue to evolve as standards of care improve, and many patients benefit from timely diagnosis and targeted supportive therapies. The condition may progress, stabilize, or show a variable course depending on symptom severity, age at diagnosis, and access to multidisciplinary care. Families often find it helpful to connect with specialists familiar with this condition to receive guidance on long-term management and monitoring.

Castleman disease is an active area of research with numerous ongoing clinical trials investigating new approaches to treatment. The presence of active trials underscores the commitment to understanding and managing this condition, and individuals interested in exploring these options can search ClinicalTrials.gov or consult their care team about eligibility.