Lung transplantation in a patient with severe bronchiolitis obliterans secondary to Castleman disease.

Abstract

Castlemann disease (CD) is a rare but often benign condition of the lymph nodes. Seldomly coinciding with CD are other conditions, such as paraneoplastic phemhigus (PMP) and bronchiolitis obliterans (BO). When these occur, patients are at risk of developing severe complications, such as respiratory failure, which may subsequently result in death. Diagnosing and managing conditions related to CD poses a challenge and may cause diagnostic delay. In this case report, we describe the course of illness in a 31-year-old female diagnosed with CD. Prior to the diagnosis of CD, the patient had been diagnosed with both paraneoplastic phemfigus and an irreversible airway obstruction. Standard surgical treatment for CD, as well as standard medical treatment for airway obstruction, had little effect. Radiologic findings supported the diagnosis of bronchiolitis obliterans. A progressive decline in pulmonary function eventually led to the patient being evaluated for and undergoing lung transplantation. The co-occurrence of CD and PMP has previously been described as increasing the risk of developing BO, irreversible pulmonary function restrictions and poor prognosis. Lung transplant may be a possible treatment for some patients with terminal respiratory failure.