Survivorship Issues in Adult Patients With Histiocytic Neoplasms

Abstract

Adult-onset histiocytoses (AOH), primarily Rosai-Dorfman disease (RDD), Erdheim-Chester Disease (ECD), and adult Langerhans cell histiocytosis (ALCH), are a group of related histiocytic neoplastic disorders featuring multisystemic manifestations. The disorders are largely incurable, and are essentially chronic neoplastic diseases with a variable prognosis. Prompt diagnosis and treatment is important to prevent debilitating and even life-threatening complications. Survivorship issues abound in AOH, due to their multisystemic manifestations and the sometimes recalcitrant chronic inflammation, which can lead to other debilitating complications such as fatigue, weakness, and pain. Because these disorders are rare, few healthcare professionals are proficient in their management; therefore the aim of these guidelines is to offer guidance on how to manage patients, and how to create survivorship care plans through the efforts of an interdisciplinary team.

Challenges and positive impact of rare cancer caregiving: A mixed-methods study of caregivers of patients with Erdheim-Chester disease and other histiocytic neoplasms

“…Increased survivorship has allowed for investigation of psychosocial outcomes, symptomatology, and caregiver experiences. 6 , 7 , 8 …”