Survivorship Issues in Adult Patients With Histiocytic Neoplasms

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  • 1 Office of the Clinical Director, National Human Genome Research Institute, NIH, Bethesda, Maryland;
  • | 2 Inova Fairfax-Virginia Commonwealth University College of Medicine, Falls Church, Virginia;
  • | 3 Clinical Research Branch, National Institute of Environmental Health Sciences, NIH, Bethesda, Maryland;
  • | 4 Eunice Kennedy Shriver National Institute of Child Health and Human Development, NIH, Bethesda, Maryland;
  • | 5 Rehabilitation Medicine Department, Clinical Center, NIH, Bethesda, Maryland;
  • | 6 Speech Language Pathology Section, Rehabilitation Medicine Department, Mark O. Hatfield Clinical Research Center, NIH, Bethesda, Maryland;
  • | 7 National Heart, Lung, and Blood Institute, NIH, Bethesda, Maryland; and
  • | 8 National Eye Institute, NIH, Bethesda, Maryland.

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.

Submitted July 6, 2021; accepted for publication September 22, 2021.

Author contributions: Patient care: O’Brien, Dave, Shekhar, Hannah-Shmouni, Comis, Solomon, Chen, FitzGibbon, Gochuico, Estrada-Veras. Data creation, entry, and analysis: O’Brien, Dave, Shekhar, Hannah-Shmouni, Comis, Solomon, Chen, Gochuico, Estrada-Veras. Manuscript preparation: All authors.

Disclosures: Dr. Dave has disclosed receiving grant/research support and consulting fees from and serving as a scientific advisor for Novartis Pharmaceuticals.

Funding: This work was supported in part by the Intramural Research Programs of the National Human Genome Research Institute; the National Heart, Lung, and Blood Institute; the Rehabilitation Medicine Department at the NIH Clinical Center; the Speech Language Pathology Section of the Rehabilitation Medicine Department at the NIH Clinical Center; Eunice Kennedy Shriver National Institute of Child Health and Human Development; the National Eye Institute, NIH; Clinical Research Branch, National Institute of Environmental Health Sciences, NIH; the National Institute of Neurologic Disorders and Stroke; and the Warren Grant Magnuson Clinical Center, NIH.

Correspondence: Kevin J. O’Brien, RN, MS, CRNP, National Human Genome Research Institute/National Institutes of Health, 10 Center Drive, Building, 10/CRC, Room 3-2551, Bethesda, MD 20892-1205. Email: obrienke@mail.nih.gov

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