Joint Topic: Rarer H/O Diagnoses — Manifestations and Management of Langerhans Cell Histiocytosis (220-2)

3:30 – 4:30 pm Friday, October 29

Langerhans cell histiocytosis (LCH) is a rare multisystem disease that arises from the myeloid dendritic cells and often presents as common childhood ailments (Zinn, Grimes, Lin, Eckstein, Allen, & McClain, 2016). Histiocytes invade many different organ systems, including the lymph nodes, lungs, thymus, liver, spleen, bone marrow, and/or the central nervous system (McClain, 2019).

The initial disease presentation may not appear as an acute or immediate life-threatening situation, so initial diagnosis can be challenging. Presenting symptoms, such as otorrhea, oral lesions, diarrhea, eczematous rash, increased thirst, increased urination, or pain, are vague (McClain, 2019). LCH can invade many organs and cause devastating illness. Untreated disease can be fatal. Treatment involves chemotherapy for an extended period, and responses to treatment can be unpredictable. Neuro-endocrine complications, such as diabetes insipidus and learning disabilities, are chronic and negatively impact quality of life. We will review case studies to illustrate common presenting symptoms, the diagnostic process, common treatments, potential complications, and neuro-endocrine and developmental late effects.