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Langerhans cell histiocytosis (LCH) is a nonmalignant disease characterized by an accumulation of dendritic cells. The disease can affect multiple organs, and the clinical picture ranges from localized bone lesions or skin disease to multiple organ involvement and severe dysfunction. LCH most commonly affects children, with the peak incidence at 1 to 4 years of age. Prognosis is dependent on the number of organ systems involved, the degree to which normal function of the organ system is affected, and the rate of progression of the disease. In the majority of children, the disease is self-resolving. For patients with multisystem disease, it is most commonly treated with steroids and chemotherapeutic agents including prednisone, vinblastine, and mercaptop-urine. Although LCH is not considered a malignancy, the pediatric oncology nurse plays a key role in disease management because these patients are often followed in inpatient and outpatient oncology settings. This article provides nurses with insight about the pathophysiology and treatment modalities of LCH and prepares the nurse to provide confident comprehensive nursing care and to educate patients and families.