LANGERHANS CELL HISTIOCYTOSIS

Course overview

LANGERHANS CELL HISTIOCYTOSIS (LCH) IS AN IDIOPATHIC CONDITION CHARACTERIZED BY PROLIFERATION OF ABNORMAL LANGERHANS (ANTIGEN-PRESENTING) CELLS. THE DISEASE HAS CHARACTERISTICS OF BOTH AN ABNORMAL REACTIVE PROCESS AND A NEOPLASTIC PROCESS. IT MAY PRESENT INITIALLY AS A RASH. IT CAN BE DISSEMINATED AND INVOLVE THE BONE MARROW, LUNGS, LIVER, SPLEEN, LYMPH NODES, GASTROINTESTINAL TRACT, AND PITUITARY GLAND. PROGNOSIS VARIES DEPENDING ON PRESENTATION AND ORGAN INVOLVEMENT. LCH CAN OCCUR AT ANY AGE BUT IS MOST COMMON FROM BIRTH TO AGE 15 YEARS. THERE IS A WIDE VARIATION IN PRESENTATION, TREATMENT, AND PROGNOSIS. REFERRAL AND LONG-TERM FOLLOW UP WITH AN ONCOLOGIST ARE IMPORTANT IN THE MANAGEMENT OF LCH. THIS ACTIVITY DESCRIBES THE PRESENTATION, EVALUATION, AND MANAGEMENT OF LANGERHANS CELL HISTIOCYTOSIS AND HIGHLIGHTS THE ROLE OF THE INTERPROFESSIONAL TEAM IN THE CARE OF AFFECTED PATIENTS.