Clinical experience in an infant with Langerhans cell histiocytosis

Abstract

Introduction: Langerhans cell histiocytosis (LCH) or Histiocytosis X is a benign proliferative disease affecting dendritic cells. It presents a wide clinical spectrum, from isolated eosinophilic bone granuloma to multisystem involvement with multiple organ failure with or without resistance to chemotherapy. Case report: a 4-month-old male infant, with no family history of hematooncologic disease; his condition began two months earlier with lesions on the scalp, trunk, axillae and inguinal folds, and irritability. The family physician prescribed non-steroidal anti-inflammatory drugs with no improvement and the patient was referred to the dermatology department. Clinical examination revealed adynamia, hyporexia and weight loss, increased irritability on palpation of the hips, increased volume of the left leg and cervical lymphadenopathy. Additional tests were performed and a diagnosis of Langerhans cell histiocytosis was made. Treatment was started with six mercaptopurine and vinblastine, blood product transfusions and treatment with Filgastrim, the patient showed clinical improvement. Conclusions: Langerhans cell histiocytosis is a complex and rare disease which pathophysiology and prognosis have not yet been widely described. It is difficult to diagnose due to its heterogeneous presentation and its treatment must be individualized. This disease represents a challenge, and it is important to raise awareness among medical personnel of the need to deepen their knowledge with reference to the disease in order to establish a timely diagnosis and management.

Keywords

• histiocitosis
• células de langerhans
• linfocitos intraepiteliales
• alergia e inmunología
• inmunología
• granuloma.