Langerhans cell histiocytosis.
Langerhans cell histiocytosis (LCH) is a poorly understood proliferative disease, with different patterns of clinical presentation. Currently it is classified according to the number and type of system involved and the degree of organ dysfunction. The aetiology of the disease remains uncertain, and in some cases the disease is polyclonal, suggesting a reactive condition. Many cytokines have been implicated in the pathogenesis of LCH. Different therapeutic approaches can be considered depending on the affected organ, including surgery, radiotherapy and chemotherapy. Long-term organ dysfunction may remain, despite disease control and/or eradication, making indefinite supportive treatment mandatory. Here we present a literature review on all of the aspects of the disease, treatment approaches and existing protocols, and finally an adult clinical case.
Gasent Blesa
Joan Manel
JM
Hospital General Universitari Marina Alta, Dènia, Alacant, Spain. joanmagasent@telefonica.net
Alberola Candel
Vicente
V
Solano Vercet
Carlos
C
Laforga Canales
Juan
J
Semler
Christof
C
Pérez Antolí
Maria Rosa
MR
Rodríguez-Galindo
Carlos
C
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002999:Clone Cells / Q000473:pathology; D003131:Combined Modality Therapy; D016207:Cytokines / Q000502:physiology; D054697:Cytostatic Agents / Q000627:therapeutic use; D004359:Drug Therapy, Combination; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000276:immunology / Q000473:pathology / Q000628:therapy; D006801:Humans; D007801:Langerhans Cells / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015337:Multicenter Studies as Topic / Q000706:statistics & numerical data; D009928:Organ Specificity; D011379:Prognosis; D016032:Randomized Controlled Trials as Topic / Q000706:statistics & numerical data; D012008:Recurrence; D055815:Young Adult
D016207:Cytokines; D054697:Cytostatic Agents