Langerhans´ cellhistiocytos: nytt ljus över patogenesen. 75 år sedan Sture Siwes klassiska arbete om "systemisk retikuloendotelios"
Engelsk titel: Langerhans cell histiocytosis: new light over pathogenesis. 75 years since Sture Siwe´ classic work on "systemic reticuloendotheliosis"
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Författare:
Fadeel B
;
Olsson S
;
Jakobson Å
;
Hjorth L
;
Österlundh G
;
Henter JI
Email: bengt.fadeel@ki.se
Språk: Swe
Antal referenser: 55
Dokumenttyp:
Översikt
UI-nummer: 09011630
Personnamn som ämnesord:
Siwe S
Sammanfattning
Langerhans cell histiocytosis (LCH) is characterized by an accumulation of dendritic Langerhans cells in granulomatous lesions in the bone, skin, and other organs. The condition was previously classified as “Histiocytosis X” to reflect its enigmatic origin, and has also previously been referred to as Hand-Schüller-Christian disease (a triad of exophthalmus, diabetes insipidus, and bone lesions), Letterer-Siwe disease (an aggressive, systemic “reticuloendotheliosis” with accumulation of so-called histiocytes), and eosinophilic granuloma, a condition affecting only solitary organs. Lesional Langerhans cells have been reported to be monoclonal; however, LCH is not considered to be a conventional neoplastic disease. In fact, numerous recent studies suggest that LCH is a reactive disorder related to a fundamental disruption of the function of Langerhans cells, key players of the immune system. This review summarizes recent advances in understanding the pathogenesis and clinical features of LCH, and the treatment modalities that are currently available. We also provide an historic background to these achievements, in dedication to the Swedish pediatrician Sture Siwe who described the systemic form of LCH 75 years ago.