Allogeneic Bone Marrow Transplantation in a Patient with Chemotherapy-Resistant Progressive Histiocytosis X

HISTIOCYTOSIS X is the name for a spectrum of rare disorders — Letterer–Siwe disease, Hand–Schüller–Christian disease, and eosinophilic granuloma 1 2 3 — that have a wide range of clinical manifestations but are all characterized by granuloma formation, with infiltration and proliferation of histioc...

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Veröffentlicht in:The New England journal of medicine 1987-03, Vol.316 (12), p.733-735
Hauptverfasser: Ringdén, Olle, Åhström, Lars, Lönnqvist, Berit, Båryd, Ingvar, Svedmyr, Erik, Gahrton, Gösta
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Sprache:eng
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Zusammenfassung:HISTIOCYTOSIS X is the name for a spectrum of rare disorders — Letterer–Siwe disease, Hand–Schüller–Christian disease, and eosinophilic granuloma 1 2 3 — that have a wide range of clinical manifestations but are all characterized by granuloma formation, with infiltration and proliferation of histiocytes. In many cases, the symptoms include pain caused by one or more bone lesions. Lymph-node involvement occurs frequently in younger patients. The benign forms of histiocytosis X are characterized by spontaneous remissions and exacerbations. Disseminated fulminant disease progresses rapidly and has an unfavorable outcome. The diagnosis is based on histologie criteria, which can be used as guidelines for prognosis . . .
ISSN:0028-4793
1533-4406
DOI:10.1056/NEJM198703193161207