[Acute disseminated histiocytosis X (Langerhans cell histiocytosis) with fatal course in an adult].
Histiocytosis X is very rare in late adulthood, usually taking a benign course with a good prognosis. A 56-year-old patient developed acute disseminated histiocytosis X with multiorgan involvement. Despite extensive diagnostic work-up the diagnosis could only be established on the grounds of a skin biopsy. Histiocytosis X cells are S 100 protein-positive and express the membrane antigens CD 1, CD 4 and HLA-DR. Electron microscopy reveals Birbeck granules. On static cytophotometry, histiocytosis X cells exhibited normal DNS content without aneuploidy. One year after the onset of disease the patient's condition deteriorated rapidly despite aggressive polychemotherapy (CHOP), and he died of multiorgan failure due to histiocytosis X.
Klinik für Dermatologie und Venerologie, Medizinischen Universität zu Lübeck.
M
M
Winzer
R
R
Krech
W
W
Früchtnicht
H H
HH
Wolff
D000208:Acute Disease; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001921:Brain / Q000473:pathology; D003520:Cyclophosphamide / Q000008:administration & dosage; D003592:Cytophotometry; D004317:Doxorubicin / Q000008:administration & dosage; D006646:Histiocytosis, Langerhans-Cell / Q000150:complications / Q000188:drug therapy / Q000473:pathology*; D006801:Humans; D007801:Langerhans Cells / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009102:Multiple Organ Failure / Q000209:etiology; D011241:Prednisone / Q000008:administration & dosage; D012867:Skin / Q000473:pathology; D014750:Vincristine / Q000008:administration & dosage
D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone
Akute disseminierte Histiozytosis X (Langerhans-Zell-Histiozytose) mit tödlichem Verlauf bei einem Erwachsenen.