Langerhans' cell histiocytosis in adults: a clinical and therapeutic analysis of 11 patients from a single institution.
BACKGROUND
Langerhans' cell histiocytosis (LCH) is a rare disorder of uncertain etiology, characterized by a wide clinical spectrum and varied behavior.


METHODS
This retrospective study analyzed 11 adult patients with a diagnosis of LCH observed at the study institution between April 1988 and March 1993.


RESULTS
Based on the sites and extent of disease at diagnosis, patients were divided into four categories. Group A was comprised of four patients with unifocal bone disease who had surgical curettage. At last follow-up only 1 patient was in continuous complete response (CCR) at 29+ months. The other 3 patients recurred at 3, 12, and 30 months, respectively, after surgery and at last follow-up were found to be in CR at 16+, 48+, and 124+ months, respectively, after therapy with vinblastine (VBL) and high dose methylprednisolone (HDMP). Group B was comprised of three patients with multifocal bone disease. Two of these patients received VBL + HDMP; at last follow-up, 1 patient was in CCR 8 months after completion of therapy, and the other developed progressive disease 11 months later. The third patient was treated with interferon (IFN) and at last follow-up was in CCR at 35+ months. Group C was comprised of 2 patients with bone and visceral disease who were treated with etoposide (VP-16) + HDMP; at last follow-up, 1 patient was in CCR at 42+ months and the other patient, who had isolated vulvar recurrence 16 months later, was in CR with treatment with local IFN. Group D was comprised of two patients with lung and lymph node involvement, one of whom was treated with VP-16 + HDMP and the other with cyclophosphamide, doxorubicin, vincristine, and prednisone; at last follow-up, both were in CCR at 30+ and 71+ months, respectively.


CONCLUSIONS
VBL + HDMP showed efficacy in patients with bone disease, in particular those treated for recurrent LCH after surgery. Therapy with VP-16 and HDMP was successfully employed in patients with visceral disease. IFN was effective both for localized disease and in patients with multiple bone lesions.
Giona
F
F
Department of Cellular Biotechnology and Hematology, University La Sapienza, Rome, Italy.
Caruso
R
R
Testi
A M
AM
Moleti
M L
ML
Malagnino
F
F
Martelli
M
M
Ruco
L
L
Giannetti
G P
GP
Annibali
S
S
Mandelli
F
F
D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001847:Bone Diseases / Q000628:therapy; D005767:Gastrointestinal Diseases / Q000628:therapy; D006646:Histiocytosis, Langerhans-Cell / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007372:Interferons / Q000627:therapeutic use; D008171:Lung Diseases / Q000628:therapy; D008206:Lymphatic Diseases / Q000628:therapy; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D012189:Retrospective Studies; D016879:Salvage Therapy; D016896:Treatment Outcome; D014747:Vinblastine / Q000627:therapeutic use
D014747:Vinblastine; D007372:Interferons; D008775:Methylprednisolone