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Pattern and course of single-system disease in Langerhans cell histiocytosis data from the DAL-HX 83- and 90-study
C Titgemeyer1, N Grois, M Minkov
1St. Anna Children's Hospital, Kinderspitalgasse 6, A-1090 Vienna, Austria.
Insights
Single-system Langerhans cell histiocytosis (LCH) typically affects bone and has a benign course. While reactivations are common, they rarely impact survival but can lead to permanent consequences.
Area of Science:
- Pediatric Oncology
- Histiocytosis Research
- Skeletal Diseases
Background:
- Single-system Langerhans cell histiocytosis (LCH) is the most frequent presentation, often involving bone, skin, or lymph nodes.
- The clinical presentation and disease course of SS-LCH are highly variable.
Purpose of the Study:
- To analyze the clinical presentation, treatment, and outcomes of single-system Langerhans cell histiocytosis (SS-LCH).
- To evaluate the diagnostic superiority of radiographic skeletal surveys over bone scans for detecting bone lesions in SS-LCH.
Main Methods:
- 170 patients with SS-LCH were registered in the DAL-HX 83/90 studies, diagnosed using uniform criteria.
- Patients were followed using a standardized schedule, and treatment modalities included surgery, irradiation, steroid instillation, and chemotherapy.
Main Results:
- Bone lesions were most common (87%), with radiographic skeletal surveys outperforming bone scans in detection (97% vs. 82%).
- 81% of patients remained disease-free after initial therapy; 18% experienced skeletal reactivations. Permanent consequences, mainly orthopedic, affected 25% of patients.
- Fatality was rare, occurring only in an infant who progressed to multi-system disease. Endocrine dysfunctions (diabetes insipidus, pituitary dysfunction) occurred in 3% and 2% respectively.
Conclusions:
- Single-system LCH generally follows a benign course with no impact on survival from skeletal reactivations.
- Reactivations primarily affect morbidity, with permanent consequences often linked to the site of disease activity.
- Early identification and management of SS-LCH are crucial for minimizing long-term sequelae.
Background:
Single-system (SS) disease is the most common presentation in Langerhans cell histiocytosis (LCH) with a heterogenous clinical picture and course. Mostly bone and rarely skin or lymph nodes are involved.
Procedure:
One hundred and seventy patients with SS-LCH were registered in the DAL-HX 83/90 studies. They were diagnosed according to uniform diagnostic criteria and followed by a standardised schedule.
Results:
Single bone lesions were most common (68%), followed by multiple bone lesions (19%), isolated skin disease (11%), and isolated lymph node involvement (4 patients). In the detection of bone lesions radiographic skeletal survey proved to be superior to bone scan (97% vs. 82%). Treatment comprised surgery, irradiation and local instillation of steroids, and standardised chemotherapy for multifocal bone disease. After initial therapy 81% of the patients remained disease free. Reactivations restricted to the skeleton occurred in 18% of both unifocal and multifocal bone disease. Two skin patients had a chronic course. Fatality occurred only in one infant with skin disease who progressed to multi-system disease. Twenty-five percent of all patients developed permanent consequences, which were already present at diagnosis in about half of these patients and comprised mainly orthopedic problems related to lesional sites. Diabetes insipidus occurred in 3% and anterior pituitary dysfunction in 2% of the patients.
Conclusions:
The course in SS%LCH was benign. In bone disease reactivations remained restricted to the skeleton and did not influence survival. However, reactivations had an impact on morbidity, as permanent consequences were mostly related to the site of disease activity. Med Pediatr Oncol 2001;37:108-114.