Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement): lines of therapy
5 standard-of-care settings across 4 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.
| Line | All comers | HR-positive |
|---|---|---|
| Screening, prevention and diagnosis | 1 | · |
| Advanced, first line | 1 | · |
| Second line | 1 | · |
| Other settings | 1 | 1 |
Screening, prevention and diagnosis
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Diagnosis and staging | Biopsy with BRAF testing; blood count, liver tests, coagulation, abdominal ultrasound, skeletal survey or whole-body MRI, pituitary assessment. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 95 |
Advanced, first line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | First line | Vinblastine and prednisone induction for six to twelve weeks, then continuation to twelve months in total (LCH-III); mercaptopurine added for risk-organ disease in some protocols. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 94 |
Second line
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Non-response at week six or risk-organ progression | Switch to cytarabine or cladribine; intensive cladribine-cytarabine for refractory risk-organ disease; BRAF inhibitor (vemurafenib, dabrafenib) for BRAF V600E-mutant disease; reduced-intensity allogeneic transplantation in selected cases. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 92 |
Other settings
| Subgroup | Setting | Approach | Products and trials | Guideline | Evidence |
|---|---|---|---|---|---|
| All comers | Reactivation | Repeat vinblastine-prednisone or cytarabine; targeted therapy for repeated reactivation; enrolment in LCH-IV. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 83 | |
| HR-positive | Long-term follow-up | Endocrine, neurological, hearing, hepatic and orthopaedic surveillance for late effects through a survivorship programme. | Histiocyte Society evaluation and treatment guidelines; NCI PDQ | 58 |
Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.