Langerhans Cell Histiocytosis
Expert management of LCH, a rare disorder involving abnormal proliferation of Langerhans cells.
Langerhans Cell Histiocytosis
Hematologic Malignancies
Overview
Expert management of LCH, a rare disorder involving abnormal proliferation of Langerhans cells.
When to Consult
Upon diagnosis of LCH or suspicious biopsy findings.
What to Bring
Biopsy reports, imaging scans (X-ray, CT, MRI), and blood test results.
Risk Factors
Causes
Treatment Options
Observation
Watchful waiting for single-system, unifocal disease that may resolve spontaneously. Appropriate for isolated skin or bone lesions without organ dysfunction, with close monitoring for progression.
Surgery
Surgical curettage or excision for isolated bone lesions. Complete surgical removal may be curative for single-site disease, particularly in accessible locations like the skull or long bones.
Chemotherapy
Multi-agent chemotherapy (vinblastine, prednisone, methotrexate, 6-mercaptopurine) for multisystem disease or high-risk patients. Treatment duration varies based on disease extent and response, typically 6-12 months.
Targeted Therapy
BRAF inhibitors (vemurafenib, dabrafenib) for BRAF-mutated LCH, and MEK inhibitors for MAP2K1-mutated cases. Precision medicine approach based on genetic mutations found in LCH cells.
Immunotherapy
Immune checkpoint inhibitors and other immunomodulatory agents under investigation for refractory disease. Helps restore immune system function to control abnormal Langerhans cell proliferation.
Supportive Care
Management of organ dysfunction including diabetes insipidus (hormone replacement), skin care, pain management, and treatment of infections. Essential component of comprehensive care for all LCH patients.
Need Treatment?
Schedule a consultation to discuss treatment options for Langerhans Cell Histiocytosis.