Expert management of LCH, a rare disorder involving abnormal proliferation of Langerhans cells.
Hematologic Malignancies
Overview
LCH is a rare disorder with variable presentation from isolated bone lesions to multisystem disease. Treatment depends on extent of involvement. Many cases resolve spontaneously, while others require aggressive therapy.
When to Consult
Upon diagnosis of LCH, bone lesions, skin rash, diabetes insipidus, or suspicious biopsy findings.
What to Bring
Biopsy reports, imaging scans (X-ray, CT, MRI, bone scan), blood test results, BRAF mutation testing, and endocrine function tests.
Risk Factors
Causes
Treatment Options
Observation
Watchful waiting for isolated, asymptomatic lesions that may resolve spontaneously. Appropriate for single-system, low-risk disease.
Surgery
Curettage or excision for isolated bone lesions. Often curative for single-site disease. May be combined with intralesional steroid injection.
Chemotherapy
Vinblastine plus prednisone for multisystem disease. Standard first-line treatment. May include other agents (methotrexate, 6-mercaptopurine) for high-risk disease.
BRAF Inhibitors
Vemurafenib or dabrafenib for BRAF V600E-mutated LCH. Targeted therapy showing excellent results. Particularly effective for refractory or high-risk disease.
MEK Inhibitors
Trametinib or cobimetinib for MAPK pathway mutations. Alternative targeted therapy option.
Radiation Therapy
Low-dose radiation for isolated lesions not amenable to surgery. Particularly useful for bone lesions in critical locations.
Topical Therapy
Topical steroids or nitrogen mustard for skin-only LCH. Less invasive option for localized disease.
Hormone Replacement
Desmopressin for diabetes insipidus. Hormone replacement for other endocrine deficiencies. Supportive care for organ dysfunction.
Stem Cell Transplantation
Allogeneic stem cell transplant for severe, refractory multisystem disease. Reserved for high-risk cases not responding to standard therapy.