LCH : Treatment
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Treatment of Langerhans Cell Histiocytosis (LCH): There are no standard therapies for LCH due to its heterogenous presentation. Treatment approaches depend upon the organ involved and the extent of the disease.
Most solitary bone lesions are treated by curettage. Isolated skin lesions in single system LCH are resected. For unresectable lesions, low-dose radiation has been used in the past. However, it is not recommended due to increased risk for secondary malignancies, especially in young children. Intralesional corticosteroid injections are effective in some cases. Localized LCH lesions may spontaneously regress.
Patients with disseminated disease and high-risk organ involvement (liver, spleen, bone marrow) receive multiagent chemotherapy. Diabetes insipidus secondary to hypothalamic-pituitary mass lesion requires lifelong hormone replacement therapy.
TARGETED THERAPIES: BRAF inhibitors such as vemurafenib have shown good results; however, the disease relapses upon discontinuation of treatment and some patients have developed treatment-related squamous cell carcinoma and KRAS-mutant papillary thyroid carcinoma. Dabrafenib (another BRAF inhibitor) has been tried with some success. Patients with MEK1 mutation have undergone trials with trametinib - a MEK1/2 inhibitor.
This image of LCH of skin shows a uniform population of Langerhans cells (LC) filling up the papillary dermis
Most solitary bone lesions are treated by curettage. Isolated skin lesions in single system LCH are resected. For unresectable lesions, low-dose radiation has been used in the past. However, it is not recommended due to increased risk for secondary malignancies, especially in young children. Intralesional corticosteroid injections are effective in some cases. Localized LCH lesions may spontaneously regress.
Patients with disseminated disease and high-risk organ involvement (liver, spleen, bone marrow) receive multiagent chemotherapy. Diabetes insipidus secondary to hypothalamic-pituitary mass lesion requires lifelong hormone replacement therapy.
TARGETED THERAPIES: BRAF inhibitors such as vemurafenib have shown good results; however, the disease relapses upon discontinuation of treatment and some patients have developed treatment-related squamous cell carcinoma and KRAS-mutant papillary thyroid carcinoma. Dabrafenib (another BRAF inhibitor) has been tried with some success. Patients with MEK1 mutation have undergone trials with trametinib - a MEK1/2 inhibitor.
This image of LCH of skin shows a uniform population of Langerhans cells (LC) filling up the papillary dermis