علاج كثرة المنسجات من خلايا لانغرهانس متعددة الأجهزة لدى البالغين

References

DOI: 10.1182/blood.2021014343

Multisystem | ≥2 organ/system involvement.

For multisystem LCH or extensive/refractory multifocal single-system LCH, systemic chemotherapy agents such as cladribine, cytarabine, or vinblastine + prednisone are recommended.

For disease that is relapsed or refractory following chemotherapy, it is reasonable to consider an alternate chemotherapy agent or a kinase inhibitor based on patient-specific factors and drug availability.

For LCH refractory to first-line treatment or with end-organ dysfunction (e.g., neurologic impairment, sclerosing cholangitis), alternate conventional treatment or targeted therapies (BRAF or MEK inhibitors) should be implemented.

Because most patients will require a dose reduction and lower doses of BRAF and MEK inhibitors are quite effective, it is reasonable to start at half of the dose currently used to treat patients with melanoma.

For initially FDG PET avid LCH, it is recommended to repeat an FDG PET-based imaging study for assessment of disease response after 2–3 mo of initiation of therapy, with subsequent imaging frequency tailored individually based on the specific clinical scenario.