म्यूकोक्यूटेनियस लैंगरहैंस सेल हिस्टियोसाइटोसिस: जब स्थानीय चिकित्सा विफल हो जाए तो उपचार

References

DOI: 10.1182/blood.2021014343

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

Among systemic treatment options for LCH, chemotherapy using either cladribine or cytarabine is preferred because of relatively high overall response rates and the potential for long-term remissions with limited cycles of treatment.

Vinblastine ± prednisone is a reasonable alternative, although it is not the preferred choice of therapy in adults given the high risk of relapse and potential for peripheral neuropathy.

Any degree of improvement in clinical or radiographic findings is considered a response.

For patients with abnormal FDG-PET/CT findings at diagnosis, the first response assessment should occur in 2 to 3 months of initiation of therapy and every 3 to 6 months thereafter.