Single-System Pulmonary LCH in an Adult Smoker When Systemic Therapy Has Not Improved Lung Function
This protocol addresses adults with isolated lung involvement from Langerhans cell histiocytosis — a predominantly smoking-related presentation — in whom a prior systemic treatment line did not achieve the intended improvement in lung function.
Clinical scenario
Single-system pulmonary Langerhans cell histiocytosis with isolated lung involvement in an adult smoker. This form is predominantly smoking-related. Cessation of smoking, vaping, and inhalation of marijuana or other substances is recommended as first-line therapy before systemic escalation.
Previous line — failure condition triggering this protocol
The prior treatment line used systemic therapy with cladribine as the preferred agent (vinblastine has limited efficacy in pulmonary LCH). This protocol is indicated when that approach has not resulted in improved lung function on pulmonary function tests.
Next-line approach (partial overview)
For patients with advanced single-system pulmonary LCH that is refractory to systemic treatments, or for those who are ineligible for them, the approach shifts toward a non-pharmacological referral pathway. The full structured protocol specifies the criteria and the pathway in detail.
References
DOI: 10.1182/blood.2021014343
Single-system pulmonary | Isolated lung involvement (predominantly smoking related). Cessation of smoking, vaping, inhalation of marijuana or other substances is recommended as first-line therapy for single-system PLCH.
For patients who develop advanced single-system PLCH refractory to or ineligible for systemic treatments, lung transplantation referral should be undertaken. Patients with advanced single-system PLCH may be candidates for lung transplantation, although the disease may relapse in the transplanted lungs.
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