Pulmonary Langerhans cell histiocytosis (PLCH) is a rare condition with a well-defined first-line management approach. The clinical goal is complete remission, and intervention at this stage is focused on a specific modifiable exposure that drives disease activity.
This protocol addresses first-line management of PLCH. The primary driver of disease in the majority of patients is an inhalation exposure — addressing this exposure is central to the therapeutic approach and can, on its own, lead to complete remission.
In patients who also present with wheezing and moderate airflow obstruction, additional inhaled pharmacological therapy may be considered alongside the primary intervention.
Management centers on complete elimination of all relevant inhalation exposures. For eligible patients with obstructive respiratory features, a specific class of inhaled therapy may be added — the full criteria, options, and sequencing are in the complete protocol.
The target is complete remission of pulmonary LCH. For many patients, addressing the primary inhalation exposure is the only intervention required to achieve this outcome.
DOI: 10.1182/blood.2021014343