Treatment of Pulmonary Langerhans Cell Histiocytosis After Cladribine Has Failed to Achieve a Response

When a patient with single-system pulmonary Langerhans cell histiocytosis (PLCH) does not achieve even a minimal response to systemic therapy, the clinical pathway moves to a next-line approach. This protocol applies to that escalation.

Prior therapy did not meet its target

Failure Condition — Previous Line

The preceding treatment consisted of systemic chemotherapy with cladribine, the preferred systemic agent for single-system PLCH. The threshold for response was any degree of improvement in clinical or radiographic findings of the lungs. When that threshold is not reached, escalation to this protocol is indicated.

Next Step — Partial Overview

For patients with advanced single-system PLCH who are refractory to or ineligible for systemic treatments, the protocol involves referral for lung transplantation.

Complete eligibility criteria, patient selection considerations, and follow-up details are available in the full structured protocol below.

Instant Access to Structured Evidence-Based Regimens

References

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.

DOI: 10.1182/blood.2021014343

View source ↗