Evaluation of Efficacy and Tolerance of Cladribine in Symptomatic Pulmonary Langerhans Cell Histiocytosis

• Status: Active, not recruiting
• Trial end date: 2022-04-01
• Target enrollment: 0
• Participant gender: All

Summary

ECLA is a phase II, multicenter study testing sub cutaneous cladribine 0.1mg/kg/j during 5 days, administrated every month for 4 courses, in symptomatic adult patients with pulmonary Langerhans cell histiocytosis and impairment of lung function patients.

• Phase: Phase 2
• Accepts Healthy Volunteers?: No

Details

• Lead Sponsor: Assistance Publique - Hôpitaux de Paris
• Treatments: Cladribine

Criteria

Inclusion Criteria:

• Age 16 to 55 yr

• Histologically proven pulmonary Langerhans cell histiocytosis ( patients with presumptive diagnosis whose lung function precludes lung biopsy may be included after revision of their medical record at the national reference center for Langerhans cell histiocytosis)

• Symptomatic pulmonary Langerhans cell histiocytosis (NYHA dyspnea class ≥2) with:

• irreversible airflow obstruction (FEV1/FVC<70%) with postbronchodilator FEV1 comprised between 30 and 70% of predicted

• and/or decrease ≥15% in FEV1, FVC or DLCO as compared to baselines values in the year preceding the inclusion

• Signed written informed consent

Exclusion Criteria:

• Women at childbearing age without adequate contraception or wishing breastfeeding

• Male without adequate contraception during the study

• Dyspnea due to severe pulmonary arterial hypertension (PAP≥35mmHg) confirmed by cardiac right catheterism

• Previous malignancy

• Current infectious disease

• Renal failure

• Liver failure

• Severe alteration of lung

• Hematologic disease unrelated to Langerhans cell histiocytosis

• Epilepsy

• Hepatic, spleen or hematology involvement by Langerhans cell histiocytosis

• Pneumothorax within a month previously to inclusion

• Previous treatment with cladribine

• Contra indication to the use of cladribine

• Previous myelosuppressive treatment

• Simultaneous participation to another interventional clinical trial