Nintedanib Treatment in Unicentric Castleman Disease

Inclusion criteria

• Age ≥ (equal to or greater than) 18 years
• Written informed consent
• Biopsy-proven diagnosis of hyaline-vascular Unicentric Castleman disease
• Unresectable or partially resectable UCD lesion or surgery refusal
• Available oral route
• Affiliated to National French social security system (registered or being a beneficiary of such a scheme)
• Women of childbearing potential should be advised and agree to avoid becoming pregnant while receiving treatment and to use highly effective contraceptive methods at initiation of, during and at least 3 months after the last dose of treatment; pregnancy testing must be conducted prior to treatment and during treatment as appropriate; breast-feeding should be discontinued during treatment
• In male patients, with WOCBP partner(s), willingness to use adequate contraceptive measures to prevent his partner from becoming pregnant during the study, prior to administration of the first dose of study treatment until 3 months after the last dose of study treatment

Exclusion criteria

• Synchronous Follicular Dendritic Cell sarcoma
• Known hypersensitivity to nintedanib, soy or peanut or to any of the excipients of the experimental drug, or known hypersensitivity to the auxiliary drugs listed or to any of their excipients.
• For women of childbearing age: negative serum or urine pregnancy test at inclusion and confirmed each month during the study, up to 3 months after the last dose.
• Inability to obtain informed consent
• Patients under legal protection
• Liver transaminases (AST and/or ALT) >3N
• End-stage liver disease (Child B or C cirrhosis)
• End-stage renal failure (CrCl<30 mL/min)
• Severe hemorrhagic or thromboembolic events in the past 6 months
• Uncontrolled systemic illness such as chronic heart failure, unstable angina, hypertension; history of myocardial infarction or stroke or aneurysm
• Major injuries in the 10 days prior to start of the study, or Recent surgery with inadequate wound healing, or Abdominal surgery in the past 4 weeks.
• Severe pulmonary hypertension
• Bleeding risk, any of the following:

• Known genetic predisposition to bleeding.
• Patients who require

• Fibrinolysis, full-dose therapeutic anticoagulation (e.g. vitamin K antagonists, direct thrombin inhibitors, heparin, hirudin) 2. High dose antiplatelet therapy corresponding to a combination of two anti-platelet aggregation treatment (aspirin + an Inhibitor of P2Y12 receptor) 14. Contraindication to the experimental drug or auxiliary drugs listed 15. Patients under guardianship or curatorship and protected adults or unable to consent 16. Enrollment in another interventional study (ongoing at the time of inclusion)