Pulmonary tumor thrombtic microangiopathy Pulmonary hypertension, adenocarcinoma
Conditions
Interventions
Patients will receive 45mg of ponatinib once daily for 2 weeks.
C14.280.832
Tyrosine kinase inhibitor
Sponsors
Sato Kimi
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: 1) Patient diagnosed with pulmonary tumor thrombotic microangiopathy (PTTM) -Histological diagnosis of PTTM will be made by presence of the tumor cells within the lumen of pulmonary pre-capillary vessels, with fibrocellular intimal hyperplasia in lung biopsy. -Clinical diagnosis will be made when patient meet either (A)+(B) or (A)+(C) of following criteria. (A) primary pulmonary hypertension. (B) presence of malignant tumor. (C) perfusion defects along the pleural margins on lung perfusion scan. 2) Patient aged 18 years or more. 3) Patient or representative person who received detailed explanation and understands the study well is willing to participate in this study. 4) Hospitalized patient.
Exclusion criteria
Exclusion criteria: 1) Patient who will be considered as inappropriate to participate the study by principal investigator 2) Patient who received tyrosine kinase inhibitors. 3) Patient with fatal or severe adverse event within 24 hours after introduction of extracorporeal membrane oxygenation (ECMO).
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| The primary outcome will be composite endpoint of all cause death and severe vascular occlusive event. We will also evaluate the prevalence of grade 2 or higher adverse event (CTCAE v4) associated with ponatinib. | — |
Secondary
| Measure | Time frame |
|---|---|
| The secondary outcome will be the change from baseline in right heart catheterization parameters (mean pulmonary artery pressure, cardiac output, pulmonary vascular resistance), echocardiographic parameters (tricuspid regurgitant velocity, estimated pulmonary artery pressure, right ventricular size), WHO functional class, vital sign, or laboratory markers (BNP, VEGF, PDGF). We will also evaluate the proportion of successful weaning from ECMO and pharmacokinetics of ponatinib. | — |
Contacts
Public ContactKimi Sato
University of Tsukuba Hospital
Outcome results
None listed