neurofibromatosis
Conditions
Interventions
Case series:None
Sponsors
Shanghai Sixth People's Hospital
Eligibility
Sex/Gender
All
Age
13 Years to No maximum
Inclusion criteria
Inclusion criteria: Patients with local advanced NF2 with local neurological damage are not available. 1)> 12 years old, both men and women are available; 2) ECOG score 0-1 points; 3) Patients with NF2 after clinical or genetic diagnosis of NF2; 4) Single/multiple neurofibroma disease that is difficult to treat surgery, or patients with recurrence after surgery; 5) At least one measuring lesion is available according to the Evaluation Standard (RECIST1.1) according to the physical tumor; 6) The expected survival period is =3 months; 7) The function of the main organs and bone marrow is normal, and the following requirements are met: a. Blood -red protein =90 g/L; (without blood transfusion within 14 days) b. The absolute count of neutral granulocytes =1.5 × 109/L; c. Platelet count =90 × 109/L; d. Total bilirubin = 1.5 times the upper limit of normal value (ULN) e. alanine aminotransferase (ALT) and aspartate aminotransferase (AST) = 2.5 times ULN; if liver metastasis, ALT and AST = 5 times ULN; f. creatinine = 1.5 times ULN; g. Left Ventricular Ejection Fraction (LVEF) =50%; QTC men <450ms, women <470ms; 8) Patients who have not received anticoagulant therapy original time international standardization ratio (INR) = 1.5, and some coagulation activation time (APTT) = 1.5 times ULN. Patients who receive a full or gastrointestinal anticoagulant drug treatment only need to stabilize the dosage of anticoagulant drugs before entering clinical research for at least 2 weeks, and the results of the coagulation testing test are within the scope of local treatment; 9) Women of childbearing age must conduct pregnancy tests (serum or urine) within 14 days before entering the group, and voluntarily use appropriate methods to use appropriate methods within 3 months of observation and last time after observation and last time after the study of drugs; for men, men should be for men. Solidarity or consent to adopt the appropriate method of contraception within 3 months after the observation period and the last time the study drugs are given; 10) Recovery from previous treatment: According to the NCI-CTC AE 5.0 version, the toxicity of the preliminary treatment has been restored to level =1 (if there is surgery, the wound has completely healed) 11) Patients voluntarily participate in and signed the informed consent form (or the signing of the legal agent). It is expected that the compliance is good and can cooperate with the research according to the requirements of the plan.
Exclusion criteria
Exclusion criteria: 1) I have received the following treatment or drugs before the first study and treatment: a. Excessive surgery within 28 days before the first study of drug treatment (the organizational biopsy required for diagnosis is allowed); b. The first study of the drug treatment for the first time or planned to be vaccinated during the study of the drug within 60 days after the research period and the end of the drug treatment; c. For the first time the first study of drug treatment, there were any methods of anti -tumor therapy, including radiotherapy, chemotherapy, molecular targeted therapy and immunotherapy, or participated in another intervention clinical trial; , No more than 4 weeks of medication or 5 semi -half -periods from the last medication; 2) There are third gap accumulation (such as a large amount of chest water or ascites) with clinical symptoms, unable to control through drainage or other methods; 3) Even after drug treatment, hypertension is still poorly controlled (the contraction pressure continues to rise =150mmHg or diastolic blood pressure =100mmHg) 4) Diseases of clinical symptoms or diseases that have unsuitable hearts, such as (1) Nyha II and above heart failure; (2) unstable angina pectoris; (3) myocardial infarction occur within 1 year; (4) clinical significance that has clinical significance Patients who need clinical intervention at room or ventricular arrhythmia; 5) Human immune defect virus (HIV) infection or is known to have acquired immune defect syndrome (AIDS), unprecedented active hepatitis (hepatitis B, defined as hepatitis B virus surface antigen [HBSAG] test results are positive, positive, HBV-DNA = 500 IU/ML and abnormal liver function; hepatitis C is defined as hepatitis C antibody [HCV-AB] positive, HCV-RNA higher than the lower limit of the analysis method and abnormal liver function) or combined with hepatitis B and hepatitis C infection together Items 6) History of any active autoimmune disease or the history of autoimmune diseases (such as inter -pneumonia, uveitis, enteritis, enteritis, hepatitis, pituitary, vasculitis, myocarditis, nephritis, hyperthyroidism, hypothyroidism (hormone replacement alternative After treatment),); Children's asthma has been completely alleviated and no intervention or vitiligo can be included after adults. Patients with bronchiectasis require medical intervention in medical intervention. 7) A concurrent weight infection within 2 weeks before the first medication (such as: intravenous antibiotics, antifungal or antiviral drugs), or fever of unknown reasons before/before the screening period/first administration> 38.5 ° C; 8) Dynamic/venous thrombosis incidents occurred within 6 months before joining the group, such as cerebrovascular accidents (including temporary ischemia, cerebral hemorrhage, cerebral infarction), deep venous thrombosis, pulmonary embolism, etc.; 9) In the past 5 years, it has suffered from or accompanied by other systematic malignant tumors (except for cured skin basal cell carcinoma and cervical in situ cancer and ovarian cancer); 10) Known who is allergic to any test drug or its auxiliary materials; 11) Patients with pregnancy and lactation, patients with reproductive ability are unwilling to take effective contraceptive measures; 12) There are clear history of nerve or mental disorders, including epilepsy and dementia; 13) The metastasis of the activated central nervous system (CNS) that is known or symptomatic or symptomatic, manifested as the gr
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Safety; | — |
Secondary
| Measure | Time frame |
|---|---|
| Objective relief rate (ORR);non-progressive survival period (PFS);clinical benefit rate (CBR); relieving duration (DOR); | — |
Countries
China
Contacts
Public ContactYin Shankai
Shanghai Sixth People's Hospital
Outcome results
None listed