Nivolumab in Treating Patients With Primary Myelofibrosis, Post-Essential Thrombocythemia Myelofibrosis, or Post-Polycythemia Vera Myelofibrosis
Condition: Hepatomegaly; Myelofibrosis Transformation in Essential Thrombocythemia; Polycythemia Vera, Post-Polycythemic Myelofibrosis Phase; Primary Myelofibrosis; Splenomegaly
MeSH terms: Polycythemia Vera; Hepatomegaly; Primary Myelofibrosis; Polycythemia; Thrombocytosis; Thrombocythemia, Essential; Splenomegaly
Intervention: Laboratory Biomarker Analysis; Nivolumab; Quality-of-Life Assessment
Intervention description: Correlative studies; Given IV; Ancillary studies
Intervention MeSH terms: Nivolumab
Phase: Phase 2
Study type: Interventional
Primary purpose: Treatment
Intervention model: Single Group Assignment
Masking: None (Open Label)
Enrollment: 8.0
Sex: All
Minimum age: 18 Years
Healthy volunteers: No

Summary:

      This phase II trial studies how well nivolumab works in treating patients with primary
      myelofibrosis, post-essential thrombocythemia myelofibrosis, or post-polycythemia vera
      myelofibrosis. Immunotherapy with monoclonal antibodies, such as nivolumab, may help the
      body's immune system attack the cancer, and may interfere with the ability of tumor cells to
      grow and spread.
    

Eligibility criteria:

        Inclusion Criteria:

          -  Diagnosis of MF (either primary or post essential thrombocythemia/polycythemia vera)
             requiring therapy, including those previously treated and relapsed or refractory, or
             if newly diagnosed, with intermediate-1 or -2 or high risk according to International
             Prognostic Scoring System (IPSS)

          -  Previously treated with ruxolitinib (unless not a good candidate for ruxolitinib
             therapy in the judgment of treating physician)

          -  Palpable splenomegaly or hepatomegaly of more than or equal to 5 cm below left or
             right, respectively, costal margin on physical exam

          -  Understanding and voluntary signing an institutional review board (IRB)-approved
             informed consent form

          -  No prior history of immune checkpoint modulator therapy

          -  Disease-free of other malignancies

          -  Eastern Cooperative Oncology Group (ECOG) performance status 0 to 2

          -  Negative pregnancy test in females of childbearing potential (FCBP); male patients
             with female partners of child-bearing potential and female patients of childbearing
             potential are required to use two forms of acceptable contraception, including one
             barrier method, during their participation in the study and for 23 weeks (for females)
             or 31 weeks (for males) following the last dose of study medication; acceptable forms
             of contraception include 1 highly effective method such as an intrauterine device
             (IUD), hormonal (birth control pills, injections, or implants), tubal ligation, or
             partner's vasectomy and at least 1 additional approved barrier method such as a latex
             condom, diaphragm, or cervical cap plus spermicide; female patients of childbearing
             potential must not be breast-feeding or planning to breast feed and must have a
             negative pregnancy test within 24 hours of the first study treatment

          -  Direct bilirubin equal to or less than 1.5 x upper limit of normal (ULN)

          -  Serum creatinine equal to or less than 1.5 x ULN

          -  Aspartate aminotransferase (AST) (serum glutamic oxaloacetic transaminase [SGOT]) and
             alanine aminotransferase (ALT) (serum glutamate pyruvate transaminase [SGPT]) equal to
             or less than 2.5 x ULN (unless considered to be related to MF or patient has known
             history of Gilberts, in which case it must be equal to or less than 5 x ULN)

        Exclusion Criteria:

          -  Use of any other standard or experimental therapy within 14 days of starting study
             therapy

          -  Lack of recovery from all toxicity from previous therapy to grade 1 or baseline

          -  Any concurrent severe and/or uncontrolled medical conditions that could increase the
             patient's risk for toxicity while in the study or that could confound discrimination
             between disease- and study treatment-related toxicities

          -  Documented history of a cerebral vascular event (stroke or transient ischemic attack),
             unstable angina, myocardial infarction, or cardiac symptoms consistent with New York
             Heart Association class III-IV within 6 months prior to their first dose of the study
             drugs

          -  Patients who are currently receiving chronic (> 14 days) treatment with
             corticosteroids at a dose equal to or more than 10 mg of prednisone (or its
             glucocorticoid equivalent) per day, or any other chronic immunosuppressive treatment
             that cannot be discontinued prior to starting study drug

          -  Patients with autoimmune diseases are excluded: patients with a history of
             inflammatory bowel disease (including Crohn's disease and ulcerative colitis) are
             excluded from this study as are patients with a history of autoimmune disease (e.g.,
             rheumatoid arthritis, systemic progressive sclerosis [scleroderma], systemic lupus
             erythematosus, autoimmune vasculitis)

          -  Pregnant or breast feeding (lactating) women, where pregnancy is defined as the state
             of a female after conception and until the termination of gestation, confirmed by a
             positive beta-human chorionic gonadotropin (HCG) laboratory test

          -  Known positive for human immunodeficiency virus (HIV) or infectious hepatitis, type A,
             B or C

          -  The use of dietary supplements or herbal medications within 7 days of starting study
             therapy