chronic active Epstein-Barr virus infection CAEBV
Conditions
Interventions
ruxolitinib tablet
Sponsors
St. Marianna University School of Medicine
Eligibility
Sex/Gender
All
Inclusion criteria
Inclusion criteria: Subjects diagnosed as CAEBV under "Guidelines for Clinical Practice for Chronic Active EB Virus Infection and Related Diseases 2016" and matched all criteria below; (1) with >= 1x10^2.5 copies/mcg DNA of EBV DNA load in peripheral blood measured within 8 weeks prior to registration (only the central measuring institution are acceptable). (2) with confirmed EBV infection on T- or NK-cells in tissue lesions or in peripheral blood. (3) with >= 3 months of either continuous or intermittent systemic inflammatory symptoms: persistent fever, liver dysfunction, multiple lymphadenopathy, progressive skin lesions, vasculitis, neuritis, uveitis, enteritis etc. However, subjects with hypersensitivity to mosquito bites and Hydroa Vacciniforme-like eruptions without sustained systemic inflammatory symptoms are not diagnosed with CAEBV and excluded. (4) negative for the following diseases: Congenital or acquired immunodeficiency, autoimmune/inflammatory disease, connective tissue disease, malignant lymphoma, leukemia, iatrogenic immunodeficiency. (5) having active disease during observation period: having fever and/or liver dysfunction defined below. 1) having fever without any other causes (>= 37.5 deg. of axillary temperature) in >= 2 days during 5-day observation. 2) having liver dysfunction defined as increase of ALT levels to two times higher than the upper limit of normal on at least two consecutive occasions. ALT should be measured twice within 28 days before registration, and both should match the criteria. The latter measurement should be performed within 7 days before registration. (6) with life expectancy of >= 3 months. (7) fulfilling the following cell count in peripheral blood in the latest examination within 7 days before registration. 1) >= 500/microL of Neutrophil 2) >= 50,000/microL of Platelet (8) 13 years of age or older at obtaining informed consent. (9) who obtained written informed consent from the subjects themselves or legal representative.
Exclusion criteria
Exclusion criteria: Subjects matched any of the followings are excluded; (1) pathological or clinical lymphoid neoplasm derived from EBV-infected T- or NK-cells of CAEBV. (2) anti-VCA-IgM Ab positive (difficult to distinguish from infectious mononucleosis). (3) with history taking JAK 1/2 inhibitors. (4) with malignant neoplasm or history of them within the last 5 years, though cervical intraepithelial carcinoma, basal cell carcinoma or squamous cell carcinoma of the skin treated properly, fully resected gastric intramucosal carcinoma can be accepted. (5) with unstoppable treatment with >200 mg/day fluconazole or strong CYP3A4 inducer (rifampicin, St. John's wart etc) at the beginning of study drug administration (excl. topical). (6) with infectious diseases requiring systemic antibiotics or antivirals. (7) with tuberculosis or HIV-positive. (8) with active hepatitis matching any of the following within 84 days before registration; -HBs Ag-positive -HBc Ab-positive or HBs Ab-positive with >= 20 IU/mL (1.3 Log IU/mL, the same below) of peripheral blood HBV DNA load HBs Ag-negative, HBs Ab-positive, HBc Ab-negative and history of the vaccination for HBV are treated as uninfected ones. -HCV Ab-positive (except when confirmed HCV-RNA-negative). * There are some conditions for judgment of HBV infection. (9) with cardiac disease of NYHA class IV. (10) having been treated with other anticancer drug (e.g. etoposide) within 14 days prior to registration. (11) with history of hypersensitivity to ingredients of ruxolitinib tablet. (12) having been treated with another investigational medication within 12 weeks prior to registration. (13) female who are pregnant, have possibility of pregnancy, or are currently breastfeeding. (14) difficult to take oral tablet. (15) judged unsuitable for participation by investigator or sub-investigator.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| Percentage of participants with complete response (CR) at week 8 or early termination. | — |
Secondary
| Measure | Time frame |
|---|---|
| Percentage of participants with complete response (CR) at week 4. Overall response rate at week 8 or early termination. Adverse event. Plasma drug concentration. Epstein-Barr virus DNA quantification . Plasma cytokine concentration. | — |
Countries
Japan
Contacts
Public ContactYuki Ohta
St. Marianna University School of Medicine Center for Clinical and Translational Science
Outcome results
None listed