This study is a prospective single-arm clinical study, focusing on Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis and Chronic Active Epstein-Barr Virus Infection with only and mainly B lymphocytes of EBV infection, to evaluate the clinical efficacy of Rituximab in the treatment of EBV-HLH and CAEBV.
Study Type
INTERVENTIONAL
Allocation
NA
Purpose
TREATMENT
Masking
NONE
Enrollment
30
Rituximab 375mg/m2. This regimen was repeated after 1 week. A total of 2-4 courses of treatment.(After two courses of treatment, EBV-DNA turned negative, no need to apply again. If EBV-DNA is still positive after two courses of treatment, 2 courses of treatment are applied again).
Zhao Wang
Beijing, China
RECRUITINGEBV-DNA
Treatment effectiveness is defined: EBV-DNA copies/ml in peripheral blood turns negative, and the involved tissues (such as lymph nodes, bone marrow, skin, etc.) are negative in EBER test or the EBV copy number has decreased by more than 2 orders of magnitude, but it is still positive.
Time frame: Change from before and 2,4,6 and 8 weeks after initiating Rituximab monotherapy
EBV-HLH Evaluation of treatment response
A complete response was defined as normalization of all of the quantifiable symptoms and laboratory markers of HLH, including levels of sCD25, ferritin, and triglyceride; hemoglobin; neutrophil counts; platelet counts; and alanine aminotransferase (ALT). A partial response was defined as at least a 25% improvement in 2 or more quantifiable symptoms and laboratory markers as follows: sCD25 response was\>1.5-fold decreased; ferritin and triglyceride decreased at least 25%; for patients with an initial neutrophil count of\<0.5 ×109/L, a response was defined as an increase by at least 100% to\>0.5×109/L; for patients with a neutrophil count of 0.5 to 2.0× 109/L, an increase by at least 100% to \>2.0 × 109/L was considered a response; and for patients with ALT \>400 U/L,response was defined as an ALT decrease of at least 50%.
Time frame: Change from before and 2,4,6 and 8 weeks after initiating Rituximab monotherapy
Progression Free Survival
from date of inclusion to date of progression, relapse, or death from any cause
Time frame: 6 months
Adverse events
Adverse events including myelosuppression, infection, hemorrhage
Time frame: 6 months
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