Unrelated Cord Blood Transplantation for EBV-associated Lymphoproliferative Disorders

Korábbi kezelés

• History of another primary malignancy within 5 years prior to the first treatment.
• Planned to receive other types of hematopoietic stem cell transplantation during the study period.
• Major surgery within 4 weeks prior to the first treatment, or anticipated need for major surgery during the study.
• Known allergy to any component of the investigational drug(s), or a history of severe allergies.

Más betegség

• Grade II or higher cardiac disease according to the New York Heart Association (NYHA) classification.
• History of organ transplantation.
• Active infections, including:
• Active hepatitis B or hepatitis C.
• History of syphilis.
• History of severe psychiatric illness or substance abuse.
• Uncontrolled infection.
• Active major visceral hemorrhage.

Laborértékek

• Positive serum HIV antigen or antibody.

Egyéb

• Uncorrected thyroid dysfunction.
• Pregnant or breastfeeding women.
• High risk of complications, such as:
• Patients deemed unable to comply with the study procedures and/or follow-up requirements.
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• All subjects must meet all of the following criteria:
• Age range: 14 to 80 years old, inclusive.
• Diagnosed with an EBV-associated lymphoproliferative disease (including Chronic Active EBV Infection, EBV-associated Hemophagocytic Lymphohistiocytosis, and EBV-associated Lymphoma).
• Meet at least one of the following disease-specific criteria (see below).
• ECOG Performance Status score of 0-2 (or Lansky Play-Performance score ≥60 for children).
• Expected survival of ≥3 months.
• Signed informed consent form (for minors, consent must be provided by a legal guardian).
• Disease-Specific Inclusion Criteria:
• CAEBV Patients: Patients diagnosed with CAEBV according to the 2016 revised WHO classification criteria, who also meet all of the following:
• Persistent or recurrent infectious mononucleosis-like symptoms for more than 3 months.
• Elevated EBV-DNA load in peripheral blood or involved tissues (peripheral blood EBV-DNA > 500 copies/ml).
• Detection of EBV in T or NK cells (via EBER in situ hybridization or LMP1 immunohistochemistry).
• Exclusion of other known immunodeficiency disorders, autoimmune diseases, or neoplastic diseases.
• EBV-HLH Patients: Meet ≥5 of the HLH-2004 diagnostic criteria:
• Persistent or recurrent infectious mononucleosis-like symptoms for more than 3 months.
• Fever ≥38.5°C.
• Splenomegaly.
• Cytopenias (affecting ≥2 of 3 lineages):
• Hb <90 g/L
• PLT <100 × 10⁹/L
• ANC <1.0 × 10⁹/L
• Hypertriglyceridemia (>3 mmol/L) and/or hypofibrinogenemia (<1.5 g/L).
• Serum ferritin >500 μg/L.
• sCD25 >2400 U/mL.
• Decreased or absent NK cell activity.
• Evidence of hemophagocytosis in bone marrow, spleen, or lymph nodes.
• EBV-associated Lymphoma Patients:
• Pathologically confirmed diagnosis according to WHO criteria.
• EBV-positive (EBER+).
• Failure of or relapse after first-line therapy.