Segmental Resection Combined With DEP Regimen for EBV-HLH Patients With Intestinal Involvement

Not yet recruiting

• Conditions: Hemophagocytic Lymphohistiocytosis; Epstein-Barr Virus; Bowel Resection
• Interventions: Other: Segmental bowel resection combined with the DEP regimen

• Registration Number: NCT06131489
• Lead Sponsor: Beijing Friendship Hospital

Brief Summary

EBV-HLH is a rare disease with high mortality, especially for those with intestinal involvement. In order to reduce disease burden and improve survival of these patients, we conduct a prospective observational study to explore the efficacy and safety of segmental resection combined with the DEP regimen.

Detailed Description

Epstein-Barr virus associated hemophagocytic lymphohistiocytosis is a rare disease with high mortality, especially for those with intestinal involvement. Some of them still died despite early control of the disease due to severe complications, such as gastrointestinal bleeding. In order to reduce disease burden and improve survival of these patients, we conduct a prospective observational study to explore the efficacy and safety of segmental resection combined with the DEP regimen.

Study Timeline

• Status Verified: 2023-09
• Study First Submitted: 2023-11-09
• Study First Submitted QC: 2023-11-09
• Last Update Submitted: 2023-11-09
• Study First Posted: 2023-11-14
• Last Update Posted: 2023-11-14
• Study Start: 2023-12-01
• Primary Completion: 2026-12-01
• Study Completion: 2027-01-01

Recruitment & Eligibility

• Status: NOT_YET_RECRUITING
• Sex: All
• Target Recruitment: 20

Inclusion Criteria

• Met HLH-2004 diagnostic criteria;
• EBV-DNA in the peripheral blood > 1000 copies/ml or EBV-encoded small RNA (EBER) detected in tissues;
• Imaging revealed severe intestinal lesions and biopsy confirmed EBV-encoded small RNA (EBER) in the tissues;
• Age >18 years old, gender is not limited;
• Estimated survival time > 1 month;
• Informed consent obtained.

Exclusion Criteria

• Active infections (viral, bacterial, fungal or parasitic);
• Diagosed with malignant tumos within 5 years；
• Uncontrolled symptoms or other diseases including, but not limited to, unstable angina pectoris, arrhythmia, respiratory failure, severe hepatic or renal insufficiency, severe coagulation dysfunction, mental illness, or other conditions deemed by the attending physician to be contraindications to surgery;
• Heart function above grade II (NYHA);
• Severe myocardial injury：TNT、TNI、CK-MB > 3 ULN;
• Accumulated dose of doxorubicin above 400mg/m2 、epirubicin above 750mg/m2、pirarubicin above 800mg/m2 or the patients treated with anthracycline induced cardiovascular disease;
• Pregnancy or lactating Women;
• Allergic to pegylated liposomal doxorubicin and etoposide;
• HIV antibody positivity;
• Acute or chronic active hepatitis B (HBsAg positivity, HBV DNA negative acceptable), acute or chronic active hepatitis C (HCV antibody negatively acceptable; HCV antibody positivity, HCV RNA negative acceptable);
• Participate in other clinical research at the same time;
• The researchers considered that patients are not suitable for the study.

Study & Design

• Study Type: OBSERVATIONAL
• Study Design: Not specified

Arm && Interventions

Group	Intervention	Description
EBV-HLH patients with intestinal involvement	Segmental bowel resection combined with the DEP regimen	Epstein-Barr virus associated hemophagocytic lymphohistiocytosis patients with imaging confirmed intestinal involvement

Primary Outcome Measures

Name	Time	Method
ORR	4 Week	The percentage of cases that achieved complete response (CR) and partial response (PR) after treatment.
Adverse events	1 month	Incidence of adverse reactions

Secondary Outcome Measures

Name	Time	Method
OS	1 years	Overall survival rate

Trial Locations

Locations (1)

Zhao Wang; 🇨🇳 Beijing, Beijing, China