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

Efficacy and Safety of Segmental Resection Combined With the DEP Regimen for Epstein-Barr Virus Associated Hemophagocytic Lymphohistiocytosis With Intestinal Involvement

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.

Study Overview

• Status: Not yet recruiting
• Conditions: Hemophagocytic Lymphohistiocytosis; Bowel Resection; Epstein-Barr Virus
• Intervention / Treatment: Other: Segmental bowel 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 Type: Observational
• Enrollment (Estimated): 20

Contacts and Locations

• Study Contact: Name: Zhao Wang, MD; Phone Number: 63138303; Email: wangzhao@ccmu.edu.cn

Study Locations

• China
  • Beijing
    • Beijing, Beijing, China, 100050
      • Zhao Wang
      • Contact: Zhao Wang, MD; Phone Number: 63138303; Email: wangzhao@ccmu.edu.cn

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: Adult; Older Adult
• Accepts Healthy Volunteers: No

• Sampling Method: Non-Probability Sample

Study Population

EBV-HLH patients with intestinal involvement

Description

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 Plan

How is the study designed?

Number of groups / cohorts

1

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
EBV-HLH patients with intestinal involvement; Epstein-Barr virus associated hemophagocytic lymphohistiocytosis patients with imaging confirmed intestinal involvement	Other: Segmental bowel resection combined with the DEP regimen; Segmental bowel resection combined with liposomal doxorubicin, etoposide, and methylprednisolone administered in 2 week cycles for 2 cycles

What is the study measuring?

Primary Outcome Measures

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

Secondary Outcome Measures

Outcome Measure	Measure Description	Time Frame
OS	Overall survival rate	1 years

Collaborators and Investigators

• Sponsor: Beijing Friendship Hospital
• Investigators: Principal Investigator: Zhao Wang, MD, Beijing Friendship Hospital

Study record dates

Study Major Dates

• Study Start (Estimated): December 1, 2023
• Primary Completion (Estimated): December 1, 2026
• Study Completion (Estimated): January 1, 2027

Study Registration Dates

• First Submitted: November 9, 2023
• First Submitted That Met QC Criteria: November 9, 2023
• First Posted (Actual): November 14, 2023

Study Record Updates

• Last Update Posted (Actual): November 14, 2023
• Last Update Submitted That Met QC Criteria: November 9, 2023
• Last Verified: September 1, 2023

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Virus Diseases; Infections; Lymphatic Diseases; DNA Virus Infections; Tumor Virus Infections; Herpesviridae Infections; Histiocytosis, Non-Langerhans-Cell; Histiocytosis; Epstein-Barr Virus Infections; Lymphohistiocytosis, Hemophagocytic
• Other Study ID Numbers: BFH20230809002/BFHHZS20230190

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No