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

Study Overview
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Brief Summary
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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
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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.

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

Conditions
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Hemophagocytic Lymphohistiocytosis, Epstein-Barr Virus, Bowel Resection

Intervention / Treatment
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* Other: Segmental bowel resection combined with the DEP regimen


Participation Criteria
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Eligibility Criteria
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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.

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

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

What is the study measuring?
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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 |