Clinical trial NCT06131489: Segmental Resection Combined With DEP Regimen for EBV-HLH Patients With Intestinal Involvement
Official title: Efficacy and Safety of Segmental Resection Combined With the DEP Regimen for Epstein-Barr Virus Associated Hemophagocytic Lymphohistiocytosis With Intestinal Involvement
Study type: OBSERVATIONAL
Overall status: NOT_YET_RECRUITING
Conditions: Hemophagocytic Lymphohistiocytosis
Epstein-Barr Virus
Bowel Resection
Lead sponsor: Beijing Friendship Hospital
Sponsor class: OTHER
First posted: 2023-11-14
Enrollment: 20
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: OTHER
Time perspective: PROSPECTIVE
Sampling method: NON_PROBABILITY_SAMPLE
Study population: EBV-HLH patients with intestinal involvement

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.

Eligibility criteria: 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.

Primary outcomes:
measure: ORR
timeFrame: 4 Week
measure: Adverse events
timeFrame: 1 month
Secondary outcomes:
measure: OS
timeFrame: 1 years
Other outcomes:
Locations:
city: Beijing
country: China
facility: Zhao Wang
state: Beijing
Study officials:
affiliation: Beijing Friendship Hospital
name: Zhao Wang, MD
role: PRINCIPAL_INVESTIGATOR
References: