Splenectomy as a Treatment for Patient With Relapsed Haemophagocytic Lymphohistiocytosis of Unknown Etiology

Study Overview
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Brief Summary
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To evaluate the clinical value of splenectomy as a treatment for relapsed haemophagocytic lymphohistiocytosis (HLH) in patient with unknown etiology.

Conditions
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Hemophagocytic Lymphohistiocytosis

Intervention / Treatment
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* Procedure: splenectomy


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Patients were older than 14 years of age Diagnosed as Hemophagocytic Lymphohistiocytosis (HLH) according to HLH-2004. patients were excluded with infection, rheumatic and immunologic diseases, malignant tumors (especially lymphoma), and primary HLH. Informed consent Exclusion Criteria: Pregnancy or lactating Women Active bleeding of the internal organs Uncontrollable infection Contraindication of splenectomy Participate in other clinical research at the same time

Ages Eligible for Study
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Minimum Age: 14 Years
Maximum Age: 60 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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Design Details

Primary Purpose: Treatment
Intervention Model: Single Group Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: Splenectomy<br>Splenectomy as a treatment for patient with relapsed haemophagocytic lymphohistiocytosis of unknown etiology | Procedure: splenectomy<br> <br> |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Evaluation of treatment response | A complete response was defined as normalization of all of the quantifiable symptoms and laboratory markers of HLH, including levels of sCD25, ferritin, and triglyceride; hemoglobin; neutrophil counts; platelet counts; and alanine aminotransferase (ALT). A partial response was defined as at least a 25% improvement in 2 or more quantifiable symptoms and laboratory markers as follows: sCD25 response was>1.5-fold decreased; ferritin and triglyceride decreased at least 25%; for patients with an initial neutrophil count of<0.5 ×109/L, a response was defined as an increase by at least 100% to>0.5× 109/L; for patients with a neutrophil count of 0.5 to 2.0 × 109/L, an increase by at least 100% to >2.0 × 109/L was considered a response; and for patients with ALT >400 U/L, response was defined as an ALT decrease of at least 50%. | Change from before and1,2,4,8,12 and 24 weeks after splenectomy | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Survival |  | from the time patients received splenectomy up to 24 months or December 2018 | 
| Adverse events that are related to treatment | Adverse events including infection, bleeding and so on. | through study completion, an average of 2 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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haemophagocytic lymphohistiocytosis, splenectomy, unknown etiology