Hemophagocytic Lymphohistiocytosis
Conditions
Keywords
haemophagocytic lymphohistiocytosis, splenectomy, unknown etiology
Brief summary
To evaluate the clinical value of splenectomy as a treatment for relapsed haemophagocytic lymphohistiocytosis (HLH) in patient with unknown etiology.
Interventions
PROCEDUREsplenectomy
Sponsors
Beijing Friendship Hospital
Study design
Allocation
NA
Intervention model
SINGLE_GROUP
Primary purpose
TREATMENT
Masking
NONE
Eligibility
Sex/Gender
ALL
Age
14 Years to 60 Years
Healthy volunteers
No
Inclusion criteria
1. Patients were older than 14 years of age 2. Diagnosed as Hemophagocytic Lymphohistiocytosis (HLH) according to HLH-2004. 3. patients were excluded with infection, rheumatic and immunologic diseases, malignant tumors (especially lymphoma), and primary HLH. 4. Informed consent
Exclusion criteria
1. Pregnancy or lactating Women 2. Active bleeding of the internal organs 3. Uncontrollable infection 4. Contraindication of splenectomy 5. Participate in other clinical research at the same time
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Evaluation of treatment response | Change from before and1,2,4,8,12 and 24 weeks after splenectomy | 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%. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Survival | from the time patients received splenectomy up to 24 months or December 2018 | — |
| Adverse events that are related to treatment | through study completion, an average of 2 years | Adverse events including infection, bleeding and so on. |
Countries
China
Contacts
Primary Contactjingshi wang, M.M.
Outcome results
None listed