Prospective Cohort for Adult Hemophagocytosis

Study Overview
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Brief Summary
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This prospective study enrolls subjects who have clinical and laboratory manifestations related with hemophagocytic lymphohistiocytosis. The purpose of the study is to evaluate clinical and biological features of adult hemophagocytic lymphohistiocytosis. The enrolled subjects into this study will be evaluated according to the HLH (hemophagocytic lymphohistiocytosis)criteria and treated with systemic immunosuppressive therapy or chemotherapy. All subjects will be regularly monitored by physicians participating in this study.

Detailed Description
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Study process is as follows. Informed consent for subjects fulfilling the inclusion criteria. Laboratory evaluation will be done for subjects according to the following diagnostic criteria. Diagnosis will be established if one of either 1 or 2 below is fulfilled A molecular diagnosis consistent with HLH Diagnostic criteria for HLH fulfilled (5 out of 8 criteria below) fever ≥ 38.5'C for ≥ 7 days splenomegaly ≥ 3 finger breadth below left subcostal margin cytopenias affecting ≥2 of 3 lineages in peripheral blood (Hemoglobin < 9 g/L, Platelets < 100 × 109/L, Absolute neutrophil count < 1.0 × 109/L) Hypertriglyceridemia and/or hypofibrinogenemia: Fasting triglycerides ≥ 265 mg/dL, fibrinogen ≤ 1.5 g/L Hemophagocytosis in bone marrow or spleen or lymph node Low or absent NK-cell activity (according to local laboratory reference) Ferritin ≥ 500 mcg/L Soluble CD25 (sIL-2 receptor) ≥ 2,400 U/mL

Official Title
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A Prospective Cohort for Subjects With Adult Hemophagocytic Lymphohistiocytosis Like Syndrome

Conditions
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Hemophagocytic Lymphohistiocytoses

Intervention / Treatment
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* Drug: Steroids
* Drug: Etoposide


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Subjects should fulfill the following criteria Subjects should have at least one of the following problems Presence of hemophagocytosis in tissue or bone marrow Presence of at least 3 conditions among 8 conditions of HLH diagnostic criteria Age > 18 years Written informed consents Exclusion Criteria: Subjects cannot satisfy the inclusion criteria

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 80 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 |
| --- | --- |
| Hemophagocytosis<br>Subjects should fulfill the following criteria Subjects should have at least one of the following problems Presence of hemophagocytosis in tissue or bone marrow Presence of at least 3 conditions among 8 conditions of HLH diagnostic criteria Age > 18 years Written informed consents Subjects receive steroids and etoposide | Drug: Steroids<br>* High dose dexamethasone 20 mg PO or IV<br>* Other names: Dexamethasone;Drug: Etoposide<br>* Etoposide 150mg/BSA<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Response | Response to steroids and etoposide | 28 days after the 1st treatment | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Progression-free survival | Time to date of progression or any kinds of death | 3 years | 
| Overall survival | Time to date of any kinds of death | 3 years | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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Hemophagocytic lymphohistiocytosis