A Prospective Cohort for Subjects With Adult Hemophagocytic Lymphohistiocytosis Like Syndrome
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 入组人数
- 81
- 试验地点
- 2
- 主要终点
- Response
研究概览
简要总结
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.
详细描述
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
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 80 Years（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •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
排除标准
- •Subjects cannot satisfy the inclusion criteria
研究组 & 干预措施
Hemophagocytosis
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
干预措施: Steroids (Drug)
Hemophagocytosis
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
干预措施: Etoposide (Drug)
结局指标
主要结局
Response
时间窗: 28 days after the 1st treatment
Response to steroids and etoposide
次要结局
- Progression-free survival(3 years)
- Overall survival(3 years)