Prospective Cohort for Adult Hemophagocytosis
For patients and families
In plain language
An automatic summary of structured registry data. It is an orientation aid, not a substitute for the official protocol or a physician assessment.
- What is being studied
- The protocol lists: Steroids, Etoposide.
- Who it may be relevant to
- Registry conditions: Hemophagocytic Lymphohistiocytoses. Basic parameters: 18 years — 80 years · All.
- What needs checking
- Age, condition and sex are only basic indicators. Prior treatment, laboratory values and other mandatory requirements appear in the eligibility criteria below.
- Where it takes place
- South Korea
- Next step
- Save the trial, show it to the treating physician, and confirm current recruitment with the study center. Costs, documents and travel →
Unsure about the terms? Read our patient guide →
Official title
A Prospective Cohort for Subjects With Adult Hemophagocytic Lymphohistiocytosis Like Syndrome
Overview
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
Study process is as follows.
1. Informed consent for subjects fulfilling the inclusion criteria. 2. 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
1. A molecular diagnosis consistent with HLH 2. 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
Interventions
- Drug Steroids
High dose dexamethasone 20 mg PO or IV - Drug Etoposide
Etoposide 150mg/BSA
Primary outcome measures
- Response [Time frame: 28 days after the 1st treatment]
Secondary outcome measures (2)
- Progression-free survival [Time frame: 3 years]
- Overall survival [Time frame: 3 years]
Eligibility criteria
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
Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.
Healthy volunteers: No
Study design
- Observational model
- Cohort
Study locations
South Korea · 1 center
- Samsung Medical Center — Seoul
Publications
- Yoon SE, Eun Y, Huh K, Chung CR, Yoo IY, Cho J, Cho D, Ko YH, Park S, Kim WS, Kim SJ. A comprehensive analysis of adult patients with secondary hemophagocytic lymphohistiocytosis: a prospective cohort study. Ann Hematol. 2020 Sep;99(9):2095-2104. doi: 10.1007/s00277-020-04083-6. Epub 2020 May 21. PMID 32440790
Identifiers
NCT: NCT03117010 · 2016-08-117