Prospective Cohort for Adult Hemophagocytosis

Hemophagocytic Lymphohistiocytoses Clinical Trial

Official title:

A Prospective Cohort for Subjects With Adult Hemophagocytic Lymphohistiocytosis Like Syndrome

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT03117010
• Other study ID #: 2016-08-117
• Status: Recruiting
• Start date: January 1, 2017
• Est. completion date: December 31, 2024

Study information

• Verified date: November 2023
• Source: Samsung Medical Center
• Contact: Seok Jin Kim, MD, PhD
• Phone: +82234101766
• Email: kstwoh[@]skku.edu
• Is FDA regulated: No
• Study type: Observational [Patient Registry]

Clinical Trial Summary

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.

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

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 81
• Est. completion date: December 31, 2024
• Est. primary completion date: December 31, 2023
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years to 80 Years

Eligibility

Inclusion Criteria:

• Subjects should fulfill the following criteria

1. Subjects should have at least one of the following problems

1. Presence of hemophagocytosis in tissue or bone marrow

2. Presence of at least 3 conditions among 8 conditions of HLH diagnostic criteria

2. Age > 18 years

3. Written informed consents

Exclusion Criteria:

• Subjects cannot satisfy the inclusion criteria

Related Conditions & MeSH terms

• Hemophagocytic Lymphohistiocytoses
• Lymphohistiocytosis, Hemophagocytic

Intervention

Drug:
• Steroids
High dose dexamethasone 20 mg PO or IV
• Etoposide
Etoposide 150mg/BSA

Locations

Country	Name	City	State
Korea, Republic of	Samsung Medical Center	Seoul

Sponsors (1)

Lead Sponsor	Collaborator
Samsung Medical Center

Country where clinical trial is conducted

Korea, Republic of, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	Response	Response to steroids and etoposide	28 days after the 1st treatment
Secondary	Progression-free survival	Time to date of progression or any kinds of death	3 years
Secondary	Overall survival	Time to date of any kinds of death	3 years