Prospective Cohort for Adult Hemophagocytosis

November 22, 2023 updated by: Samsung Medical Center

A Prospective Cohort for Subjects With Adult Hemophagocytic Lymphohistiocytosis Like Syndrome

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.

Study Overview

Status

Recruiting

Conditions

Intervention / Treatment

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

Study Type

Observational

Enrollment (Estimated)

81

Contacts and Locations

This section provides the contact details for those conducting the study, and information on where this study is being conducted.

Study Contact

  • Name: Seok Jin Kim, MD, PhD
  • Phone Number: +82234101766
  • Email: kstwoh@skku.edu

Study Contact Backup

Study Locations

  • Korea, Republic of
      • Seoul, Korea, Republic of, 135-710
        • Recruiting
        • Samsung Medical Center
        • Principal Investigator:
          • Seok Jin Kim, MD, PhD
        • Sub-Investigator:
          • Won Seog Kim, MD, PhD
        • Contact:
          • Seok Jin Kim, MD, PhD
          • Phone Number: 82-2-3410-1766
          • Email: kstwoh@skku.edu

Participation Criteria

Researchers look for people who fit a certain description, called eligibility criteria. Some examples of these criteria are a person's general health condition or prior treatments.

Eligibility Criteria

Ages Eligible for Study

18 years to 80 years (Adult, Older Adult)

Accepts Healthy Volunteers

No

Sampling Method

Probability Sample

Study Population

Subjects who have clinical and laboratory manifestations related with hemophagocytic lymphohistiocytosis.

Description

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

Study Plan

This section provides details of the study plan, including how the study is designed and what the study is measuring.

How is the study designed?

Design Details

Cohorts and Interventions

Group / Cohort
Intervention / Treatment
Hemophagocytosis

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

Subjects receive steroids and etoposide
Drug: Steroids
High dose dexamethasone 20 mg PO or IV
Other Names:
  • Dexamethasone
Drug: Etoposide
Etoposide 150mg/BSA

What is the study measuring?

Primary Outcome Measures

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

Secondary Outcome Measures

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

Collaborators and Investigators

This is where you will find people and organizations involved with this study.

Sponsor

Samsung Medical Center

Investigators

  • Principal Investigator: Seok Jin Kim, MD, PhD, Samsung Medical Center

Publications and helpful links

The person responsible for entering information about the study voluntarily provides these publications. These may be about anything related to the study.

General Publications

Study record dates

These dates track the progress of study record and summary results submissions to ClinicalTrials.gov. Study records and reported results are reviewed by the National Library of Medicine (NLM) to make sure they meet specific quality control standards before being posted on the public website.

Study Major Dates

Study Start (Actual)

January 1, 2017

Primary Completion (Estimated)

December 31, 2023

Study Completion (Estimated)

December 31, 2024

Study Registration Dates

First Submitted

April 12, 2017

First Submitted That Met QC Criteria

April 12, 2017

First Posted (Actual)

April 17, 2017

Study Record Updates

Last Update Posted (Actual)

November 27, 2023

Last Update Submitted That Met QC Criteria

November 22, 2023

Last Verified

November 1, 2023

More Information

Terms related to this study

Keywords

Additional Relevant MeSH Terms

Other Study ID Numbers

  • 2016-08-117

Plan for Individual participant data (IPD)

Plan to Share Individual Participant Data (IPD)?

UNDECIDED

Drug and device information, study documents

Studies a U.S. FDA-regulated drug product

No

Studies a U.S. FDA-regulated device product

No

This information was retrieved directly from the website clinicaltrials.gov without any changes. If you have any requests to change, remove or update your study details, please contact register@clinicaltrials.gov. As soon as a change is implemented on clinicaltrials.gov, this will be updated automatically on our website as well.

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