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Trial record 4 of 9 for:    Dexamethasone | "familial hemophagocytic lymphohistiocytosis"

Prospective Cohort for Adult Hemophagocytosis

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ClinicalTrials.gov Identifier: NCT03117010
Recruitment Status : Recruiting
First Posted : April 17, 2017
Last Update Posted : January 1, 2019
Sponsor:
Information provided by (Responsible Party):
Samsung Medical Center

Brief 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.

Condition or disease Intervention/treatment
Hemophagocytic Lymphohistiocytoses Drug: Steroids Drug: Etoposide

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

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Study Type : Observational [Patient Registry]
Estimated Enrollment : 81 participants
Observational Model: Cohort
Time Perspective: Prospective
Target Follow-Up Duration: 3 Years
Official Title: A Prospective Cohort for Subjects With Adult Hemophagocytic Lymphohistiocytosis Like Syndrome
Actual Study Start Date : January 1, 2017
Estimated Primary Completion Date : December 31, 2020
Estimated Study Completion Date : December 31, 2022


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 Name: Dexamethasone

Drug: Etoposide
Etoposide 150mg/BSA




Primary Outcome Measures :
  1. Response [ Time Frame: 28 days after the 1st treatment ]
    Response to steroids and etoposide


Secondary Outcome Measures :
  1. Progression-free survival [ Time Frame: 3 years ]
    Time to date of progression or any kinds of death

  2. Overall survival [ Time Frame: 3 years ]
    Time to date of any kinds of death


Biospecimen Retention:   Samples With DNA
  1. Serum sample collection will be done as follows: Baseline, 4 weeks after treatment, Relapse or progression
  2. Germline DNA collection from peripheral blood will be done at the enrollment


Information from the National Library of Medicine

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Ages Eligible for Study:   18 Years to 80 Years   (Adult, Older Adult)
Sexes Eligible for Study:   All
Accepts Healthy Volunteers:   No
Sampling Method:   Probability Sample
Study Population
Subjects who have clinical and laboratory manifestations related with hemophagocytic lymphohistiocytosis.
Criteria

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

Information from the National Library of Medicine

To learn more about this study, you or your doctor may contact the study research staff using the contact information provided by the sponsor.

Please refer to this study by its ClinicalTrials.gov identifier (NCT number): NCT03117010


Contacts
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Contact: Seok Jin Kim, MD, PhD +82234101766 kstwoh@skku.edu
Contact: Silvia Park, MD, PhD +82234103459 silvia.park@samsung.com

Locations
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Korea, Republic of
Samsung Medical Center Recruiting
Seoul, Korea, Republic of, 135-710
Contact: Seok Jin Kim, MD, PhD    82-2-3410-1766    kstwoh@skku.edu   
Principal Investigator: Seok Jin Kim, MD, PhD         
Sub-Investigator: Won Seog Kim, MD, PhD         
Sponsors and Collaborators
Samsung Medical Center
Investigators
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Principal Investigator: Seok Jin Kim, MD, PhD Samsung Medical Center

Additional Information:

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Responsible Party: Samsung Medical Center
ClinicalTrials.gov Identifier: NCT03117010     History of Changes
Other Study ID Numbers: 2016-08-117
First Posted: April 17, 2017    Key Record Dates
Last Update Posted: January 1, 2019
Last Verified: December 2018
Individual Participant Data (IPD) Sharing Statement:
Plan to Share IPD: Undecided

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Studies a U.S. FDA-regulated Drug Product: No
Studies a U.S. FDA-regulated Device Product: No

Keywords provided by Samsung Medical Center:
Hemophagocytic lymphohistiocytosis

Additional relevant MeSH terms:
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Dexamethasone
Dexamethasone acetate
BB 1101
Lymphohistiocytosis, Hemophagocytic
Histiocytosis, Non-Langerhans-Cell
Histiocytosis
Lymphatic Diseases
Etoposide
Etoposide phosphate
Anti-Inflammatory Agents
Antiemetics
Autonomic Agents
Peripheral Nervous System Agents
Physiological Effects of Drugs
Gastrointestinal Agents
Glucocorticoids
Hormones
Hormones, Hormone Substitutes, and Hormone Antagonists
Antineoplastic Agents, Hormonal
Antineoplastic Agents
Protease Inhibitors
Enzyme Inhibitors
Molecular Mechanisms of Pharmacological Action
Antineoplastic Agents, Phytogenic
Topoisomerase II Inhibitors
Topoisomerase Inhibitors