RECRUITING

Hemophagocytic Lymphohistiocytosis (HLH) Evaluation and Research of Clinical, ImmUnoLogic and TranscriptomE Study

Study Overview

This clinical trial focuses on testing the efficacy of different digital interventions to promote re-engagement in cancer-related long-term follow-up care for adolescent and young adult (AYA) survivors of childhood cancer.

Description

Background: Hemophagocytic lymphohistiocytosis (HLH) is a disease caused by disrupted immune function. People with HLH are prone to fevers and illnesses, which can be fatal. Some people develop a genetic form of this disease (pHLH), but researchers do not understand why some other people develop a nongenetic form (sHLH). They also do not have good ways to diagnose and treat sHLH. Objective: To learn about sHLH and why some people get it and others do not. Eligibility: Adults aged 18 years and older with sHLH. Design: Participants will be admitted to the study based on a review of their medical records. Those who join will have at least 3 clinical evaluations over 9 to 12 months. These may occur during an inpatient hospitalization if they require medical care or in the outpatient clinic. Participants will also have a physical exam at each visit. Up to half a cup of blood will be drawn at each visit. Participants may also have their blood drawn by their own doctors, who will send the samples to the researchers. Researchers may also contact these participants by telephone or video calls. The blood will be used for clinical tests as well as research. No new treatments will be administered as part of this study; however, standard medications and treatments may be recommended. Participants may opt to continue their visits once a year for 3 more years. Participants may also opt for an extra clinial evaluation 1 week after starting a new treatment. ...

Official Title

Hemophagocytic Lymphohistiocytosis (HLH) Evaluation and Research of Clinical, ImmUnoLogic and TranscriptomE Study

Quick Facts

Study Start:2024-07-02
Study Completion:2031-04-01
Study Type:Not specified
Phase:Not Applicable
Enrollment:Not specified
Status:RECRUITING

Study ID

NCT06339177

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.

Ages Eligible for Study:18 Years to 120 Years
Sexes Eligible for Study:ALL
Accepts Healthy Volunteers:No
Standard Ages:ADULT, OLDER_ADULT
Inclusion CriteriaExclusion Criteria
  1. * Aged 18 years or older.
  2. * Established diagnosis of sHLH defined by meeting any published criteria, per Table 1:
  3. * Meeting the HLH-2004 criteria.
  4. * HScore of \>168. For those without a bone marrow biopsy to evaluate for hemophagocytosis (worth 35 points in the criteria), HScore\>134 will be used.
  5. * For those with underlying rheumatologic disease: meeting the 2016 American College of Rheumatology criteria for macrophage activation syndrome.
  6. * Agree to storage and sharing of study data and biospecimens for future research use.
  7. * Fever (\>38.4 Degrees Celcius)
  8. * Splenomegaly
  9. * Cytopenias affecting \>=2 of 3 lineages: Hgb \<9 g/dL, platelets \<10\^5/microliter, neutrophils \<10\^6/microliter
  10. * Hypertriglyceridemia (\>256 mg/dL) and/or fibrinogen \<1.5 g/L
  11. * Hemophagocytosis on biopsy
  12. * Serum ferritin \>=500 ng/mL
  13. * Increased serum sCD25 (\>2400 U/mL)
  14. * Low or absent NK cell activity
  15. * Platelets \<=181,000/microliter
  16. * AST \>48 IU/mL
  17. * Triglycerides \>156 mg/dL
  18. * Fibrinogen \<=3.6 g/L
  1. * Currently pregnant.
  2. * Any condition that, in the judgment of the investigator, may put the participant at undue risk or make them unsuitable for participation in the study.

Contacts and Locations

Study Contact

Joseph M Rocco, M.D.
CONTACT
(301) 312-2858
joseph.rocco@nih.gov

Principal Investigator

Joseph M Rocco, M.D.
PRINCIPAL_INVESTIGATOR
National Institute of Allergy and Infectious Diseases (NIAID)

Study Locations (Sites)

National Institutes of Health Clinical Center
Bethesda, Maryland, 20892
United States

Collaborators and Investigators

Sponsor: National Institute of Allergy and Infectious Diseases (NIAID)

  • Joseph M Rocco, M.D., PRINCIPAL_INVESTIGATOR, National Institute of Allergy and Infectious Diseases (NIAID)

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 Registration Dates

Study Start Date2024-07-02
Study Completion Date2031-04-01

Study Record Updates

Study Start Date2024-07-02
Study Completion Date2031-04-01

Terms related to this study

Keywords Provided by Researchers

  • Hemophagocytic Lymphohistiocytosis
  • HLH
  • Macrophage Activation Syndrome
  • MAS
  • Cytokine Storm Syndrome
  • Hyperinflammatory Syndrome
  • Hyperinflammation
  • Still's Disease
  • Hyperferritinemia
  • Immune Dysregulation

Additional Relevant MeSH Terms

  • Lymphohistiocytosis, Hemophagocytic
  • Secondary Hemophagocytic Lymphohistiocytosis
  • Macrophage Activation Syndrome
  • Hyperinflammatory Syndromes