Clinical Trial: Prospective Cohort for Adult Hemophagocytosis

Study Status: Recruiting
Recruit Status: Recruiting
Study Type: Observational [Patient Registry]

Official Title: A Prospective Cohort for Subjects With Adult Hemophagocytic Lymphohistiocytosis Like Syndrome

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.

Detailed Summary:

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

Sponsor: Samsung Medical Center

Current Primary Outcome: Response [ Time Frame: 28 days after the 1st treatment ]

Response to steroids and etoposide


Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Progression-free survival [ Time Frame: 3 years ]
    Time to date of progression or any kinds of death
  • Overall survival [ Time Frame: 3 years ]
    Time to date of any kinds of death


Original Secondary Outcome: Same as current

Information By: Samsung Medical Center

Dates:
Date Received: April 12, 2017
Date Started: January 1, 2017
Date Completion: December 31, 2022
Last Updated: April 12, 2017
Last Verified: April 2017