NCT03117010

Prospective Cohort for Adult Hemophagocytosis

A Prospective Cohort for Subjects With Adult Hemophagocytic Lymphohistiocytosis Like Syndrome

• Status: Recruiting
• Phase: —
• Study type: Observational
• Enrollment: 81 (estimated)
• Sponsor: Samsung Medical Center · Academic / Other
• Sex: All
• Age: 18 Years – 80 Years
• Healthy volunteers: Not accepted

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 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

Conditions

• Hemophagocytic Lymphohistiocytoses

Interventions

Type	Name	Description
DRUG	Steroids	High dose dexamethasone 20 mg PO or IV
DRUG	Etoposide	Etoposide 150mg/BSA

Timeline

• Start date: 2017-01-01
• Primary completion: 2025-12-31
• Completion: 2026-12-31
• First posted: 2017-04-17
• Last updated: 2024-08-07

Locations

1 site across 1 country: South Korea