Prospective Cohort for Adult Hemophagocytosis

Hemophagocytic Lymphohistiocytoses Clinical Trial

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

Status Recruiting

Clinical Trial 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.

Clinical Trial 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 ;

Related Conditions & MeSH terms

• Hemophagocytic Lymphohistiocytoses
• Lymphohistiocytosis, Hemophagocytic

• NCT number: NCT03117010
• Study type: Observational [Patient Registry]
• Source: Samsung Medical Center
• Contact: Seok Jin Kim, MD, PhD
• Phone: +82234101766
• Email: kstwoh@skku.edu
• Status: Recruiting
• Start date: January 1, 2017
• Completion date: December 31, 2024