Therapeutic Efficacy of Leukocytapheresis for Procoagulant Microparticles during Hemophagocytic Syndrome

Hemophagocytic syndrome (HPS) presents with signs of persistent remittent fever, hepatosplenomegaly, pancytopenia, hepatic dysfunction, and disseminated intravascular coagulation because of hypercytokinemia caused by activated T lymphocytes and macrophages. In recent years leukocytapheresis using a leukocyte removal filter (known as lymphocytapheresis, LCAP) has been applied to the treatment of various autoimmune diseases. The removal of activated monocytes during LCAP treatment appears useful for hypercytokinemia. We experienced a 32-year-old Japanese man with HPS with elevated tissue factor-enriched monocyte-derived microparticles (MDMPs) and pro-inflammatory cytokines/chemokines. Improvements in the level of MDMPs and hypercytokinemia were observed after LCAP treatment. LCAP treatment performed for HPS can be considered a therapeutic strategy for patients with a risk of fetal hemorrhage.