Serum cytokine profiles in hemophagocytic syndrome following allogeneic hematopoietic stem cell transplantation

摘要

Hemophagocytic syndrome (HPS) is a rare and potentially fatal disorder characterized by hypercytokinemia caused by hyperactivation of CD8+ T lymphocytes and macrophages, and infiltration of these cells into organs [1]. Several clinical symptoms including cytopenias can be explained by overproduction of T helper 1 (Thl) cyto-kines including interferon-gamma, interleukin-2, and tumor necrosis factor-alpha [1, 2]. HPS can be generally classified according to the underlying etiology into familial or secondary to infectious agents, malignancy, or rheumatic diseases [1]. Although it has been assumed that genetic or acquired cytotoxic defects of natural killer T cells and cytotoxic T cells and immune dysregulation may play a key role in the pathogenesis, the exact mechanism remains unclear.