Figure 2.
Farber patients have a unique plasma cytokine profile that is normalized following HSCT and different from JIA. Cytokines were measured in plasma from controls (n=39), untreated Farber patients (n=13), HSCT-treated Farber patients (n=5), and JIA patients (n=5) using a multiplex assay. MCP-1 (A), IP-10 (B), IL-6 (C), IL-12 (D) and VEGF (E) levels are shown. Unbiased hierarchical clustering was performed on all patients using these five cytokines (F) or MCP-1 (G). A cluster dendrogram is seen at the top (branches), followed by the patient color key (single row of colored rectangles) and a heatmap of the relative cytokine levels (green, black, and red rectangles). The relative amount of each cytokine is seen horizontally corresponding to its row, where green represents relatively low expression, black medium expression, and red relatively high expression. *p<0.05. HSCT, hematopoietic stem cell transplanted Farber patients; JIA, Juvenile Idiopathic Arthritis; ECD, Erdheim-Chester disease; MCP-1, monocyte chemotactic protein 1; IP-10, interferon gamma-induced protein 10; IL-6, interleukin 6; IL-12, interleukin 12; VEGF, vascular endothelial growth factor; ACDase, acid ceramidase.