Research ArticleImmune thrombocytopenic purpura

Plasmacytoid Dendritic Cells, Interferon Signaling, and FcγR Contribute to Pathogenesis and Therapeutic Response in Childhood Immune Thrombocytopenia

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Science Translational Medicine  10 Jul 2013:
Vol. 5, Issue 193, pp. 193ra89
DOI: 10.1126/scitranslmed.3006277

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Type I Interferons—A Sticky Situation?

“If you prick us, do we not bleed?” – William Shakespeare

One symptom of patients with low platelet numbers is not only do they bleed, they may have limited ability to stop bleeding. Immune thrombocytopenia (ITP) is one such disorder where the person’s own immune system attacks and destroys these critical clotting factors. Fortunately, infusion of intravenous immunoglobulins (IVIGs) can help to treat this disease. Now, Sehgal et al. shed some light on the mechanisms underlying both ITP pathogenesis and the effectiveness of IVIGs in ITP patients.

The authors found that immune cells and plasma from ITP patients had increased amounts of type I interferons and downstream interferon response (IR) genes. Patients with therapy-induced remission lacked the increased IR signature. When treated with IVIG, monocytes from ITP patients altered the activating/inhibitory ratio of cell surface receptors that bind the Fc portion of IgG. These changes altered the ability of these cells to respond to type I interferon, and suggest that type I IR may contribute to ITP pathogenesis.