• Clinical outcome in IL-10- and IL-10 receptor-deficient patients with or without hematopoietic stem cell transplantation

    In health, there is always a fine balance between the proteins that cause inflammation and the proteins that suppress inflammation. IL-10 is a key protein of the immune system of our body involved in suppressing inflammation. When an individual’s genetic make-up has severe mutations (or variations from normal) in the gene that controls IL-10, this balance is lost. This results in inflammation and ulcers in the gut causing an IBD like illness usually in very young children. Since 2009 there have been several reports by different research groups describing these severe mutations in “very early onset IBD”, many patients have received bone marrow transplants as treatment.

    In the current paper, 40 patients with onset of IBD in the first 4 years of life were assessed for IL-10 mutations. Of these, 9 patients were found to have severe mutations in the IL-10 gene. All these patients had features of an extremely severe disease showing very poor response to medical treatment and most underwent surgery. Three of these patients underwent a bone marrow transplant (as all the IL-10 producing cells in the body are derived from the bone marrow) and showed a very good response. The current paper is very insightful and forward thinking in terms of consolidating a futuristic role for bone marrow transplant in patients with severe IL-10 mutations.

    This paper and also previous reports recommend that all children presenting with a very early onset IBD (less than 5 years of age) should have their immune system assessed for IL-10.

    Bone marrow transplant in this particular group of patients with these severe genetic defects in IL-10, can offer a potential cure (of course mindful of the fact that a bone marrow transplant is a heavy undertaking and has its own side-effects).

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