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A Case of Sclerodermatous Graft‐versus‐Host Disease Responsive to Imatinib Therapy
Author(s) -
Lazar Jozef,
Poonawalla Tasneem,
Teng Joyce M.C.
Publication year - 2011
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.2010.01301.x
Subject(s) - imatinib mesylate , medicine , imatinib , tyrosine kinase inhibitor , tyrosine kinase , immunology , stem cell factor , cytokine , hematopoietic stem cell transplantation , growth factor , graft versus host disease , cancer research , transplantation , stem cell , haematopoiesis , receptor , biology , microbiology and biotechnology , myeloid leukemia , cancer
Sclerodermatous graft‐versus‐host disease (sGVHD) is a rare, late complication of hematopoietic cell transplantation. Classified as a variant of chronic graft‐versus‐host disease, sGVHD is thought to be predominantly an immune‐mediated response characterized by aberrant T‐cell function and dysregulation of tyrosine kinase cascades. Recently, the profibrotic cytokine transforming growth factor B and stimulatory autoantibodies against the platelet‐derived growth factor receptor have been implicated in the pathogenesis of sGVHD. Treatment of sGVHD remains disappointing and largely limited by systemic side effects. Imatinib mesylate is a small molecule tyrosine kinase inhibitor that has been shown to selectively inhibit both the platelet‐derived growth factor receptor and transforming growth factor‐β signaling pathways. We report a case of sGVHD in a pediatric patient that was resistant to traditional therapy but showed improvement in cutaneous symptoms following daily treatment with 400 mg of imatinib mesylate. Due to its favorable side‐effect profile, specificity for molecular pathways deranged in sGVHD and proven efficacy in other sclerodermoid diseases, imatinib mesylate is a promising new tool in the management of this challenging disease.