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Imatinib as a Treatment Option for Systemic Non-Langerhans Cell Histiocytoses
Jochen Utikal, MD;
Selma Ugurel, MD;
Hjalmar Kurzen, MD;
Philipp Erben, MD;
Andreas Reiter, MD;
Andreas Hochhaus, MD;
Thomas Nebe, MD;
Ralf Hildenbrand, MD;
Uwe Haberkorn, MD;
Sergij Goerdt, MD;
Dirk Schadendorf, MD
Arch Dermatol. 2007;143(6):736-740.
Background Systemic non-Langerhans cell histiocytoses are disorders characterized by the accumulation of histiocytes that do not meet the criteria for Langerhans cells in various organs. So far, no causative treatment is known.
Observations Herein, we report the case of a 41-year-old man with Rosai-Dorfman disease, a form of systemic non-Langerhans cell histiocytoses, with histiocytic infiltrations in the skin, bone marrow, liver, and spleen. Histiocytes were positive for the imatinib target proteins platelet-derived growth factor receptor β and KIT. The disease completely responded to treatment with 400 to 600 mg daily of imatinib for more than 7 months.
Conclusion This case shows that imatinib is a powerful treatment option for patients with non-Langerhans cell histiocytoses.
Author Affiliations: Department of Dermatology, Venereology, and Allergology (Drs Utikal, Ugurel, Kurzen, Goerdt, and Schadendorf), Department of Internal Medicine III (Drs Erben, Reiter, and Hochhaus), Central Laboratory (Dr Nebe), and Department of Pathology (Dr Hildenbrand), University Medical Center Mannheim, Ruprecht-Karl-University of Heidelberg, Mannheim, Germany; Skin Cancer Unit, German Cancer Research Center, Heidelberg, Germany (Drs Ugurel and Schadendorf); and Department of Nuclear Medicine, Ruprecht-Karl-University of Heidelberg, Heidelberg (Dr Haberkorn). Dr Utikal is now also with Massachusetts General Hospital Cancer Center, Harvard Medical School, Boston, Mass.
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