Imatinib as a treatment option for systemic non-Langerhans cell histiocytoses.
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.


METHODS
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 beta and KIT. The disease completely responded to treatment with 400 to 600 mg daily of imatinib for more than 7 months.


CONCLUSIONS
This case shows that imatinib is a powerful treatment option for patients with non-Langerhans cell histiocytoses.
Utikal
Jochen
J
Department of Dermatology, Venereology, and Allergology, Central Laboratory, University Medical Center Mannheim, Ruprecht-Karl-University of Heidelberg, Germany. jutikal@mgh.harvard.edu
Ugurel
Selma
S
Kurzen
Hjalmar
H
Erben
Philipp
P
Reiter
Andreas
A
Hochhaus
Andreas
A
Nebe
Thomas
T
Hildenbrand
Ralf
R
Haberkorn
Uwe
U
Goerdt
Sergij
S
Schadendorf
Dirk
D
D000328:Adult; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D001549:Benzamides; D003937:Diagnosis, Differential; D015616:Histiocytosis, Non-Langerhans-Cell / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D000068877:Imatinib Mesylate; D008279:Magnetic Resonance Imaging; D008297:Male; D010879:Piperazines / Q000008:administration & dosage / Q000627:therapeutic use*; D049268:Positron-Emission Tomography; D047428:Protein Kinase Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use*; D011505:Protein-Tyrosine Kinases / Q000037:antagonists & inhibitors*; D011743:Pyrimidines / Q000008:administration & dosage / Q000627:therapeutic use*; D012720:Severity of Illness Index
D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D047428:Protein Kinase Inhibitors; D011743:Pyrimidines; D000068877:Imatinib Mesylate; D011505:Protein-Tyrosine Kinases