THE RELATION OF LANGERHANS CELL HISTIOCYTOSIS TO ACUTE-LEUKEMIA, LYMPHOMAS, AND OTHER SOLID TUMORS - THE LCH-MALIGNANCY STUDY-GROUP OF THE HISTIOCYTE-SOCIETY
Rm. Egeler et al., THE RELATION OF LANGERHANS CELL HISTIOCYTOSIS TO ACUTE-LEUKEMIA, LYMPHOMAS, AND OTHER SOLID TUMORS - THE LCH-MALIGNANCY STUDY-GROUP OF THE HISTIOCYTE-SOCIETY, Hematology/oncology clinics of North America, 12(2), 1998, pp. 369
The frequency of Langerhans cell histiocytosis (LCH) and a malignant n
eoplasm occurring in the same individual appears to be greater than pr
eviously recognized. To define the occurrence and the pattern of these
events, a Study Group of the Histiocyte Society initiated a registry
of patients in whom this association occurred synchronously or asynchr
onously. Evaluation of 54 patients detected two patterns of associatio
ns between LCH and other disorders. First, it is possible that therapy
of LCH promotes a secondary malignancy. Second, it is possible that a
genetic predisposition, with or without the immunosuppression associa
ted therapy for the malignancy, plays a role in the development and ex
pression of disseminated LCH. Data collected by the LCH Malignancy Stu
dy Group may provide insights into the etiology and pathophysiology of
LCH.