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Blood, 1 April 2003, Vol. 101, No. 7, pp. 2473-2475
CHEMOKINES
Brief report
Coincident expression of the chemokine receptors CCR6 and CCR7 by
pathologic Langerhans cells in Langerhans cell histiocytosis
Mark D. Fleming,
Jack L. Pinkus,
Marcia V. Fournier,
Sarah W. Alexander,
Carmen Tam,
Massimo Loda,
Stephen E. Sallan,
Kim E. Nichols,
David F. Carpentieri,
Geraldine S. Pinkus, and
Barrett J. Rollins
From the Department of Medical Oncology, Dana-Farber
Cancer Institute and Department of Medicine, Brigham & Women's
Hospital, Harvard Medical School, Boston, MA; Department of Pathology,
Brigham & Women's Hospital, Harvard Medical School, Boston, MA;
Department of Pediatric Oncology, Dana-Farber Cancer Institute and
Division of Hematology/Oncology, Children's Hospital, Harvard Medical
School, Boston, MA; Department of Pathology, Children's Hospital,
Harvard Medical School, Boston, MA; Departments of Pediatric Oncology
and Pathology, Children's Hospital of Pennsylvania, University of
Pennsylvania, Philadelphia.
It has been suggested that a switch in chemokine receptor
expression underlies Langerhans cell migration from skin to lymphoid tissue. Activated cells are thought to down-regulate CCR6, whose ligand
macrophage inflammatory protein-3 (MIP-3)/CCL20 is
expressed in skin, and up-regulate CCR7, whose ligands are in lymphoid
tissues. In Langerhans cell histiocytosis (LCH), pathologic Langerhans cells (LCs) accumulate in several tissues, including skin, bone, and lymphoid organs. We have examined 24 LCH cases and find that pathologic LCs expressed CCR6 and CCR7 coincidentally in all cases. Furthermore, MIP-3/CCL20 is expressed by keratinocytes in involved skin and by macrophages and osteoblasts in involved bone. Expression of
CCR6 by pathologic LCs may contribute to their accumulation in
nonlymphoid organs such as skin and bone, whereas CCR7 expression may
direct them to lymphoid tissue. Histiocytes in Rosai-Dorfman disease
and hemophagocytic syndrome also coexpressed CCR6 and CCR7, suggesting
that this may be a general attribute of abnormal histiocytes.
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