Langerhans Cell Histiocytosis (LCH) is a rare disease
caused by the proliferation of abnormal Langerhans cells (LCs) which are the
primary antigen presenting cells of skin. Predominantly, but not exclusively,
a disease of childhood, LCH can be mild and easily treatable, or aggressive
and fatal. It is characterized by collections of abnormal LCs in secondary lymphoid
organs, skin, bone, and other organs. Because LCs are leukocytes and because
their normal migration is controlled by chemokines, we have been investigating
clinical material obtained from patients seen at the Jimmy Fund Clinic for abnormalities
in the chemokine system. We have found, for example, that unlike normal LCs
which exchange one chemokine receptor on their surfaces for another when they
become activated, pathological LCs coexpress both receptors. Furthermore, the
chemokines that activate these receptors are found in the organs these cells
invade, suggesting that this may be the reason for patterns of tissue involvement
in LCH. We are now interested in developing therapies that take advantage of
these observations. We have also embarked on a gene discovery project in LCH
using DNA from Bernese Mountain Dogs which develop a disease that looks very
similar to LCH.
If you or a family member have histiocytosis
and would like to contact other families in the Boston area about what they
are doing to help fight this disease, please email Sharon
DiNicola or Allyson Condon.
Although this work is now supported by a grant from the NIH, it began with funding
from Team Histio, and continues with the generous help of both Team Histio and
Team Danny Condon - two of the groups that participate in the Boston Marathon®
Jimmy Fund Walk. Anyone interested in contributing to this event should follow
the site links below:
Team
Histio
Team
Danny Condon
Publications
- Fleming MD, Pinkus JL, Alexander SW, Fournier MV, Tam C, Loda M, Sallan
SE, Nichols KE, Carpentieri DF, Pinkus GS, Rollins BJ. Coincident
expression of the chemokine receptors CCR6 and CCR7 by pathologic Langerhans
cells in Langerhans Cell Histiocytosis. Blood 2003; 101:2473-75.
Barrett J Rollins, MD, PhD, Department of Medical Oncology, Dana-Farber Cancer Institute, Barrett J Rollins, MD, PhD, Department of Medical Oncology, Dana-Farber Cancer Institute, Barrett J Rollins, MD, PhD, Department of Medical Oncology, Dana-Farber Cancer Institute |
