Found the cause of a rare disease (LCH)
Found the cause of a rare disease (LCH).
Swedish researchers have identified the origins of the cancer-like disease, Hand-Schüller-Christian LCH or Langerhans cell histiocytosis (LCH).
Researchers at Karolinska Institutet, in collaboration with Karolinska University Hospital, Stockholm, have identified the origins of Langerhans’ cell histiocytosis, a rare pathology involving the irregular proliferation of mononuclear dendritic cells, involving many organs and parts of the body (spleen , liver, lung, central nervous system, hematopoietic system, skin, but also teeth and bones). The findings, presented in Science Immunology, could lead to new targeted treatments.
LCH is a serious type of cancer-like disease that mostly affects children and can be fatal in severe cases. According to Swedish data, about five to ten children fall ill with this disease every year, usually before the age of ten. “The origin of LCH cells has been debated for decades. Some researchers believe it comes from a certain type of immune cell called dendritic cells , while others believe it comes from related cells called monocytes says Egle Kvedaraite, a physician and researcher in the Department of Medical Biochemistry and Biophysics at Karolinska Institutet and first author of the new study. Researchers from Karolinska Institutet, together with scientists from the Singapore Immunology Network and Newcastle University, have now succeeded in proving that both theories are close to the truth: They have combined so-called single cell sequencing, sample microscopy and cell tracking of patients recruited from, among others, Karolinska University Hospital.
They found that the mutated LCH cells had similar properties to monocytes and dendritic cells, as well as a relatively recent type of dendritic cell, the so-called dendritic cell type 3 (DC3). “We now know that DC3 has a separate developmental pathway distinct from that of other dendritic cells and monocytes, and this knowledge was central to our study,” says Kvedaraite. Researchers have found that the different cell types can communicate with each other to promote the development of LCH, thereby creating a self-reinforcing effect.
“Among the treatment options for Langerhans cell histiocytosis, targeted therapy can be applied successfully, but the disease returns when treatment is stopped. This presents a serious challenge for patients, as a lifetime treatment for children is not a good option due to side effects,” Kvedaraite says. New understanding of the origin of this type of cancer may help develop new targeted treatments.