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Langerhans Cell Histiocytosis: A Two-Punch Therapy Shows Promise Against This Rare Cancer

Langerhans Cell Histiocytosis: A Two-Punch Therapy Shows Promise Against This Rare Cancer
The combined therapy led to a significant decrease in both diseased LCH cells and infiltrating T cells.

Chemotherapy cures fewer than half of patients with Langerhans Cell Histiocytosis. This combination therapy offers a new hope to those whose disease has relapsed. Read on to know more about this rare cancer.

Written by Longjam Dineshwori |Updated : July 1, 2021 12:26 PM IST

Langerhans cell histiocytosis (LCH) is a rare cancer involving dendritic cells, a type of white blood cell that usually helps fight infections. These cells are especially found in the skin, lymph nodes, spleen, lungs, liver, and bone marrow. LCH is often diagnosed in childhood, usually between ages 2 and 3, but can appear at any age. While Langerhans cell histiocytosis is considered a form of cancer by many researchers, this classification remains controversial. Chemotherapy is the current standard of care for LCH, but it cures fewer than half of patients. Now, a new two-punch therapy developed by researchers at Baylor College of Medicine has shown promising results in an animal study.

Dr. Rikhia Chakraborty, assistant professor of pediatrics -- hematology and oncology at Baylor College of Medicine, and her team have found a new therapeutic option for LCH. The new approach uses immunotherapy to target the PD-1 receptors (an immune checkpoint protein that turns the T cells off, preventing an attack on the cancer cells) in combination with MAPK inhibition to target the dendritic cells that cause the disease.

Using murine models of LCH, the researchers measured the response to MAPK inhibitor treatment alone, anti-PD-1 treatment alone and the combination of the two treatments.

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The MAPK inhibitor treatment led to a reduction in diseased LCH cells but not infiltrating T cells. The opposite was observed in the anti-PD-1 treatment a reduction in infiltrating T cells but not the diseased LCH cells. But the combined therapy led to a significant decrease in both diseased LCH cells and infiltrating T cells. The results of the study are published in the journal Blood.

Based on these findings, Chakraborty noted that the combined MAPK and checkpoint inhibitors could be studied as a potential therapeutic strategy in future pre-clinical and clinical trials for LCH. The combination therapy could offer hope to patients whose disease has relapsed as well as reduce toxicity seen with the current standard of care, she said.

Signs and symptoms of Langerhans cell histiocytosis

In approximately 80 percent of Langerhans cell histiocytosis patients, one or more tumours, called granulomas, develop in the bones, causing pain and swelling. Such tumours that usually occur in the skull or the long bones of the arms or legs, may even cause the bone to fracture.

The granulomas that occur in the skin may appear as blisters, reddish bumps, or rashes which can be mild to severe. Langerhans cell histiocytosis can also affect the pituitary gland, leading to delayed or absent puberty or an inability to have children (infertility). Pituitary gland damage may also cause diabetes insipidus (production of excessive amounts of urine) and thyroid dysfunction.

In some cases, LCH can cause damage to the lungs, liver, or blood-forming (hematopoietic) system, which may be life-threatening. Lung involvement can lead to stiffening of the lung tissue, breathing problems, and increased risk of infection. Hematopoietic involvement can lead to pancytopenia (reduction in the number of blood cells). This can cause symptoms like fatigue, frequent infections, and clotting problems.

Depending on which organs and tissues are affected, other signs and symptoms of LCH include swollen lymph nodes, abdominal pain, yellowing of the skin and whites of the eyes (jaundice), delayed puberty, protruding eyes, dizziness, irritability, and seizures. Deterioration of neurological function is also observed in about 1 in 50 affected individuals.