Cd40 Ligand

CD40 ligand deficiency: Is there hope?

Based on an article published in The Journal of allergy and clinical immunology written by Mitsui-Sekinaka on March 3, 2015, the overall prognosis of patients with hyper-IgM syndrome that is caused by mutations in the CD40 ligand is not good due to the long term infections that the patient will endure over the course of their life.

Hematopoietic stem cell transplants have been used for many types of diseases and have had a positive outcome. If it can work for other diseases that have similarities with CD40 ligand deficiency then doing the transplant in these type of patients should help boost their immune system to be able to better fight off infections.

The National Defense Medical College, Department of Pediatrics, in Japan enrolled 56 Japanese patients with hyper-IgM, 29 who had received the hematopoietic stem cell transplants and analyzed the data recorded of their health.

The patients that did not receive the stem cell transplant have a poor long-term survival rate. The overall survival rate of patients that received the transplant was significantly higher than those that did not receive it. “Moreover, event-free and disease-free survival rates were significantly greater in patients 5 years old or younger at the time of transplantation than in older patients.”(J Allergy Clin Immunol 2015)

Based off these results, it is to be determined that hematopoietic stem cell transplantation significantly improved the outcome of patients with hyper-IgM and at an age of 5 years or younger would be the best time to administer the transplant. Patients older than 6 have suffered from multiple infections and have been exposed to severe organ damage and that influences the outcome later in life