Hyper IgM Syndrome

What is hyper IgM syndrome?

If your child has hyper IgM syndrome, he or she has elevated levels of serum IgM antibodies and reduced levels of IgG and IgA antibodies in their blood. This happens because your child’s T cells and B cells—both types of immune cells—don’t cooperate. The most common form of hyper IgM syndrome is found only in males, but less common forms can be found in both male and female patients. Symptoms of hyper IgM syndrome include recurrent bacterial infections and pneumonia infections in newborn infants.

How do we treat hyper IgM syndrome at Stanford Medicine Children’s Health?

The only curative therapy for hyper IgM syndrome is stem cell transplantation from a healthy identical related, mismatched related, or unrelated donor.

Using a matched donor reduces the risk of a serious complication called graft-versus-host disease (GVHD), in which the donated immune cells attack the patient’s tissues. But using a technique pioneered by Stanford’s Alice Bertaina, MD, PhD, we can now remove T cells that cause GVHD from stem cells before they are given to the recipient. As a result, your child can safely receive a transplant using stem cells from a mismatched donor.

Your child will receive chemotherapy before transplantation to allow the donor stem cells to engraft. Your child will likely be discharged from the hospital 30-40 days after transplantation when he or she has developed adequate immune and blood function.