Hyper IgM Syndrome

What is Hyper IgM syndrome?

Patients with Hyper IgM syndrome have elevated levels of serum IgM immunoglobulins and reduced levels of IgG and IgA immunoglobulins in their blood. This happens because the patient’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 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, patients can be safely transplanted using stem cells from a mismatched donor.

Patients receive chemotherapy prior to transplantation to allow the donor stem cells to engraft. Patients are usually discharged from the hospital 30 – 40 days after transplantation when they have developed adequate immune and blood function.