Question:
My daughter was originally diagnosed with B Cell Lymphopenia when she was 4 after recurrent sinus infections and pneumonia, put on prophylactic antibiotics and IVIG/SCIG for about 4 years. She was off the IG treatments for about 4 years and then got a really nasty round of pneumonia a few years ago and spent almost a month in the hospital. (Before then, her longest stay in the hospital for pneumonia had been 10 days.) They changed her prophylactic antibiotic and put her back on SCIG. She’s now 18 and still on SCIG and prophylactic antibiotics which has kept her healthy for the most part. I’m just curious if this could be indicative of it being a life-long issue? At one point one early on, one of the immunologists said one of the numbers in her blood work made it look like she could need infusions for life but before she went into the hospital, most of her numbers were in the normal range. I fully trust our immunologists but know they don’t specialize in Down syndrome so would love to have information from someone who specializes in individuals with Down syndrome so that I might be able to take that information back to the immunologist in case it was something they might not have picked up on.
Answer:
Thank you for your question. I am sorry to hear of your daughter’s challenges.
B cell lymphopenia is a decrease in a subset of white blood cells called lymphocytes. More information is available on the NIH website.
We found several articles (see below) describing lymphocyte deficiency in people with DS but no articles describing use of IVIG (intravenous immunoglobulin) or SCIG (subcutaneous immunoglobulin) in people with DS for B cell lymphopenia. Specifically, we could not find information answering the question regarding deciding on how long people with DS should be treated with IVIG or SCIG or what lab parameters can guide that decision in people with DS. Therefore, if we had a patient with this condition, we would defer to the treating immunologist.
Articles describing lymphocyte deficiency:
- Immune Dysregulation in Children with Down Syndrome – a good but quite technical summary of immune issues in people with DS
- Intrinsic Abnormalities of Lymphocyte Counts in Children with Down Syndrome
One additional consideration is whether zinc levels have been checked. Zinc deficiency can contribute to immune dysfunction and zinc supplement may be helpful in some individuals with DS, but the studies are older and had mixed results (see below). It seems quite unlikely that zinc supplementation would improve the immune system enough to be a deciding factor in stopping IVIG or SCIG for someone with immune deficiency significant enough to require those treatments.
- Immune Dysfunction in Down’s Syndrome: Primary Immune Deficiency or Early Senescence of the Immune System?
- Oral Zinc Supplementation in Down’s Syndrome Subjects Decreased Infections and Normalized Some Humoral and Cellular Immune Parameters
- Immunodeficiency and Plasma Zinc Levels in Children with Down’s Syndrome: A Long-Term Follow-Up of Oral Zinc Supplementation