Cancer Treatment Today

Children with Down syndrome have an increased risk of developing leukemia, both myeloid and acute lymphoblastic leukemia (ALL). historically children with Down syndrome treated with risk-stratified treatment protocols for ALL have had worse outcomes than children without Down syndrome. The poorer outcome in the Down syndrome patient population has been attributed to several factors including inherent differences in tumor biology, increased therapy-related toxicities, and higher rate of infectious complications. It has long been known that children with Down’s syndrome have reduced immunoglobulin levels and that they can be increased with zinc and selenium supplementation.

It has been conjectured that adding IVIG would improve outcomes but that had not been confirmed in studies. Whitlock suggests, in an unreferenced statement, that IVIG should be considered for children with Downs’s syndrome and ALL, but experimental support is lacking. I have not found any recent studies that have investigated this this issue. A O88 study by Graham-Pole suggested that all patients with ALL can benefit from IVIG, but this has not been accepted a standard of care. Similarly, Gimeis at al looked at 60 patients who received IVIG and thought that it benefited them – again, the field had not accepted this as standard of care.

• Nishida Y, Sano Y, Akaoka I, Maruki M, Suzuki T, Maruki K: “Abnormal serum immunoglobulin levels in Down’s syndrome patients.” Am J Ment Defic 1978 Jul;83(1):16-20
• Anneren G, Magnusson C G, Nordvall S L: “Increase in serum concentrations of IgG2 and IgG4 by selenium supplementation in children with Down’s syndrome.” Arch Dis Child 1990 Dec;65(12):1353-5
• Whitlock JA: Down syndrome and acute lymphoblastic leukaemia. Br J Haematol 135 (5): 595-602, 2006.
• Arico M, Ziino O, Valsecchi MG, et al.: Acute lymphoblastic leukemia and Down syndrome: presenting features and treatment outcome in the experience of the Italian Association of Pediatric Hematology and Oncology (AIEOP). Cancer 113 (3): 515-21, 2008.
• Buitenkamp TD, Izraeli S, Zimmermann M, et al.: Acute lymphoblastic leukemia in children with Down syndrome: a retrospective analysis from the Ponte di Legno study group. Blood 123 (1): 70-7, 2014.
• Graham-Pole J, Camitta B, Casper J, Elfenbein G, Gross S, Herzig R, Koch P, Mahoney D, Marcus R, Munoz L, et al. Intravenous immunoglobulin may lessen all forms of infection in patients receiving allogeneic bone marrow transplantation for acute lymphoblastic leukemia: a pediatric oncology group study. Bone Marrow Transplant. 1988 Nov;3(6):559–566.