Immune status of patients with inherited bone marrow failure syndromes

Neelam Giri, Blanche P. Alter, Keri Penrose, Roni T. Falk, Yuanji Pan, Sharon A. Savage, Marcus Williams, Troy J. Kemp, Ligia A. Pinto

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Abstract

Immune function abnormalities have been reported in patients with Fanconi anemia (FA), dyskeratosis congenita (DC) and, rarely, in Shwachman-Diamond syndrome (SDS), and Diamond-Blackfan anemia (DBA), but large systematic studies are lacking. We assessed immunological parameters in 118 patients with these syndromes and 202 unaffected relatives. We compared the results in patients with reference values, and with values in relatives after adjusting for age, sex, corticosteroid treatment, and severe bone marrow failure (BMF). Adult patients (≥18 years) with FA had significantly lower immunoglobulins (IgG, IgA and IgM), total lymphocytes, and CD4 T cells than reference values or adult relatives (P

Original languageEnglish (US)
Pages (from-to)702-708
Number of pages7
JournalAmerican Journal of Hematology
Volume90
Issue number8
DOIs
StatePublished - Aug 1 2015
Externally publishedYes

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ASJC Scopus subject areas

  • Hematology

Cite this

Giri, N., Alter, B. P., Penrose, K., Falk, R. T., Pan, Y., Savage, S. A., Williams, M., Kemp, T. J., & Pinto, L. A. (2015). Immune status of patients with inherited bone marrow failure syndromes. American Journal of Hematology, 90(8), 702-708. https://doi.org/10.1002/ajh.24046