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Immune status of patients with inherited bone marrow failure syndromes.

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?

SUBMITTER: Giri N

PROVIDER: S-EPMC4509864 | biostudies-literature | 2015 Aug

REPOSITORIES: biostudies-literature

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Immune status of patients with inherited bone marrow failure syndromes.

Giri Neelam N Alter Blanche P BP Penrose Keri K Falk Roni T RT Pan Yuanji Y Savage Sharon A SA Williams Marcus M Kemp Troy J TJ Pinto Ligia A LA

American journal of hematology 20150528 8

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 ...[more]

PMID: 25963299

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Inherited Bone Marrow Failure Syndromes

Project description:Inherited Bone Marrow Failure Syndromes

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Inherited Bone Marrow Failure Syndromes

Project description:Inherited Bone Marrow Failure Syndromes

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Neonatal manifestations of inherited bone marrow failure syndromes.

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