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

doi:10.1002/ajh.24046

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

Research output: Contribution to journal › Article › peer-review

20 Scopus citations

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 language	English (US)
Pages (from-to)	702-708
Number of pages	7
Journal	American Journal of Hematology
Volume	90
Issue number	8
DOIs	https://doi.org/10.1002/ajh.24046
State	Published - Aug 1 2015
Externally published	Yes

ASJC Scopus subject areas

Hematology

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title = "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",

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

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T1 - Immune status of patients with inherited bone marrow failure syndromes

AU - Giri, Neelam

AU - Alter, Blanche P.

AU - Penrose, Keri

AU - Falk, Roni T.

AU - Pan, Yuanji

AU - Savage, Sharon A.

AU - Williams, Marcus

AU - Kemp, Troy J.

AU - Pinto, Ligia A.

PY - 2015/8/1

Y1 - 2015/8/1

N2 - 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

AB - 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

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Immune status of patients with inherited bone marrow failure syndromes

Abstract

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