Dataset Information

Serum B-Cell Maturation Antigen (BCMA) Levels Differentiate Primary Antibody Deficiencies.

ABSTRACT:

Background

Primary antibody deficiencies (PADs) are the most prevalent primary immunodeficiencies. More severe forms of PADs-common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA)-require immunoglobulin replacement therapy (IRT) and may have serious complications. Differentiating severe PAD from milder hypogammaglobulinemia not requiring IRT can involve prolonged evaluations and treatment discontinuation. Severe PAD is defined by plasma cell deficiency, but this requires a biopsy to establish. Serum B-cell maturation antigen (sBCMA) is elevated in multiple myeloma, but levels are reduced among patients with myeloma in remission who have hypogammaglobulinemia.

Objective

To measure the sBCMA level in 165 subjects to determine whether it differentiates severe PAD-CVID and XLA-from less severe forms not requiring IRT and those without PAD.

Methods

sBCMA, B cells, and tissue plasma cells were measured among subjects with and without PAD, and correlated to clinical and laboratory data.

Results

Subjects with an IgG level of less than 600 mg/dL had reduced sBCMA levels compared with subjects with PAD with IgG levels of greater than or equal to 600 mg/dL and controls without PAD. sBCMA level was lower in patients with CVID and XLA compared with patients with IgA or IgG deficiency and controls. sBCMA level correlated with gastrointestinal plasma cells. sBCMA level of less than 15 ng/mL had 97% positive predictive value for CVID or XLA, whereas 25 ng/mL or more had an 88% negative predictive value.

Conclusions

sBCMA level is profoundly reduced in patients with severe PAD, including those with CVID and XLA and those with IgG levels of less than 600 mg/dL. sBCMA level measurement has potential to augment clinical evaluation of PAD. Prospective studies are needed to evaluate sBCMA for new PAD diagnosis and determine the necessity of IRT.

SUBMITTER: Maglione PJ

PROVIDER: S-EPMC6980522 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature

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Publications

Serum B-Cell Maturation Antigen (BCMA) Levels Differentiate Primary Antibody Deficiencies.

Maglione Paul J PJ Ko Huaibin M HM Tokuyama Minami M Gyimesi Gavin G Soof Camilia C Li Mingjie M Sanchez Eric E Chen Haiming H Radigan Lin L Berenson James J Cunningham-Rundles Charlotte C

The journal of allergy and clinical immunology. In practice 20190817 1

<h4>Background</h4>Primary antibody deficiencies (PADs) are the most prevalent primary immunodeficiencies. More severe forms of PADs-common variable immunodeficiency (CVID) and X-linked agammaglobulinemia (XLA)-require immunoglobulin replacement therapy (IRT) and may have serious complications. Differentiating severe PAD from milder hypogammaglobulinemia not requiring IRT can involve prolonged evaluations and treatment discontinuation. Severe PAD is defined by plasma cell deficiency, but this re ...[more]

PMID: 31430592

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Dataset Information

Serum B-Cell Maturation Antigen (BCMA) Levels Differentiate Primary Antibody Deficiencies.

Background

Objective

Methods

Results

Conclusions

Publications

Serum B-Cell Maturation Antigen (BCMA) Levels Differentiate Primary Antibody Deficiencies.

Similar Datasets

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