Immunodeficiencies Caused by B-Cell Defects

Bonilla, Francisco A.; Geha, Raif S.

doi:10.1016/b978-012053641-2/50027-7

Cited by 1 publication

(2 citation statements)

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“…Noninfectious complications have been described for XLA, including a higher incidence of juvenile rheumatoid arthritis, aseptic polyarthritis, sensorineural hearing loss, and colorectal cancer (12,20,75). Autoimmune diseases such as Crohn's disease and type I diabetes mellitus have been described for a few patients with XLA, albeit much less frequently than for CVID (15). The occurrence of a dermatomyositis-like syn-VOL.…”

Section: Primary Antibody Deficiencies Agammaglobulinemiasmentioning

confidence: 99%

“…Evidence for an increased susceptibility to mycoplasma infections in PAD patients is the increased incidence of pneumonia caused by Ureaplasma urealyticum, which is rarely associated with nonurogenital infections in immunocompetent hosts (117). In rare cases of pneumonia in patients with XLA or CVID, other organisms have been isolated, including Pneumocystis jirovecii, Mycobacterium hominis, Mycobacterium avium, and adenovirus (15,32,114,141). A case of measles pneumonia, which developed after exposure to wild-type virus, has been reported for a patient with XLA who had absent B cells (141).…”

Section: Respiratory Infectionsmentioning

confidence: 99%

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Pathogenesis, Diagnosis, and Management of Primary Antibody Deficiencies and Infections

2009

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SUMMARY Primary antibody deficiencies are the most common primary immunodeficiency diseases. They are a heterogeneous group of disorders with various degrees of dysfunctional antibody production resulting from a disruption of B-cell differentiation at different stages. While there has been tremendous recent progress in the understanding of some of these disorders, the etiology remains unknown for the majority of patients. As there is a large spectrum of underlying defects, the age at presentation varies widely, and the clinical manifestations range from an almost complete absence of B cells and serum immunoglobulins to selectively impaired antibody responses to specific antigens with normal total serum immunoglobulin concentrations. However, all of these disorders share an increased susceptibility to infections, affecting predominantly the respiratory tract. A delay of appropriate treatment for some diseases can result in serious complications related to infections, while timely diagnosis and adequate therapy can significantly decrease morbidity and increase life expectancy and quality of life.

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