Addine RBC, Casado HI, Marrón GR, Hidalgo FM

Language: Spanish
References: 0
Page: 853-861
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ABSTRACT

Bruton agammaglobulinemia is a rare disease that is transmitted linked to the X chromosome. It manifests as a humoral deficit, with decrease of all the isotypes of antibodies and the population of B lymphocytes, associated with recurrent bacterial infections. It is reported with the objective of characterizing Bruton's aganmaglobulinemia and its evolution in an atypical case of Granma province, Cuba. Male patient of 5 years of age, with a history of recurrent infectious diseases and with low levels of immunoglobulin G and normal values of the rest of the antibody isotopes. A quantification of cell populations was performed by flow cytometry, confirming an almost total deficit of B lymphocytes. Bruton agammaglobulinemia was diagnosed, with an atypical presentation, marked by dermatological clinical pictures and a selective deficit of immunoglobulin G. The patient evolved favorably after being treated with human gamma globulin substitutive.