Sunday, 7 December 2014
Exhibition Hall-Poster Area (Sul America)
Introduction: The CVI is the second most common primary immunodeficiency. The prevalence of CIV in the world is approximately 1:25,000. The age of presentation of ICV has peaks in the first decade if life and the beginning of the third decade. Both sexes are affected. Diagnostic criteria according to ESID/PAGID are: - recurrent infections; -age more 4 years; -reduced levels of IgG; decrease of IgA and/or IgM; exclusion of the other causes of hypogammaglobulinemia; - no isohemaglutininas and response to the vaccine. Five clinical phenotypes are described: only infections, autoimmunity, polyclonal lymphocytic infiltration, malignancy and enteropathy. The treatament of patients with CVI is performed with the use of an infusion of immunoglobulin. Objective: to report a patient with CVI associated with infections/ infestations recurrent and splenomegaly. Case description: H.S.M.A, male, 8 years old, eight pneumonia, low weight gain, infestation by Giardia lamblia and splenomegaly (physical examination and ultrasound). Tests: HIV ELISA negative. IgG=614mg/dl; IgM=19mg/dl; IgA=24mg/dl; CD4=24 (742mm3); CD8=70% (2150/mm3) and CD19=247/mm3. Lymphocyte B total=9.2%. Discussion: the CVI can be associated with some phenotypes, such as the occurrence of infections/infestations recurrent and splenomegaly. The levels of immunoglobulins are below the 3 percentile for age, the percentage of b lymphocytes in the minimum threstold, CD8 high. Conclusion: reiterate the importance of the diagnosis of primary immunodeficiency in patients with infections/infestations recurrent splenomegaly and in this case the CVI by important prevalence.
Bibliography
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- Chapel H, Cunningham-Rundles C. Update in understanding common variable immunodeficiency disorders (CVIDs) and the management of patients with these conditions. British journal of haematology. 2009;145(6):709-27.