1103 Thrombocytopenic purpura associated with common variable immunodeficiency and bronchiectasis

Sunday, 7 December 2014
Exhibition Hall-Poster Area (Sul America)

Thais Martins Souza , Pediatric, Ufjf, Juiz de Fora, Brazil

Luiz Carlos Bandoli Gomes Junior , Pediatric, HU-Ufjf, Juiz de Fora, Brazil

Ana Paula Da Rosa Pereira , Pediatric, Ufjf, Brazil

Barbara Souza Cunto Sobrinho , Pediatric, Ufjf, Brazil

Fernanda Fernandes Fonseca , Pediatric, Ufjf, Brazil

Pedro Carlos Salomão , Pediatric, Ufjf, Brazil

Priscila Rodrigues Silveira Martins , Pediatric, Ufjf, Brazil

Larissa Felizardo Oliveira , Pediatric, Ufjf, Brazil

Patricia Borges Gomes , Pediatric, Ufjf, Brazil

Patricia Cristina Gomes Pinto , Pediatric, Ufjf, Brazil

INTRODUCTION: Common variable immunodeficiency (CVID) is the most prevalent primary immunodeficiency (PID) and progresses with hypogammaglobulinaemia, recurrent infections and other prevalent alterations in humoral immunity. With respect to infections, pulmonary involvement stand and its complications

OBJECTIVE: Describe a case of patient with ICV who started  with thrombocytopenia and evolved early with bronchiectasis.

CASE REPORT: Boy with 13 years old diagnosed with thrombocytopenic purpura and a normal myelogram. He presented low levels of immunoglobulins (A = 7 mg / dl, M = 14,2mg / dl and G = 140 mg / dl). Immunophenotyping of lymphocytes was altered with CD19 = 7.9% (81cells / mm3); CD4 = 56.5% (574cells / mm3) and CD8 = 12.3% (125cells / mm3). The research of vaccine antibodies (tetanus, mumps) was negative and did not produce antibodies against the pneumococcal vaccine. In his personal history was report of pneumonia at age 12 and chicken pox infection with secondary prolonged evolution and secondary bacterial infection. A tomography of the chest showed signs of diffuse bronchiectasis. After excluding other causes of hypogammaglobulinemia and the diagnosis of CVID was initiated monthly treatment with intravenous immunoglobulin and physiotherapy.

CONCLUSIONS: Reiterate the importance of diagnosis of bronchiectasis in patients with CVID, even under a single episode of pneumonia in evolution. These patients may have few signs and symptoms of respiratory and progress to complications, such as bronchiectasis, even in the presence of adequate monitoring. Furthermore, we emphasize the importance of investigating the causes of thrombocytopenic purpura associated with autoimmunity by some patients with CVID.

 BIBLIOGRAPHY

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  2. Park MA, Li JT, Hagan JB, Maddox DE, Abraham RS. Common variable immunodeficiency: a new look at an old disease. Lancet. 2008;372(9637):489-502.