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Thrombocytopenic purpura associated with common variable immunodeficiency and bronchiectasis

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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.

Consent

Written informed consent was obtained from the patient for publication of this abstract and any accompanying images. A copy of the written consent is available for review by the Editor of this journal.

References

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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. 10.1111/j.1365-2141.2009.07669.x.

  2. 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. 10.1016/S0140-6736(08)61199-X.

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Author information

Correspondence to Thais Martins Souza.

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This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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