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Not the Typical Pneumonia: An Unusual Case of Immunodeficiency FREE TO VIEW

Jamie Berke, MD; Claudia Halaby, MD
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Winthrop University Hospital, Mineola, NY

Chest. 2013;144(4_MeetingAbstracts):971A. doi:10.1378/chest.1700267
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SESSION TITLE: Miscellaneous Student/Resident Case Report Posters II

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM

INTRODUCTION: Recurrent pneumonia is defined as having at least 2 pneumonia episodes in 1 year, or more than 3 at any time, with radiographic clearing between episodes. 1 Children with recurrent pneumonia present a diagnostic challenge as they frequently have an underlying illness such as aspiration pneumonia secondary to oropharyngeal incoordination, an immune disorder, congenital heart disease, asthma, respiratory system anomalies, or gastroesophageal reflux. 2

CASE PRESENTATION: A 15 year old male was referred to the Pediatric Pulmonary Clinic with symptoms of persistent cough, wheezing and chest congestion, treated with antibiotics and bronchodilators. The patient emigrated from El Salvador three years ago. He was healthy until he was 8 years old, when he had developed pneumonia with pleural effusion, requiring thoracostomy tube placement. In the past 3 years, he has frequently taken antibiotics for clinical pneumonias. Physical examination was significant, as both height and weight were below the third percentile. He was febrile, with diffuse rhonchi. An outpatient workup ruled out cystic fibrosis, but revealed pan-hypogammaglobulinemia. He was admitted for antibiotic treatment, immunoglobulin replacement therapy and further workup. A chest x-ray on admission revealed a 1.5cm nodule in the right lung base. A chest CT identified focal areas of consolidation suspicious for pneumonia and a nodule in the right lower lobe. Flow cytology had normal T lymphocyte subclasses, and almost absent B lymphocytes (2.6%).

DISCUSSION: Primary pan-hypogammaglobulinemia includes X-linked agammaglobulinemia (XLA), common variable immunodeficiency (CVID), and transient hypogammaglobulinemia of infancy. In all, an impaired antibody response to pathogens leads to increased susceptibility to bacterial infections, especially of the respiratory system. Our patient had clinical and laboratory features of both XLA and CVID. Differentiating CVID from XLA is important. Patients with XLA have respiratory infections starting in the first year of life, almost absent B lymphocytes and immunoglobulins, and close to normal life expectancy. Patients with CVID have respiratory infections usually later in childhood, but a high incidence of autoimmune diseases and malignancy, requiring intensive monitoring and counseling. Whether it is CVID or XLA, recurrent infections can be fatal, and the treatment is lifelong immunoglobulin replacement therapy.

CONCLUSIONS: When an immune deficiency is the cause of recurrent pneumonia, establishing the diagnosis is important as the long term prognosis varies. Differentiation between CVID and XLA in this case requires further genetic testing (identification of BTK gene mutation - the hallmark of XLA)

Reference #1: Wald E Recurrent and non-resolving pneumonia in children. Semin Respir Infect. 1993;846- 58

Reference #2: Abdullah F. Owayed et all, Underlying Causes of Recurrent Pneumonia in Children. Arch Pediatr Adolesc Med. 2000;154(2):190-194. doi:10.1001/archpedi.154.2.190.

DISCLOSURE: The following authors have nothing to disclose: Jamie Berke, Claudia Halaby

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