CRA72 COMMON VARIABLE IMMUNODEFICIENCY COMPLICATED BY FULMINANT STREPTOCOCCAL SEPSIS AND MULTIPLE CUTANEOUS ABSCESSES: A CASE REPORT

Sarvin Vignesh Murugayah¹, Rong Lih Ho¹, Larry Nyanti^1,2, K Tharmaraj K Kunaraj¹, Pin Jin Yong³, Shan Min Lo¹, Hema Yamini Ramarmuty¹, Kunji Kannan Sivaraman Kannan¹

¹Department of Respiratory Medicine, Hospital Queen Elizabeth, Sabah.
²Medical Department, Faculty of Medicine and Health Sciences, University Malaysia Sabah, Sabah.
³Medical Department, Hospital Labuan, Sabah.

Introduction
Primary Immunodeficiency Disorders (PIDs) are rare genetic disorders that impairs immunity leading to recurrent infections typically involving the respiratory tract, gastrointestinal tract and skin. Up to 60% of these patients manifest with thoracic abnormalities.

Case Report
A 14-year-old girl presented with recurrent cutaneous abscesses and chronic cough. Biohazard and diabetic screening were negative. CT thorax revealed right middle and lower lobe collapse with bilateral cystic bronchiectasis. Flexible bronchoscopy demonstrated whitish purulent secretions, though lavage results were negative. The patient underwent TBNA of station 7 lymph node and endobronchial biopsy of endoluminal nodule, complicated by severe bleeding. The bleeding was successfully managed with embolization of the right bronchial artery and blood transfusion. She developed Streptococcus pneumoniae bacteraemia which was treated with antibiotics. Low immunoglobulin levels (IgG <1.09, IgM 0.07) confirmed PID. The patient was commenced on Intravenous Immunoglobulin (IVIG) therapy and discharged well under room air.

Discussion
Recurrent cutaneous and thoracic infection with CT evidence of bronchiectasis in a post-pubertal patient should raise the suspicion of CVID, although initial suspicions may revolve around tuberculosis and malignancy. Rapid detection and initiation of treatment helped improve patient’s outcome in this case. Severe bleeding may have resulted from friable airways due to ongoing infection and the lack of ultrasonographic guidance during lymph node TBNA.

Conclusion
Although rare, PID should be suspected in young patients presenting with frequent infections with no evidence of other immunocompromising illnesses.