Disorders of the Mediastinum: Student/Resident Case Report Poster - Disorders of the Mediastinum |

What’s the Good Word? A Case of Immunodeficiency With Thymoma FREE TO VIEW

Sumeet Soni, MBBS; Brent Kaufmann, MD
Author and Funding Information

University of Illinois College of Medicine Urbana-Champaign, Urbana, IL

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):555A. doi:10.1016/j.chest.2016.08.643
Text Size: A A A
Published online

SESSION TITLE: Student/Resident Case Report Poster - Disorders of the Mediastinum

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Good syndrome was first described by Dr. Robert Good in 1954 as a rare cause of immunodeficiency associated with a thymoma. It is associated with absent or reduced B cells and CD 4+ T cell dysfunction.

CASE PRESENTATION: A 56 year old male presented to the clinic with nasal congestion for a few weeks and cough with fever for a few days. He was found to have cold sores on his lips and cellulitis of the right knee, right anterior chest and medial lower abdomen. The patient was started on amoxicillin-clavulanic acid for 2 weeks along with acyclovir. He returned in 2 weeks with acute sinusitis and persisting productive cough and was prescribed another 3 weeks of antibiotics. He returned a month later with worsening symptoms and right sided chest pain. Chest X ray showed bilateral infiltrates and was started on levofloxacin. He presented to the ED 2 days later with shortness of breath and hemoptysis. He was also found to have oral thrush. CT chest showed bilateral infiltrates in lower lobes and 9.3 X 8.1 cm right sided mass. Given his recurrent infections, immunoglobulin panel was ordered which showed reduced levels of IgG 190(650-1650), IgA 33.7(85-543) and IgM 12.6(39-276). Immunoglobulin profile showed absent CD19+ B cells and a low CD4:CD8 ratio. The patient tested negative for HIV. Bronchoscopy was done and BAL was positive for CMV PCR and Haemophilus influenzae. Given his immunocompromised state suppressive antibiotic therapy, valgancyclovir and IVIG infusions were initiated. The patient underwent right thoracotomy and an encapsulated mediastinal mass was removed. Pathology revealed thymoma AB spindle type. The patient continues to require IVIG infusions and adjuvant radiotherapy is being considered for possible microscopic invasion.

DISCUSSION: Patients with Good syndrome usually present with sinopulmonary infections in the 4th or 5th decade of life. Thymoma is usually picked up on chest X ray but in 20-25% of the cases is only diagnosed on CT chest. Pathogenesis is poorly understood but is thought to involve a defect in the bone marrow affecting B cell production. However unlike other humoral immunodeficiency diseases, patients with Good syndrome are prone to opportunistic infections as well due to T cell dysfunction. Thymoma associated with Good syndrome is usually a well encapsulated spindle type thymoma. Treatment involves removing the mass, although removal of the tumor does not correct the immunological deficiencies. These patients usually require long term immunoglobulin replacement and aggressive treatment of any infection.

CONCLUSIONS: Good syndrome should be considered as a part of the differential diagnoses in any patient over the age of 40 presenting with recurrent infections. Early detection and aggressive management is necessary to reduce morbidity and complications.

Reference #1: Kelleher, P; Misbah, SA. What is Good’s Syndrome? Immunological abnormalities in patients with thymoma.Journal of Clinical Pathology2003;56:12-16.

DISCLOSURE: The following authors have nothing to disclose: Sumeet Soni, Brent Kaufmann

No Product/Research Disclosure Information




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543