Case report
Thymoma and immunodeficiency (Good syndrome): a report of 2 unusual cases and review of the literature

https://doi.org/10.1016/S1081-1206(10)60695-3Get rights and content

Background

Good syndrome is a rare cause of combined B- and T-cell immunodeficiency that occurs in association with a thymoma. Patients affected with Good syndrome have increased susceptibility to bacterial, fungal, viral, and opportunistic infections.

Objective

To describe 2 unusual cases of infections in patients with Good syndrome and review the literature.

Methods

Case 1 describes a 51-year-old woman with Good syndrome who presented with a 10-day history of diarrhea, nausea, and fevers. During her hospitalization she became pancytopenic and underwent a bone marrow biopsy and evaluation of her peripheral blood smear. Case 2 describes an 89-year-old man with Good syndrome who presented with a nonhealing leg ulcer, which underwent biopsy. A literature search through MEDLINE was performed. Keywords included Good syndrome, thymoma, hypogammaglobulinemia, immunodeficiency, and infection.

Results

The peripheral blood smear in patient 1 showed ring-formed parasites in red blood cells suggestive of babesiosis. She began treatment with azithromycin, atovaquone, and doxycycline and recovered completely. Patient 2 underwent a biopsy of the foot. Immunohistochemical staining was positive for human herpesvirus 8 consistent with Kaposi sarcoma.

Conclusions

The concomitant occurrence of immunodeficiency and thymoma is known as Good syndrome. In contrast to other humoral immune defects, patients with this syndrome can develop opportunistic infections, and the prognosis appears less favorable compared with X-linked agammaglobulinemia or common variable immunodeficiency. Immunological investigations, including T-cell subsets, B cells, and quantitative immunoglobulins, should be considered part of the routine diagnostic evaluation in patients with a thymoma and recurrent infections.

Section snippets

INTRODUCTION

Good syndrome is a rare association of thymoma and immunodeficiency first described more than 50 years ago. Patients are most commonly between the ages of 40 and 70 years and have a thymoma, low to absent B cells in the peripheral blood, hypogammaglobulinemia, and defects in cell-mediated immunity. As reviewed by Tarr et al,1 patients often present with recurrent infections due to encapsulated bacteria, fungi, and viruses. Herein, we describe 2 patients with Good syndrome and their presentation

METHODS

We searched the literature using the MEDLINE (National Library of Medicine, Bethesda, MD) database. Keywords used in the search included Good syndrome, thymoma, hypogammaglobulinemia, immunodeficiency, and infection. Additional cases were identified using references of publications found. Only articles and abstracts published in English were included.

Case 1

A 51-year-old white woman who was previously healthy presented with a persistent cough, several bouts of pneumonia, and oral ulcerations. A chest x-ray examination in May 2000 revealed a large thymoma, which was resected. Despite the resection, she continued to have oral ulcerations and a cough with shortness of breath. In October 2000, a lung biopsy specimen demonstrated fragments of alveolar parenchyma with large intra-alveolar myxoid tissue plugs consistent with cryptogenic organizing

Definition

The association between the presence of a thymoma and adult-onset hypogammaglobulinemia was first described by Dr Robert Good in 1955.2 There are a number of definitions for Good syndrome. Practice parameters in 20053 define it as a subset of common variable immunodeficiency; however, the reduced numbers of peripheral B cells noted in Good syndrome are not a feature of common variable immunodeficiency, which typically shows impaired B-cell maturation. Others choose to define it as

CONCLUSIONS

In patients with a thymoma, recognition of hypogammaglobulinemia is an important clinical consideration, since the prognosis for patients with Good syndrome appears worse when compared with common variable immunodeficiency or X-linked agammaglobulinemia.29 The major causes of death in patients with Good syndrome include infections, autoimmune diseases, and hematologic complications.1, 12 In addition, the clinical course of disease may be more severe for those patients who require

REFERENCES (29)

  • NG Ryman et al.

    Good's syndrome with primary intrapulmonary thymoma

    J R Soc Med

    (2005)
  • SM Arend et al.

    Good's syndrome: the association of thymoma and hypogammaglobulinemia

    Clin Infect Dis

    (2001)
  • S Ide et al.

    Good's syndrome presenting with cytomegalovirus pneumonia

    Intern Med

    (2000)
  • K Oritani et al.

    Limitin: an interferon-like cytokine that preferentially influences B-lymphocyte precursors

    Nat Med Immunol

    (2000)
  • Cited by (55)

    • Phenocopies of inborn errors of immunity

      2021, Inborn Errors of Immunity: A Practical Guide
    • Precursor B-cell development in bone marrow of Good syndrome patients

      2019, Clinical Immunology
      Citation Excerpt :

      Additionally, murine models have shown a role for interferon-like cytokines such as Limitin, which is produced by bone marrow stromal cell line. This was hypothesized to be involved in BCP differentiation, promoting cell cycle arrest upon impaired differentiation [7]. Aberrancies in BM stromal environment could cause a maturation arrest of the BCP.

    • Pulmonary Complications of Primary Immunodeficiencies

      2015, Murray and Nadel's Textbook of Respiratory Medicine: Volume 1,2, Sixth Edition
    View all citing articles on Scopus
    View full text