Good's Syndrome Patients Hospitalized for Infections

Abstract

Good's syndrome (GS) is a rare combination of thymoma and hypogammaglobulinemia, resulting in immunodeficiency. Patients with GS are highly susceptible to bacterial infection, particularly encapsulated bacterial infection in upper and lower respiratory tracts. Good's syndrome patients with moderate-to- severe infection are often hospitalized. Clinical features of GS patients remain to be characterized.Patients with the discharge diagnosis of GS and simultaneous infection from Peking Union Medical College Hospital between January 2001 and July 2015 were retrospectively analyzed.Among 14 hospitalized GS patients, 12 of them were admitted for severe infections. Mean patient age was 56.7 + 10.1 years. Average concentrations of serum IgG, IgA, and IgM were 2.3 + 1.9 g/L, 0.28 + 0.28 g/L, and 0.06 + 0.07 g/L, respectively. Respiratory and intestinal tracts were the most common sites for infection, which occurred in 7 and 4 patients, respectively. Pathogens identified in 10 patients included cytomegalovirus in 5 patients, Pneumocystis jirovecii, Clostridium difficile in 2 patients, Klebsiella pneumonia in 2 patients, and Streptococcus pneumonia and Hemophilus influenza in 1 patient. Ten patients were treated with antibiotics and immunoglobulin replacement. Only 1 patient who was on immunosuppressant therapy died from P. jirovecii pneumonia.Infection was the most frequent cause for hospitalization of GS patients. Both respiratory and intestinal tracts were the most common sites of infection. Cytomegalovirus and P. jirovecii represented 2 common opportunistic pathogens isolated from hospitalized GS patients with infections.

Clinical and laboratory features of seventy-eight UK patients with Good’s syndrome (thymoma and hypogammaglobulinaemia)

“…Most patients (94%) presented with infections and nearly half (45%) had radiological evidence of bronchiectasis. Although there were numerical reductions of T cells in a number of patients, infections usually associated with T cell deficiencies were not a prominent feature of this cohort, which is at odds with reported smaller studies [17,18]. Three patients had candidiasis, but antibiotics may have contributed to this problem.…”

Role of Chemokine Signalling in the Pathogenesis of Good’s Syndrome-Case Reports, Clinical Characterization from Single-Centre Perspective

“…GS is rare combined immunodeficiency, but pathological characteristic is difficult because thymectomy usually precede immunological examination, coexistence between thymoma and other abnormalities may be result of immunodefect after thymectomy and opportunistic infections. B and T cell immunodysregulation in thymoma patients is sometimes referred to as GS [4,20], but it represents rather a different disease with distinct etiology and pathogenesis where B cell lymphopenia due to differentiation arrest seems to be crucial. Weak definition of GS is source misconception: severe hipogammaglobulinaemia or decrease of all isotypes is not sometimes observed [4,20], the humoral type spectrum of opportunistic infections was therefore described [20].…”

A Good Case of Recurrent Pneumonia

“…4 A mean age at the time of diagnosis is 59.1 years (12-90 years). 6 In 2.4% of patients who first presented with thymoma, 37.9% presented with both thymoma and infection, whereas only 19.7% presented with infection. 7 Autoimmunity is a common phenomenon in patients with Good’s syndrome.…”