Is Hypogammaglobulinemia a Constant Feature in Good's Syndrome?

Abstract

Thymomas are rare tumors, which can be associated to a variety of paraneoplastic syndromes, including a fatal hypogammaglobulinemia, namely Good's Syndrome (GS). Although the combination of thymoma and hypogammaglobulinemia is regarded as sufficient for diagnosis of Good's syndrome, some thymoma patients with a clear clinical picture of immunodeficiency present normal levels of immunoglobulins. We describe the case of a patient, with a 20-year history of thymoma, who underwent several operations and lines of chemotherapy, and suffered from recurrent infections, including one rare skin infection from Pseudoallescheria boydii. The patient constantly presented normal levels of gammaglobulins.

Practice parameter for the diagnosis and management of primary immunodeficiency

“…''Common variable immunodeficiency with thymoma,'' ''immunodeficiency with thymoma,'' and ''Good syndrome'' all denote a form of adult-onset hypogammaglobulinemia/agammaglobulinemia in association with thymoma. 376,377 The spectrum of bacterial sinopulmonary infections and pathogens is similar to that associated with the more prevalent form of CVID. However, Good syndrome is associated more frequently with opportunistic infections, including mucocutaneous candidiasis, severe varicella infection, PCP, CMV, and recurrent HSV.…”

“…Autoimmune disease is a frequent complication of Good syndrome, most notably pure red cell aplasia and neutropenia. 376,377 Patients with Good syndrome often have chronic diarrhea of unclear cause.…”

“…Panhypogammaglobulinemia is a consistent finding in patients with Good syndrome. 376,377 Unlike the majority of patients with CVID, immunophenotypic analysis of peripheral blood lymphocytes frequently shows absent or very low numbers of B cells, reduced CD4 1 T-cell counts, absent cutaneous delayed hypersensitivity responses, and a reduced in vitro T-cell response to mitogen.…”

Good's syndrome and IgA monoclonal gammopathy of undetermined significance

“…The most frequent are mucocutaneous candidiasis, herpes zoster and recurrent herpes simplex virus infections. Hypogammaglobulinaemia is considered as a characteristic feature of GS, although some patients with a thymoma and a clear clinical picture of immunodeficiency have been described with a normal immunoglobulin plasmatic level 8. The level of gammaglobulins seems to be a good predictor of immunity.…”

Ectopic Cervical Thymoma Accompanied by Good's Syndrome

“…Although there remains some controversy, GS is currently defined as immunodeficiency with thymoma, most commonly characterized by hypogammaglobulinemia. 1) The incidence of hypogammaglobulinemia is reported as 6%-11% in patients with thymoma in English literature, 2) while that is reported as 0.2% in Japanese patients. 3) This difference might be explained by the racial difference in incidence and/or the definition of the disease.…”