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GOOD OR NOT GOOD? A NOVEL CASE OF CHRONIC DIARRHEA ASSOCIATED WITH HYPOGAMMAGLOBULINEMIA AFTER THYMECTOMY

T Xenodemetropoulos, M Messieh, J Ramsay, LWC Liu
Department of Medicine, Divisions of Gastroenterology and Immunology, Department of Pathology, McMaster University, Hamilton, Ontario

Immunodeficiency in association with thymoma (Good's syndrome) is a rare cause of increased susceptibility to opportunistic microbial infection and is commonly defined as the presence of hypogammaglobulinemia and reduced/absent B-cells in patients with thymoma, although variability in diagnostic characteristics exists. We describe a case of an 80-year-old man with a past history of thymectomy for a spindle/lymphocytic cell thymoma who presented with a 15 month period of severe, non-hemorrhagic diarrhea and malabsorption. Serological investigations were notable for hypogammaglobulinemia (IgA=0.43, IgG=2.15 and IgM<0.18), slight CD3 T-cell elevation and mild CD19 B-cell reduction with an otherwise normal lymphocyte subset analysis. Proper B-cell function was confirmed with normal serum tetanus and C pneumoniae antibody titers. Phytohemagglutinin lymphocyte stimulation testing was normal. He was initially diagnosed with small bowel bacterial overgrowth (SBBO) by breath test and Crohn's Disease (CD) by colonoscopic histology. However, he was unresponsive to antibiotics for SBBO and immunosuppressive and 5-aminosalicylate therapy for his CD. He later progressed to severe malnutrition and anasarca, requiring total parental nutrition. Repeat esophogastroduodenoscopy and pathology demonstrated evidence of Celiac's sprue that was refractory to gluten-free diet. The patient was successfully treated with IVIg therapy, achieving gradual symptomatic resolution and endoscopically-confirmed normalization of intestinal mucosa.
The clinical presentation thus described may represent a unique immunological variant of Good's syndrome not previously described in the current literature. To the best of our knowledge, this is also the first documentation of a single patient with Good's syndrome presenting with both Celiac and Crohn's Disease gastrointestinal pathology. Although the mechanism for these morphological changes remains unclear, the occurrence of both of these histopathologies in our patient suggests the possibility of common immunological pathways in these diseases pathological manifestations.

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