Good's Syndrome with a Block in the Early Stage of B Cell Differentiation and Complicated by Campylobacter fetus Sepsis.

Yamazaki, Kohichi; Watanabe, Naomi; Hasegawa, Atsushi; Yamaguchi, Etsuro; Miyamoto, Kenji; Sakiyama, Yukio; Kawakami, Yoshikazu

doi:10.2169/internalmedicine.33.496

Cited by 16 publications

(11 citation statements)

References 11 publications

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“…19 According to the latter hypothesis, T cell dysfunction could result in both thymoma and hypogammaglobulinemia, and may explain the maintenance of GS symptoms after thymectomy. 20 In contrast, oral lichenoid lesions of the GS patient improve after thymectomy.…”

Section: Discussionmentioning

confidence: 98%

Cytokine Profiles Contribute to Understanding the Pathogenic Difference Between Good Syndrome and Oral Lichen Planus

et al. 2015

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We described and analyzed the pathogenic difference between Good syndrome (GS) and oral lichen planus (OLP) in oral mucosa.Good syndrome (GS) is a rare disease characterized by B and T cell immunodeficiency associated with hypogammaglobulinemia and thymoma. GS patients frequently develop oral lichenoid lesions with lymphocytic infiltration beneath the basal layer. Oral lichen planus (OLP) is a chronic inflammatory disease of the oral mucosa characterized by destruction of basal cells by Langerhans cells, macrophages, and T lymphocytes. Although the histological features of the lesions of both diseases are very similar, the pathogenesis of GS in the oral mucosa remains unknown. In this study, we thus investigated the expression of infiltrating lymphocyte subsets (CD3, CD20, CD4, and CD8) and T helper (Th) cytokines including interferon (IFN)-γ (Th1 type), interleukin (IL)-4 (Th2 type), IL-17 (Th17 type), and IL-10 (regulatory T cell type) by immunohistochemistry in buccal mucosa specimens from 2 GS patients compared with 15 OLP patients. All patients showed a predominance of CD3+ T cells over CD20+ B cells, and CD4+ Th cells over CD8+ cytotoxic T cells. This polarization was especially prominent in GS. IFN-γ and IL-10 were strongly detected in the infiltrating lymphocytes of all patients. However, IL-4 and IL-17 were detected in OLP patients only.These results suggest that the pathogenesis of GS is different from that of OLP. GS is a unique inflammatory disorder characterized by dysfunction of Th2 and Th17 immune reactions via abnormal T–B cell interaction.

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Section: Discussionmentioning

confidence: 98%

Cytokine Profiles Contribute to Understanding the Pathogenic Difference Between Good Syndrome and Oral Lichen Planus

et al. 2015

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“…There are some hypotheses of immunodeficiency in Good's syndrome. For example, the main function of the fetal thymus is T‐cell depletion of autoreactive clones and the presence of thymoma breaks the balance of immunosurveillance; the compensation of immunodeficiency is presented by hyperplasia of the thymus; there are irrelative events of immunodeficiency and thymoma, such as cytokines released from bone marrow stromal cells, influencing both thymic and B cell precursors [8]. Around 50% of Good's syndrome patients are characterized with B‐lymphopenia and 20% with panhypogammaglobulinemia.…”

Section: Discussionmentioning

confidence: 99%

Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma

Chen

Tsai

Lai

et al. 2012

The Kaohsiung J of Med Scie

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Good's syndrome, also known as thymoma with combined immunodeficiency, is rare. The immunodeficiency may precede, arise concurrently with or follow the diagnosis of thymoma. In addition to myasthenia gravis and Good's syndrome, paraneoplastic syndromes associated with thymoma can also be manifested with hematological disorders, such as pure red cell aplasia, aplastic anemia, agranulocytosis, hemolytic anemia, pernicious anemia, and paroxysmal nocturnal hemoglobinuria. Myelodysplastic syndrome is a group of clonal hematopoietic stem cell diseases characterized by cytopenia(s), dysplasia in one or more lineages, ineffective hematopoiesis, and potential precursors of acute leukemia. One proposed pathogenesis of myelodysplasia is autoantibodies that directly reject against hematopoietic cells, but this situation is rare in thymoma. Herein, we report a thymoma patient with unique paraneoplastic syndromes who developed myelodysplasia prior to Good's syndrome. Early and accurate diagnosis of myelodysplastic syndrome is important for disease management, especially in patients whose myelodysplastic syndrome is possibly derived from autoimmunity. For thymoma patients with recurrent infections, comprehensive immunologic studies to exclude the possibility of Good's syndrome and prophylactic intravenous immunoglobulin infusion in suitable candidates are warranted.

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“…Rashal et al [9] showed that the CD4 and CD8 T lymphocytes express the marker CD45R0, suggesting the predominance of lymphocyte sub-population of T-type memory. The absence of circulating B cells appears to come from a very early blocking differentiation B to the spinal level [3].…”

Section: …………………………………………………………………………………………………… Introduction:-mentioning

confidence: 96%

“…Discussion:good's syndrome is a rare disease that is responsible for recurrent bacterial, viral or fungal in 95% of cases. The pathophysiology of this syndrome remains unclear, it combines hypogammaglobulinemia, the absence or presence of low levels of B cells in the blood and defects in cell-mediated immunity [3], the syndrome patients have increased susceptibility to sinus and lung infections, pure red cell aplasia is also often associated with particularly good's syndrome in thymoma Type AB.…”

Section: …………………………………………………………………………………………………… Introduction:-mentioning

Good's Syndrome with a Block in the Early Stage of B Cell Differentiation and Complicated by Campylobacter fetus Sepsis.

Cited by 16 publications

References 11 publications

Cytokine Profiles Contribute to Understanding the Pathogenic Difference Between Good Syndrome and Oral Lichen Planus

Cytokine Profiles Contribute to Understanding the Pathogenic Difference Between Good Syndrome and Oral Lichen Planus

Myelodysplasia followed by Good's Syndrome: A unique manifestation associated with thymoma

Goods Syndrome:report of Case.

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