Good's Syndrome Accompanied by Agranulocytosis Following a Rapid Clinical Course

Okusu, Takahiro; Sato, Taiki; Ogata, Yoshitaka; Nagata, Shinpei; Kozumi, Kazuhiro; Kim, Sung Hoon; Yamamoto, Shuichi; Yamayoshi, Shigeru

doi:10.2169/internalmedicine.55.5542

Cited by 8 publications

(3 citation statements)

References 11 publications

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“…Survived Type A thymoma CD4/CD8 ratio reversed γ-globulin fraction reduced Low IgA and IgM 0.2 × 10 9 /L 5.7 × 10 9 /L 11 Okusu et al [18] 72/M Hypoplasia of only leukocyte progenitor cells…”

Section: G-csf Ivig Dexamethasonementioning

confidence: 99%

Pure white cell aplasia before and after thymectomy in the rare conundrum of thymoma: A case report and review of the literature

Yang,

Chen,

Zheng

et al. 2024

Medicine

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Rationale: Pure white cell aplasia (PWCA) is a rare paraneoplastic syndrome that occurs in patients with thymomas. Currently, the pathogenesis and treatment of this disease remain in the exploratory stage. Patient concerns: We report a 68-year-old woman with thymoma experienced PWCA involvement as her first presentation. The patient had high fever and agranulocytosis at the onset of the disease. The white blood cell count in the complete blood count was 1.9 × 109/L with a neutrophil of 0.1 × 109/L. The bone marrow aspirates showed decreased granulocyte proliferation. Computed tomography showed a large mass in the anterior mediastinum. Diagnoses: The final diagnosis of our patient was PWCA and thymoma. Interventions: She underwent a thymectomy and cyclosporine A administration during first remission. Outcomes: Long-term remission was achieved following the readministration of cyclosporine A after the disease recurrence. Lessons: PWCA or agranulocytosis with thymoma has been confirmed to be an extremely rare disease. Thymomas with PWCA correlate with autoimmunity. From this case study and the literature review, we concluded that the pathogenesis of thymomas in PWCA is mainly related to the activation of autoreactive T cells. Thymectomy and the immunosuppressive drug, cyclosporine A, were chosen for treatment. The patient’s granulocyte levels were unable to recover after surgery because of the inability to promptly clear activated T cells. After surgery, cyclosporine A continued to take for a long time. Thymectomy combined with prolonged cyclosporine A administration may be an effective method for treating this rare disease.

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Section: G-csf Ivig Dexamethasonementioning

confidence: 99%

Pure white cell aplasia before and after thymectomy in the rare conundrum of thymoma: A case report and review of the literature

Yang,

Chen,

Zheng

et al. 2024

Medicine

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“…Candida [18,23,30,62,89] 4.8% Bacteremia Staphylococcus [90] Escherichia coli [61] Alcaligenes sp. [91] Achromobacter Xylosoxidans [74] 3.8% Septicemia Campylobacter [23,54,92] 2.9% clinical features pathogen percentage…”

Section: Oral Candidiasismentioning

confidence: 99%

Good syndrome combined with myelodysplastic syndrome and multiple microbial pulmonary infections: A case report and literature review

Ye,

Wang,

Bao

et al. 2023

Preprint

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Introduction Good syndrome (GS) is a rare acquired immunodeficiency disease characterized by thymoma and hypogammaglobulinemia that predisposes to the risk of recurrent infections with multiple pathogens. Case representation We describe the case of a 37-year-old male with GS who had a history of thymoma resection combined with myelodysplastic syndrome and was hospitalized multiple times for infections. The patient was admitted to our hospital with reoccurring fever, empirical antimicrobial therapy was ineffective, and Streptococcus pneumoniae, cytomegalovirus, cytomegalovirus, fine cyclic virus, Candida albicans, and Pneumocystis japonicus infections were identified by high-throughput sequencing technology (NGS) testing of alveolar lavage fluid, and the patient's symptoms resolved and immunity was improved with targeted anti-infective therapy. Conclusion When patients with thymoma have recurrent abnormal infections, immunologic testing should be performed to clarify whether GS is present, and anti-infective therapy should be aggressively administered.

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“…This elicits neutralizing autoantibodies to IL17associated cytokines and impairs cytokine-dependent macrophage activation that, in turn, is needed to keep Candia in check [30]. Thymoma-associated immunodeficiency is a diagnostic challenge and can contribute to severe morbidity and even mortality [31][32][33][34].…”

Section: Immunodeficiency In Thymoma Patientsmentioning

confidence: 99%

Molecular pathology of thymomas: implications for diagnosis and therapy

et al. 2021

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Thymomas exhibit a unique genomic landscape, comprising the lowest on average total mutational burden among adult human cancers; a unique point mutation in the GTF2I gene in WHO type A and AB thymomas (and rarely others); almost unique KMT2A-MAML2 translocations in rare WHO type B2 and B3 thymomas; a unique YAP1-MAML2 translocation in almost all metaplastic thymomas; and unique miRNA profiles in relation to GTF2I mutational status and WHO histotypes. While most thymomas can be diagnosed solely on the basis of morphological features, mutational analyses can solve challenging differential diagnostic problems. No molecular biomarkers have been identified that predict the response of unresectable thymomas to chemotherapy or agents with known molecular targets. Despite the common and strong expression of PDL1 in thymomas, immune checkpoint inhibitors are rarely applicable due to the poor predictability of common, life-threatening autoimmune side effects that are related to the unrivaled propensity of thymomas towards autoimmunity.

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Good's Syndrome Accompanied by Agranulocytosis Following a Rapid Clinical Course

Cited by 8 publications

References 11 publications

Pure white cell aplasia before and after thymectomy in the rare conundrum of thymoma: A case report and review of the literature

Pure white cell aplasia before and after thymectomy in the rare conundrum of thymoma: A case report and review of the literature

Good syndrome combined with myelodysplastic syndrome and multiple microbial pulmonary infections: A case report and literature review

Molecular pathology of thymomas: implications for diagnosis and therapy

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