Is There a Correlation Between Immune Thrombocytopenia and Immunoglobulin G4-Related Disease?

IF 1.3 Q4 HEMATOLOGY
Journal of hematology Pub Date : 2024-08-01 Epub Date: 2024-08-15 DOI:10.14740/jh1260
Dorela Lame, Michelangelo Pianelli, Erika Morsia, Attilio Olivieri, Antonella Poloni
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引用次数: 0

Abstract

Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated condition causing organ swelling and fibrosis. Rarely, it coexists with primary immune thrombocytopenia (ITP), characterized by low platelet count (< 100 × 106/L) without an underlying cause. We present a case of a 56-year-old woman diagnosed with ITP in 2005, successfully treated with dexamethasone and intravenous immunoglobulins (IVIG). In 2011, she was diagnosed with IgG4-RD, type I autoimmune pancreatitis, initially treated with steroids then azathioprine with no response. ITP relapses were managed with prednisone/IVIG, rituximab, and thrombopoietin-receptor agonist therapy. Fostamatinib provided temporary relief, but platelet count dropped again in 2023. Combination therapy with small doses of prednisone and mycophenolate showed a partial response, maintaining platelet count over 50 × 106/L. Further investigation is warranted to explore any correlation between these two conditions, especially considering the patient's prolonged response to immunosuppressors.

免疫性血小板减少症与免疫球蛋白 G4 相关疾病之间有关联吗?
免疫球蛋白 G4 相关疾病(IgG4-RD)是一种免疫介导的疾病,会导致器官肿胀和纤维化。它与原发性免疫性血小板减少症(ITP)并存的情况非常罕见,原发性免疫性血小板减少症的特点是血小板计数低(< 100 × 106/L),但没有潜在的病因。我们报告了一例 56 岁女性的病例,她于 2005 年被诊断为 ITP,并成功接受了地塞米松和静脉注射免疫球蛋白(IVIG)治疗。2011 年,她被诊断出患有 IgG4-RD、I 型自身免疫性胰腺炎,最初使用类固醇治疗,后来又使用硫唑嘌呤治疗,但均无反应。泼尼松/IVIG、利妥昔单抗和血小板生成素受体激动剂疗法治疗了ITP复发。福斯他替尼暂时缓解了病情,但血小板计数在2023年再次下降。小剂量泼尼松和霉酚酸酯联合疗法显示出部分反应,血小板计数维持在 50 × 106/L 以上。考虑到患者对免疫抑制剂的长期反应,有必要进一步研究这两种情况之间的相关性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Journal of hematology
Journal of hematology HEMATOLOGY-
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