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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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.

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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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Journal of hematology HEMATOLOGY-

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