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?

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
{"title":"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?","authors":"Dorela Lame, Michelangelo Pianelli, Erika Morsia, Attilio Olivieri, Antonella Poloni","doi":"10.14740/jh1260","DOIUrl":null,"url":null,"abstract":"<p><p>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 × 10<sup>6</sup>/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 × 10<sup>6</sup>/L. Further investigation is warranted to explore any correlation between these two conditions, especially considering the patient's prolonged response to immunosuppressors.</p>","PeriodicalId":15964,"journal":{"name":"Journal of hematology","volume":"13 4","pages":"174-177"},"PeriodicalIF":1.3000,"publicationDate":"2024-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11379049/pdf/","citationCount":"0","resultStr":"{\"title\":\"Is There a Correlation Between Immune Thrombocytopenia and Immunoglobulin G4-Related Disease?\",\"authors\":\"Dorela Lame, Michelangelo Pianelli, Erika Morsia, Attilio Olivieri, Antonella Poloni\",\"doi\":\"10.14740/jh1260\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><p>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 × 10<sup>6</sup>/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 × 10<sup>6</sup>/L. Further investigation is warranted to explore any correlation between these two conditions, especially considering the patient's prolonged response to immunosuppressors.</p>\",\"PeriodicalId\":15964,\"journal\":{\"name\":\"Journal of hematology\",\"volume\":\"13 4\",\"pages\":\"174-177\"},\"PeriodicalIF\":1.3000,\"publicationDate\":\"2024-08-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11379049/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of hematology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.14740/jh1260\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/8/15 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q4\",\"JCRName\":\"HEMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of hematology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.14740/jh1260","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/8/15 0:00:00","PubModel":"Epub","JCR":"Q4","JCRName":"HEMATOLOGY","Score":null,"Total":0}
引用次数: 0

摘要

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

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.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Journal of hematology
Journal of hematology HEMATOLOGY-
自引率
0.00%
发文量
29
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信