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卡泊单抗成功治疗难治性血栓性血小板减少性紫癜(TTP):一例报告

Successful Treatment of Refractory Thrombotic Thrombocytopenic Purpura (TTP) With Caplacizumab: A Case Report.

作者信息

Pramanik Debolina, Bhardwaj Divyashish, Karmani Vikash K, Raval Girindra G, Kutlar Abdullah

机构信息

Medicine, Medical College Kolkata, Kolkata, IND.

Medicine, Jawaharlal Nehru Medical College, Aligarh, IND.

出版信息

Cureus. 2023 Jul 25;15(7):e42423. doi: 10.7759/cureus.42423. eCollection 2023 Jul.

DOI:10.7759/cureus.42423
PMID:37637544
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10448709/
Abstract

We report a female patient who presented with generalized weakness, episodes of altered mental status and slurred speech, and a history of systemic lupus erythematosus. Initial investigations showed profound thrombocytopenia and schistocytosis on peripheral blood smear. PLASMIC score was promptly calculated, and plasma exchange with steroids was initiated based on the initial high PLASMIC score. Bone marrow examination showed hypocellular marrow without any other obvious abnormalities. The patient's platelet counts initially improved but had a quick decline, on which, rituximab and subsequently caplacizumab were introduced. The patient was discharged after stabilization with plasma exchange (PLEX) therapy as needed on an outpatient basis.

摘要

我们报告了一名女性患者,她出现全身无力、精神状态改变发作和言语不清,并有系统性红斑狼疮病史。初步检查显示外周血涂片有严重血小板减少和裂片红细胞。迅速计算了PLASMIC评分,并根据最初较高的PLASMIC评分开始进行血浆置换联合类固醇治疗。骨髓检查显示骨髓细胞减少,无任何其他明显异常。患者的血小板计数最初有所改善,但随后迅速下降,在此基础上,引入了利妥昔单抗,随后又使用了卡帕单抗。患者在门诊根据需要进行血浆置换(PLEX)治疗稳定后出院。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51cd/10448709/5e0683408984/cureus-0015-00000042423-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51cd/10448709/e33a0bb64866/cureus-0015-00000042423-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51cd/10448709/5e0683408984/cureus-0015-00000042423-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51cd/10448709/e33a0bb64866/cureus-0015-00000042423-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/51cd/10448709/5e0683408984/cureus-0015-00000042423-i02.jpg

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本文引用的文献

1
Frontiers in pathophysiology and management of thrombotic thrombocytopenic purpura.血栓性血小板减少性紫癜的病理生理学与管理前沿
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Immune-mediated thrombotic thrombocytopenic purpura in patients with and without systemic lupus erythematosus: a retrospective study.免疫介导的血栓性血小板减少性紫癜患者伴或不伴系统性红斑狼疮:一项回顾性研究。
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Successful treatment of refractory thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus with combination of plasma exchange and low-dose rituximab.成功治疗系统性红斑狼疮相关难治性血栓性血小板减少性紫癜:联合应用血浆置换和低剂量利妥昔单抗。
Lupus. 2020 Dec;29(14):1961-1967. doi: 10.1177/0961203320953473. Epub 2020 Aug 24.
8
Severe primary refractory thrombotic thrombocytopenic purpura (TTP) in the post plasma exchange (PEX) and rituximab era.血浆置换(PEX)和利妥昔单抗时代的严重原发性难治性血栓性血小板减少性紫癜(TTP)。
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Caplacizumab in adult patients with acquired thrombotic thrombocytopenic purpura.卡泊单抗用于成年获得性血栓性血小板减少性紫癜患者。
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Caplacizumab Treatment for Acquired Thrombotic Thrombocytopenic Purpura.卡普拉珠单抗治疗获得性血栓性血小板减少性紫癜。
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