颅内出血背景下的周期性血小板减少症:诊断和治疗挑战。

IF 1.3 Q4 HEMATOLOGY Journal of hematology Pub Date : 2023-10-01 Epub Date: 2023-10-21 DOI:10.14740/jh1171
Ryan Sweeney, Maitreyee Rai, Harmeet Kharoud, Rama Bhagavatula, Robert Kaplan, Deep Shah
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引用次数: 0

摘要

循环性血小板减少症(CTP)顾名思义表现为周期性血小板减少,最初经常被误诊为免疫性血小板减少。在对免疫性血小板减少症的常见治疗缺乏持续反应或反应异常增加后,可以对CTP进行正确诊断。先前的报告显示,有一部分患者对环孢菌素a有反应。在这里,我们介绍了一例CTP病例,该病例最初在另一家机构被认为患有免疫性血小板减少性紫癜并接受了治疗。然而,在多次尝试使用类固醇、静脉注射免疫球蛋白(IVIG)、利妥昔单抗和艾曲波帕治疗后,严重血小板减少症和血小板增多症的发作仍在继续。在其中一次严重血小板减少症发作的情况下,患者最终出现脑出血(ICH),并随后出现多种并发症,不幸的是,患者未能康复。只有在发生脑出血后,才在具有血液学咨询能力的中心对患者进行了评估,在对其病例进行详细审查和模式识别后,开始使用环孢菌素对CTP进行正确诊断。该病例因需要在脑室-腹腔分流术后保持足够的血小板阈值而进一步复杂化,这是由于他的脑出血所必需的,并且是在诊断CTP之前放置的。虽然CTP是一种罕见的诊断,但这种情况更需要正确诊断和考虑环孢菌素治疗CTP,因为它对一些患者有效,可能有助于预防患者发病率,尤其是灾难性出血并发症。
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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Cyclic Thrombocytopenia in the Setting of Intracranial Hemorrhage: A Diagnostic and Therapeutic Challenge.

Cyclic thrombocytopenia (CTP) as the name suggests presents with cyclic episodes of thrombocytopenia and is frequently initially misdiagnosed as immune thrombocytopenia. Following a lack of sustained response or abnormally increased response to common treatments used for immune thrombocytopenia, a proper diagnosis of CTP can then be made. Prior reports have shown a subset of patients who respond to cyclosporin A. Here, we present a case of CTP that was initially at another facility presumed to have and treated for immune thrombocytopenic purpura. However, after multiple attempts to treat with steroids, intravenous immunoglobulin (IVIG), rituximab, and eltrombopag, episodes of severe thrombocytopenia followed by thrombocytosis continued. The patient ultimately developed intracerebral hemorrhage (ICH) in the setting of one of the episodes of severe thrombocytopenia and developed multiple subsequent complications from which the patient unfortunately did not recover. It was only after developing ICH that the patient had been evaluated at a center with hematology consultation capabilities, at which time after a detailed review of his case and pattern recognition the proper diagnosis of CTP was made with initiation of cyclosporine. This case was further complicated by need to maintain an adequate platelet threshold post-ventriculoperitoneal shunt placement which was necessary due to his ICH and was placed before diagnosis of CTP could be made. While CTP is a rare diagnosis, this case reinforces a greater need to properly diagnose and consider cyclosporine treatment for CTP, as it has been effective in some patients and may help to prevent patient morbidity and especially catastrophic bleeding complications.

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Journal of hematology
Journal of hematology HEMATOLOGY-
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