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脾切除术后发现的由Janus激酶2突变引起的原发性血小板增多症。

Essential Thrombocythemia Due to Janus Kinase 2 Mutation Unmasked After Splenectomy.

作者信息

Khanduri Archana, Gupta Rahul, Gupta Jyoti, Ammar Houssem

机构信息

Gastrointestinal Surgery, Synergy Institute of Medical Sciences, Dehradun, IND.

Radiation Oncology, Swami Rama Himalayan University, Dehradun, IND.

出版信息

Cureus. 2021 May 31;13(5):e15357. doi: 10.7759/cureus.15357. eCollection 2021 May.

DOI:10.7759/cureus.15357
PMID:34239790
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8245623/
Abstract

Reactive thrombocytosis after splenectomy is common and often self-limiting. However, thrombocytosis can be multifactorial, especially extreme thrombocytosis (platelet count > 100 x 10/cubic mm). It can lead to thrombotic or hemorrhagic complications. Hence, in patients with rising platelet count after splenectomy, detailed evaluation may be required to rule out other causes of thrombocytosis, such as infection, iron deficiency, and myeloproliferative disorders. Timely treatment of patients with thrombocytosis can prevent the development of life-threatening complications. The index case highlights the importance of regular follow-up of the patients after splenectomy to detect thrombocytosis and suspect other causes if the spleen was diseased or the platelet count fails to resolve spontaneously.

摘要

脾切除术后反应性血小板增多症很常见,且通常为自限性。然而,血小板增多症可能是多因素导致的,尤其是极端血小板增多症(血小板计数>100×10⁹/立方毫米)。它可导致血栓形成或出血并发症。因此,对于脾切除术后血小板计数升高的患者,可能需要进行详细评估以排除血小板增多症的其他原因,如感染、缺铁和骨髓增殖性疾病。及时治疗血小板增多症患者可预防危及生命的并发症的发生。该病例强调了脾切除术后对患者进行定期随访的重要性,以便在脾脏患病或血小板计数未能自发恢复正常时检测到血小板增多症并怀疑其他原因。

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本文引用的文献

1
Disease and Clinical Characteristics of Patients With a Clinical Diagnosis of Essential Thrombocythemia Enrolled in the MOST Study.参与MOST研究的临床诊断为原发性血小板增多症患者的疾病及临床特征
Clin Lymphoma Myeloma Leuk. 2021 Jul;21(7):461-469. doi: 10.1016/j.clml.2021.02.011. Epub 2021 Mar 1.
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A Rare Case of Triple-Negative Essential Thrombocythemia in a Young Postsplenectomy Patient: A Diagnostic Challenge.一名年轻脾切除术后患者的三阴性原发性血小板增多症罕见病例:诊断挑战
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Essential thrombocythemia treatment algorithm 2018.2018 年原发性血小板增多症治疗算法
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Blast transformation and fibrotic progression in polycythemia vera and essential thrombocythemia: a literature review of incidence and risk factors.真性红细胞增多症和原发性血小板增多症中的原始细胞转化和纤维化进展:发病率及危险因素的文献综述
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Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis.分子注释的原发性血小板增多症、真性红细胞增多症和骨髓纤维化的长期生存及原始细胞转化
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6
Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: an international study of 891 patients.WHO 定义的原发性血小板增多症患者的动静脉血栓形成的危险因素:一项国际 891 例患者研究。
Blood. 2011 Jun 2;117(22):5857-9. doi: 10.1182/blood-2011-02-339002. Epub 2011 Apr 13.
7
Postsplenectomy reactive thrombocytosis.脾切除术后反应性血小板增多症。
Proc (Bayl Univ Med Cent). 2009 Jan;22(1):9-12. doi: 10.1080/08998280.2009.11928458.
8
Prospective study of the incidence and risk factors of postsplenectomy thrombosis of the portal, mesenteric, and splenic veins.脾切除术后门静脉、肠系膜静脉和脾静脉血栓形成的发病率及危险因素的前瞻性研究。
Arch Surg. 2006 Jul;141(7):663-9. doi: 10.1001/archsurg.141.7.663.
9
Extreme thrombocytosis: what are the etiologies?极度血小板增多症:病因有哪些?
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Angiology. 2004 May-Jun;55(3):319-23. doi: 10.1177/000331970405500312.