以神经系统症状为主诉的血栓性血小板减少性紫癜:1例报道及文献复习

孙亚蒙, 张瑛, 管阳太

神经病学与神经康复学杂志 ›› 2016, Vol. 12 ›› Issue (2) : 112-116.

PDF(1317 KB)
PDF(1317 KB)
神经病学与神经康复学杂志 ›› 2016, Vol. 12 ›› Issue (2) : 112-116. DOI: 10.12022/jnnr.2016-0049
病例报道

以神经系统症状为主诉的血栓性血小板减少性紫癜:1例报道及文献复习

  • 孙亚蒙, 张瑛, 管阳太
作者信息 +

Thrombotic thrombocytopenic purpura presenting with variable neurological symptoms: A case report and literature review

  • SUN Yameng, ZHANG Ying, GUAN Yangtai
Author information +
文章历史 +

摘要

本文报道了1例以神经功能受损为主诉的血栓性血小板减少性紫癜患者的诊治过程。该患者男性65岁,急性起病,症状表现为失语、肢体麻木及无力,症状波动而多变,并伴有发热。血液学检查发现贫血、血小板计数下降;有轻度肾功能受损;外周血涂片见红细胞碎片;血浆ADAMTS13活性显著降低,ADAMTS13抑制物阳性。该例患者被诊断为特发性血栓性血小板减少性紫癜,经及时诊断及积极进行血浆置换治疗后,病情得以缓解。

Abstract

This paper reports the diagnosis and management of a 65-year-old male patient with thrombotic thrombocytopenic purpura (TTP) presenting with neurological symptoms. The onset of the disease was sudden, presenting with cerebral manifestations including aphasia, limb numb and weakness. The symptoms observed were variable and fluctuant. After admission, this patient had a fever. The laboratory examination revealed anemia, peripheral thrombocytopenia, moderate renal involvement, schistocytosis, a severe functional deficiency in ADAMTS13 and positive anti-ADAMTS13 antibodies. Clinical and biological anomalies ascertained the diagnosis of idiopathic TTP. With the rapid diagnosis and therapeutical plasma exchange immediately performed, the patient reached a complete recovery.

关键词

血栓性血小板减少性紫癜 / 血小板减少 / 贫血 / 神经症状

Key words

Thrombotic thrombocytopenic purpura / Thrombocytopenia / Anemia / Neurological symptoms

引用本文

导出引用
孙亚蒙, 张瑛, 管阳太. 以神经系统症状为主诉的血栓性血小板减少性紫癜:1例报道及文献复习[J]. 神经病学与神经康复学杂志. 2016, 12(2): 112-116 https://doi.org/10.12022/jnnr.2016-0049
SUN Yameng, ZHANG Ying, GUAN Yangtai. Thrombotic thrombocytopenic purpura presenting with variable neurological symptoms: A case report and literature review[J]. Journal of Neurology and Neurorehabilitation. 2016, 12(2): 112-116 https://doi.org/10.12022/jnnr.2016-0049

参考文献

[1] TSAI H M, LIAN E C. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura[J]. N Engl J Med, 1998, 339(22):1585-1594.
[2] FEYS H B, ROODT J, VANDEPUTTE N, et al. Thrombotic thrombocytopenic purpura directly linked with ADAMTS13 inhibition in the baboon[J]. Blood, 2011, 116(12):2005-2010.
[3] HOSLER G A, CUSUMANO A M, HUTCHINS G M. Thrombotic thrombocytopenic purpura and hemolytic uremic syndrome are distinct pathologic entities:a review of 56 autopsy cases[J]. Arch Pathol Lab Med, 2003, 127(7):834-839.
[4] LEVY G G, NICHOLS W C, LIAN E C, et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura[J]. Nature, 2001, 413(6855):488-494.
[5] COPPO P, BUSSON M, VEYRADIER A, et al. HLA-DRB1*11: a strong risk factor for acquired severe ADAMTS13 deficiency-related idiopathic thrombotic thrombocytopenic purpura in Caucasians[J]. J Thromb Haemost, 2010, 8(4):856-859.
[6] COPPO P, VEYRADIER A. Thrombotic microangiopathies: towards a pathophysiology based classification[J]. Cardiovasc Hematol Disord Drug Targets, 2009, 9(1):36-50.
[7] RIDOLFI R L, BELL W R. Thrombotic thrombocytopenic purpura. Report of 25 cases and review of the literature[J]. Medicine (Baltimore), 1981, 60(6):413-428.
[8] MARIOTTE E, VEYRADIER A. Thrombotic thromcytopenic purpura: from diagnosis to therapy[J]. Curr Opin Crit Care, 2015, 21(6):593-601.
[9] SCHNEPPENHEIM R, BUDDE U, OYEN F, et al. von Willebrand factor cleaving protease and ADAMTS13 mutations in childhood TTP[J]. Blood, 2003, 101(5):1845-1850.
[10] SCULLY M, HUNT B J, BENJAMIN S, et al. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies[J]. Br J Haematol, 2012, 158(3):323-335.
[11] SARODE R, BANDARENKO N, BRECHER M E, et al. Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research[J]. J Clin Apher, 2014, 29(3):148-167.
[12] ROCK G A, SHUMAK K H, BUSKARD N A, et al. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura[J]. N Engl J Med, 1991, 325(6):393-397.
[13] 中华医学会血液学分会血栓与止血学组. 血栓性血小板减少性紫癜诊断与治疗中国专家共识(2012年版)[J]. 中华血液学杂志, 2012, 33(11):983-984.
[14] CRAWLEY J T, SCULLY M A. Thrombotic thrombocytopenic purpura: basic athophysiology and therapeutic strategies[J]. Hematology Am Soc Hematol Educ Program, 2013:292-299.
[15] LIM W, VESELY S K, GEORGE J N. The role of rituximab in the management of patients with acquired thrombotic thrombocytopenic purpura[J]. Blood, 2015, 125(10):1526-1531.

PDF(1317 KB)

Accesses

Citation

Detail

段落导航
相关文章

/