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

SUN Yameng; ZHANG Ying; GUAN Yangtai

doi:10.12022/jnnr.2016-0049

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Journal of Neurology and Neurorehabilitation ›› 2016, Vol. 12 ›› Issue (2) : 112-116. DOI: 10.12022/jnnr.2016-0049

Case Report

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

SUN Yameng, ZHANG Ying, GUAN Yangtai

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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.

Key words

Thrombotic thrombocytopenic purpura / Thrombocytopenia / Anemia / Neurological symptoms

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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

References

[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.