Thrombotic thrombocytopenic purpura with clinical neurological presentation - case report

Authors

  • Isa Kadi UMHATEM „N. I. Pirogov”
  • Ya. Grancharova UMHATEM „N. I. Pirogov”
  • I. Tonev National specialized hospital for active treatment of hematological diseases – EAD
  • V. Plachkov UMHATEM „N. I. Pirogov”
  • M. Dimitrovа UMHATEM „N. I. Pirogov”

Keywords:

ADAMTS 13, acute ischemic stroke, microangiopathy, subarachnoid hemorrhage, thrombotic thrombocytopenic purpura

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a microangiopathic hemolytic anemia classically characterized by the pentad of fever, hemolytic anemia, thrombocytopenia, renal, and neurologic deficits. TTP is a life-threatening multisystem disease characterized by thrombocytopenia and fluctuating neurological symptoms due to micro infarcts [12]. TTP is a thrombotic microangiopathy that can be classified as idiopathic or secondary to another disease [8, 9, 17]. In many cases of this coagulation disease, a greatly reduced activity of the ADAMTS 13 enzyme is observed (<10%) [8]. The disease is extremely rare, with a frequency of 3.7/1,000,000/year [25]. Cases of ischemic stroke (IS) due to TTP have been reported in scientific papers [6, 11, 20]. However, IS due to arterial thrombosis is rarely observed in patients with TTP [11].
Objective: To present a clinical case of a patient with ischemic stroke and subarachnoid hemorrhage, as the first clinical manifestation of thrombotic thrombocytopenic purpura, emphasizing how key is rapid and accurate diagnosis and good collaboration between specialists from different clinical units for timely and correct treatment of patients with a similar type of multisystem disease.

References

Amin Asnafi, A., Jalali, M.T., Pezeshki, S.M.S,. Jaseb, K., Saki, N. The Association Between Human Leukocyte Antigens and ITP, TTP, and HIT. J Pediatr Hematol Oncol. 2019 Mar, 41, 2, 81-86.

Bell, W.R., Braine, H.G., Ness, P.M., Kickler, T.S. Improved survival in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome. Clinical experience in 108 patients. N Engl J Med. 1991 Aug, 325, 6, 398-403.

Boattini, M., Procaccianti, G. Stroke due to typical thrombotic thrombocytopenic purpura treated successfully with intravenous thrombolysis and therapeutic plasma exchange. BMJ Case Rep. 2013 Jan 28, 2013, 2012008426.

Coppo, P., Cuker, A., George, J.N. Thrombotic thrombocytopenic purpura: Toward targeted therapy and precision medicine. Res Pract Thromb Haemost. 2019 Jan, 3, 1, 26-37.

Cox, E.C. Thrombotic thrombocytopenic purpura: report of three additional cases and a short review of the literature. J S C Med Assoc. 1966 Dec, 62, 12, 465-470.

Downes, K.A., Yomtovian, R., Tsai, H.M., Silver, B., Rutherford, C., Sarode, R. Relapsed thrombotic thrombocytopenic purpura presenting as an acute cerebrovascular accident. J Clin Apher. 2004, 19, 2, 86-89.

Gavriilaki, E., Anagnostopoulos, A., Mastellos, D.C. Complement in Thrombotic Microangiopathies: Unraveling Ariadne's Thread Into the Labyrinth of Complement Therapeutics. Front Immunol. 2019, 10, 337.

George, J.N. How I treat patients with thrombotic thrombocytopenic purpura: 2010. Blood. 2010 Nov 18, 116, 20, 4060-4069.

George, J.N. Thrombotic thrombocytopenic purpura. N Engl J Med 2006, 354, 1927-1935.

Gurkan, E., Baslamisli, F., Guvenc, B.et al Thrombotic thrombocytopenic purpura in southern Turkey: a single-center experience of 29 cases.Clin Lab Haematol 2005, 27, 121-125.

Idowu, M., Reddy, P. Atypical thrombotic thrombocytopenic purpura in a middle-aged woman who presented with a recurrent stroke. Am J Hematol. 2013 Mar, 88, 3, 237-239.

Joly, B.S., Coppo, P., Veyradier, A. Thrombotic thrombocytopenic purpura. Blood. 2017 May 25, 129, 21, 2836-2846.

Kinoshita, S., Yoshioka, A., Park, Y.D. et al. Upshaw-Schulman syndrome revisited: a concept of congenital thrombotic thrombocytopenic purpura. Int. J. Hematol. 2001, 74, 101-108.

Moschcowitz, E. Hyaline thrombosis of the terminal arterioles and capillaries: a hitherto undescribed disease. Proc. N.Y. Pathol. Soc. 1924, 24, 21-24.

O’Brien, T.E., Crum, E.D. Atypical Presentations of Thrombotic Thrombocytopenic Purpura. Int J Hematol. 2002, 76, 5, 471-473.

Peyvandi, F,. Ferrari, S., Lavoretano, S. et al. von Willebrand factor cleaving protease (ADAMTS-13) and ADAMTS-13 neutralizing autoantibodies in 100 patients with thrombotic thrombocytopenic purpura. Br J Haematol 2004, 127, 433-439.

Rosove, M.H. Thrombotic microangiopathies. Semin Arthritis Rheum 2014, 43, 797-805.

Sadler, J.E, Moake, J. L., Miyata, T. et al. Recent advances in thrombotic thrombocytopenic purpura. Hematology (Am Soc Hematol Educ Program). 2004, 407-423.

Schulman, I., Pierce, M., Lukens, A. et al. Studies on thrombopoiesis. I. A factor in normal human plasma required for platelet production; chronic thrombocytopenia due to its deficiency. Blood. 1960, 16, 943-957.

Sevy, A., Doche, E., Squarcioni, C. et al. Stroke in a young patient treated by alteplase heralding an acquired thrombotic thrombocytopenic purpura. J Clin Apher 2011, 26, 152-155.

Shelat, S.G., Ai, J., Zheng, X.L. Molecular biology of ADAMTS13 and diagnostic utility of ADAMTS13 proteolytic activity and inhibitor assays. Semin Thromb Hemost. 2005 Dec, 31, 6, 659-672.

Swart, L., Schapkaitz, E., Mahlangu, J.N. Thrombotic thrombocytopenic purpura: A 5-year tertiary care centre experience. J Clin Apher. 2019 Feb, 34, 1, 44-50.

Tanner, L., Müller, M.M. [Blood Transfusion: a Guide to Clinical Decision Making]. Anasthesiol Intensivmed Notfallmed Schmerzther. 2019 Mar, 54, 3, 194-205.

Terrell, D.R., Vesely, S.K., Kremer Hovinga, J.A., Lämmle, B., George, J.N. Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes. Am J Hematol. 2010 Nov, 85, 11, 844-847.

Torok, T.J., Holman, R.C., Chorba, T.L. Increasing mortality from thrombotic thrombocytopenic purpura in the United States-analysis of national mortality data. Am J Hematol. 1995, 50, 84-90.

Upshaw, J.D., Jr. Congenital deficiency of a factor in normal plasma that reverses microangiopathic hemolysis and thrombocytopenia. N. Engl. J. Med. 1978, 298, 1350-1325.

van Dorland, H.A., Taleghani, M.M., Sakai, K., Friedman, K.D., George JN, Hrachovinova, I., Knöbl, P.N., von Krogh, A.S, Schneppenheim, R., Aebi-Huber, I., Bütikofer, L., Largiadèr, C.R., Cermakova, Z., Kokame, K., Miyata, T., Yagi, H., Terrell, D.R., Vesely, S.K., Matsumoto, M., Lämmle, B., Fujimura, Y., Kremer Hovinga, J.A. Hereditary TTP Registry. The International Hereditary Thrombotic Thrombocytopenic Purpura Registry: key findings at enrollment until 2017. Haematologica. 2019 Oct, 104, 10, 2107-2115.

Vesely, S.K., George, J. N., Lammle, B.et al. ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients. Blood. 2003, 102, 60-68.

Wiernek, S.L., Jiang, B., Gustafson, G.M., Dai, X. Cardiac implications of thrombotic thrombocytopenic purpura. World J Cardiol. 2018 Dec 26, 10, 12, 254-266.

Zander, C.B., Cao, W., Zheng, X.L. ADAMTS13 and von Willebrand factor interactions. Curr Opin Hematol. 2015 Sep, 22, 5, 452-459.

Published

30.11.2024

How to Cite

Kadi, I., Grancharova, Y., Tonev, I., Plachkov, V., & Dimitrovа M. (2024). Thrombotic thrombocytopenic purpura with clinical neurological presentation - case report. Bulgarian Neurology, 25(2), 79–82. Retrieved from https://www.nevrologiabg.com/journal/index.php/neurology/article/view/166

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