Allford, S. L., Hunt, B. J., Rose, P., and Machin, S. J. 2003. Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias. Br J Haematol, 120, 556–73.Google Scholar

Alungal, J., Abdulla, M. C., Koya, J. M., and Jenner, P. F. 2014. Multiple hemorrhagic infarcts in thrombotic thrombocytopenic purpura. Neurol India, 62, 582–3.Google Scholar

Amorosi, E. L. and Ultmann, J. E. 1966. Thrombotic thrombocytopenic purpura: Report of 16 cases and review of the literature. Medicine (Baltimore), 45, 139–59.Google Scholar

Asada, Y., Sumiyoshi, A., Hayashi, T., Suzumiya, J., and Kaketani, K. 1985. Immunohistochemistry of vascular lesion in thrombotic thrombocytopenic purpura, with special reference to factor VIII related antigen. Thromb Res, 8, 469–79.Google Scholar

Bakshi, R., Shaikh, Z. A., Bates, V. E., and Kinkel, P. R. 1999. Thrombotic thrombocytopenic purpura: Brain CT and MRI findings in 12 patients. Neurology, 52, 1285–8.CrossRefGoogle Scholar

Balduini, C.L., Gugliotta, L., Luppi, M., et al. 2010. High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: A randomized study. Ann Hematol, 89, 591–6.Google Scholar

Bellante, F., Redondo Saez, P., Springael, C., and Dethy, S. 2014. Stroke in thrombotic thrombocytopenic purpura induced by thyrotoxicosis: A case report. J Stroke Cerebrovasc Dis, 23, 1744–6.CrossRefGoogle ScholarPubMed

Ben-Yehuda, D., Rose, M., Michaeli, T., and Eldor, A. 1998. Permanent neurological complications in patients with thrombotic thrombocytopenic purpura. Am J Hematol, 29, 74–8.Google Scholar

Bettoni, G., Palla, R., Valsecchi, C., et al. 2012. ADAMTS-13 activity and autoantibodies classes and subclasses as prognostic predictors in acquired thrombotic thrombocytopenic purpura. J Thromb Haemost, 10, 1556–65.CrossRefGoogle ScholarPubMed

Blombery, P. and Scully, M. 2014. Management of thrombotic thrombocytopenic purpura: Current perspectives. J Blood Med, 5, 15–23.Google Scholar

Cataland, S. R. and Wu, H. M. 2005. Immunotherapy for thrombotic thrombocytopenic purpura. Curr Opin Hematol, 12, 359–63.CrossRefGoogle ScholarPubMed

Cataland, S. R. and Wu, H. M. 2015. Acquired thrombotic thrombocytopenic purpura: New therapeutic options and their optimal use. J Thromb Haemost, 13 (Suppl 1), S223–9.Google Scholar

Crawley, J. T. B. and Scully, M. A. 2013 Thrombotic thrombocytopenic purpura: Basic pathophysiology and therapeutic strategies. Hematol Am Soc Hematol Educ Program 2013, 292–9.Google ScholarPubMed

D’Aprile, P., Farchi, G., Pagliarulo, R., and Carella, A. 1994. Thrombotic thrombocytopenic purpura: MR demonstration of reversible brain abnormalities. Am J Neuroradiol, 15, 19–20.Google Scholar

Ellchuk, T. N., Shah, L. M., Hewlett, R. H., and Osborn, A. G. 2011. Suspicious neuroimaging pattern of thrombotic microangiopathy. Am J Neuroradiol, 32, 734–8.Google Scholar

Fagniez, O., Tertian, G., Dreyfus, M., et al. 2011. Hematological disorders related cerebral infarctions are mostly multifocal. J Neurol Sci, 304, 87–92.Google Scholar

Froissart, A., Buffet, M., Veyradier, A., et al. 2012. Efficacy and safety of firstline rituximab in severe, acquired thrombotic thrombocytopenic purpura with a suboptimal response to plasma exchange. Experience of the French Thrombotic Microangiopathies Reference Center. Crit Care Med, 40, 104–11.Google Scholar

Fujikawa, K., Suzuki, H., McMullen, B., and Chung, D. 2001. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood, 98, 1662–6.CrossRefGoogle ScholarPubMed

Fujimura, Y., Matsumoto, M., Isonishi, A., et al. 2011. Natural history of Upshaw–Schulman syndrome based on ADAMTS13 gene analysis in Japan. J Thromb Haemost, 9 (Suppl 1), 283–301.CrossRefGoogle ScholarPubMed

Furlan, M. and Lämmle, B. 2001. Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: The role of von Willebrand factor-cleaving protease. Best Pract Res Clin Haematol, 14, 437–54.CrossRefGoogle ScholarPubMed

Furlan, M., Robles, R., Galbusera, M., et al. 1998. Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome. N Engl J Med, 339, 1578–84.CrossRefGoogle ScholarPubMed

George, J. N. 2010. How I treat patients with thrombocytopenic thrombotic purpura. Blood, 116, 4060–9CrossRefGoogle Scholar

Griffin, D., Al-Nouri, Z. L., Muthurajah, D., et al. 2013 First symptoms in patients with thrombotic thrombocytopenic purpura (TTP): What are they and when do they occur? Transfusion, 53, 235–7.Google Scholar

Gurkan, E., Baslamisli, F., Guvenc, B., et al. 2005. Thrombotic thrombocytopenic purpura in southern Turkey: A single-center experience of 29 cases. Clin Lab Haematol, 27, 121–5.Google Scholar

Han, B., Page, E. E., Stewart, L. M., et al. 2015. Depression and cognitive impairment following recovery from thrombotic thrombocytopenic purpura. Am J Hematol, 90, 709–14.Google Scholar

Hoirisch-Clapauch, S. and Nardi, A. E. 2014. A role for tissue plasminogen activator in thrombotic thrombocytopenic purpura. Med Hypotheses, 83, 747–50.CrossRefGoogle ScholarPubMed

Horton, T. M., Stone, J. D., Yee, D., et al. 2003. Case series of thrombotic thrombocytopenic purpura in children and adolescents. J Pediatr Hematol Oncol, 25, 336–9.Google Scholar

Imanirad, I., Rajasekhar, A., and Zumberg, M. 2012. A case series of atypical presentations of thrombotic thrombocytopenic purpura. J Clin Apher, 27, 221–6.CrossRefGoogle ScholarPubMed

Kay, A. C., Solberg, L. A., Nichols, D. A., and Pettit, R. M. 1991. Prognostic significance of computed tomography of the brain in thrombotic thrombocytopenic purpura. Mayo Clin Proc, 66, 602–7.CrossRefGoogle ScholarPubMed

Kelly, P. J., McDonald, C. T., O’Neill, G., et al. 1998. Middle cerebral artery main stem thrombosis in two siblings with familial thrombotic thrombocytopenic purpura. Neurology, 50, 1157–60.Google Scholar

Kondo, K., Yamawaki, T., Nagatsuka, K., Miyashita, K., and Naritomi, H. 2003. Reversible stenosis of major cerebral arteries demonstrated by MRA in thrombotic thrombocytopenic purpura. J Neurol, 250, 995–7.Google Scholar

Kremer Hovinga, J. A., Vesely, S. K., Terrell, D. R, Lämmle, B., and George, J. N. 2010. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood, 115, 1500–11.Google Scholar

Kremer Hovinga, J. A. and Lämmle, B. 2012. Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. Hematol Am Soc Hematol Educ Program 2012, 610–6.Google Scholar

Kroll, M. H., Hellums, J. D., McIntire, L. V., Schafer, A. I., and Moake, J. L. 1996. Platelets and shear stress. Blood, 88, 1525–41.CrossRefGoogle ScholarPubMed

Kwaan, H. C. 1987. Clinicopathological features of thrombotic thrombocytopenic purpura. Semin Hematol, 24, 74–81.Google ScholarPubMed

Kwaan, H. C. and Boggio, L. N. 2005. The clinical spectrum of thrombotic thrombocytopenic purpura. Semin Thromb Hemost, 31, 673–80.Google Scholar

Lämmle, B., Kremer Hovinga, J. A., and Alberio, L. 2005. Thrombotic thrombocytopenic purpura. J Thromb Haemost, 3, 1663–75.Google Scholar

Levy, G. A., Nichols, W. C., Lian, E. C., et al. 2001. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature, 413, 488–94.CrossRefGoogle Scholar

Lotta, L. A., Garagiola, I., Palla, R., Cairo, A., and Peyvandi, F. 2010. ADAMTS13 mutations and polymorphisms in congenital thrombotic thrombocytopenic purpura. Hum Mutat, 31, 11–9.CrossRefGoogle ScholarPubMed

Lucchesi, A., Fattori, P. P., Ronconi, S., et al. 2015. Atypical presentations of thrombotic thrombocytopenic purpura in middle-aged women with recurrent cerebral macrovascular thrombosis: A case report. Ann Hematol, 94, 1597–8.CrossRefGoogle ScholarPubMed

Martin, J. N. Jr., Bailey, A. P., Rehberg, J. F., et al. 2008. Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955–2006. Am J Obstet Gynecol, 199, 98–104.CrossRefGoogle ScholarPubMed

Meloni, G., Proia, A., Antonini, G., et al. 2001. Thrombotic thrombocytopenic purpura: Prospective neurologic neuroimaging and neurophysiologic evaluation. Haematologica, 86, 1194–9.Google ScholarPubMed

Moake, J. L. 2002. Thrombotic microangiopathies. N Engl J Med, 347, 589–600.CrossRefGoogle ScholarPubMed

Moake, J. L. and McPherson, P. D. 1989. Abnormalities of von Willebrand factor multimers in thrombotic thrombocytopenic purpura and the hemolytic uremic syndrome. Am J Med, 87, 9–15.Google ScholarPubMed

Moake, J. L., Rudy, C. K., Troll, J. H., et al. 1982. Unusually large plasma factor VIII: Von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med, 307, 1432–5.Google Scholar

Moschkowitz, E. 1924. Hyaline thrombosis of the terminal arterioles and capillaries: A hitherto undescribed disease. Proc N Y Pathol Soc, 24, 21–4.Google Scholar

Müller, J., Czinyéri, J., Sasvári, I., Garami, M., and Kovács, G. 2001. Thrombotic thrombocytopenic purpura, Moschkowitz syndrome. Int Pediatr, 16, 144–9.Google Scholar

Paliwal, P. R., Teoh, H. L., and Sharma, V. K. 2014. Association between reversible cerebral vasoconstriction syndrome and thrombotic thrombocytopenic purpura. J Neurol Sci, 339, 47–51.CrossRefGoogle Scholar

Park, S. A., Lee, T. K., Sung, K. B., and Park, S. K. 2005. Extensive brain stem lesions in thrombotic thrombocytopenic purpura: Repeat magnetic resonance findings. J Neuroimag, 15, 79–81.Google Scholar

Petitt, R. M. 1980. Thrombotic thrombocytopenic purpura: A thirty-year review. Semin Thromb Hemost, 6, 350–5.Google Scholar

Peyvandi, F., Ferrari, S., Lavoretano, S., Canciani, M. T., and Mannucci, P. M. 2004. Von Willebrand factor cleaving protease (ADAMTS-13) and ADAMTS-13 neutralizing autoantibodies in 100 patients with thrombotic thrombocytopenic purpura. Br J Hematol, 127, 433–9.Google Scholar

Peyvandi, F., Lavoretano, S., Palla, R., et al. 2008. ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica, 93, 232–9.Google Scholar

Piastra, M., Currò, V., Chiaretti, A., et al. 2001. Intracranial hemorrhage at the onset of thrombotic thrombocytopenic purpura in an infant: Therapeutic approach and intensive care management. Pediatr Emerg Care, 17, 42–5.Google Scholar

Reese, J. A., Muthurajah, D. S., Kremer Hovinga, J. A., et al. 2013. Children and adults with thrombotic thrombocytopenic purpura associated with severe, acquired ADAMTS 13 deficiency: Comparison of incidence, demographic and clinical features. Pediatr Blood Cancer, 60, 1676–82.Google Scholar

Ridolfi, R. L. and Bell, W. R. 1981. Thrombotic thrombocytopenic purpura: A report of 25 cases and a review of the literature. Medicine (Baltimore), 60, 413–28.CrossRefGoogle Scholar

Rieger, M., Mannucci, P. M., Kremer Hovinga, J. A., et al. 2005. ADAMTS 13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases. Blood, 106, 1262–7.Google Scholar

Rinkel, G. J. E. and Wijdicks, E. F. M. 1991. Stroke in relapsing thrombotic thrombocytopenic purpura. Stroke, 28, 1087–9.Google Scholar

Rock, G. A., Shumak, K. H., Buskard, N. A., et al. 1991. Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group. N Engl J Med, 325, 393–7.Google Scholar

Rock, G., Anderson, D., Clark, W., et al. 2005. Does cryosupernatant plasma improve outcome in thrombotic thrombocytopenic purpura? No answer yet. Br J Haematol, 129, 79–86.Google Scholar

Rojas, J.C., Banerjee, C., Siddiqui, F., Nourbakhsh, B., and Powell, C. M. 2013. Acute ischemic stroke caused by atypical thrombotic thrombocytopenic purpura. Neurology, 80, e235–e38.Google Scholar

Ruggeri, Z. M., Dent, J. A., and Saldívar, E. 1999. Contribution of distinct adhesive interactions to platelet aggregation in flowing blood. Blood, 94, 172–8.Google Scholar

Sadler, J. E. 1998. Biochemistry and genetics of von Willebrand factor. Annu Rev Biochem, 67, 395–424.CrossRefGoogle ScholarPubMed

Sadler, J. E., Moake, J. L., Miyata, T., and George, J. M. 2004. Recent advances in thrombotic thrombocytopenic purpura. Hematol Am Soc Hematol Educ Prog, 407–23.Google Scholar

Sarode, R., Gottschall, J. L., Aster, R. H., and McFarland, J. G. 1997. Thrombotic thrombocytopenic purpura: Early and late responders. Am J Hematol, 54, 102–7.3.0.CO;2-0>CrossRefGoogle ScholarPubMed

Sarode, R. 2009. Atypical presentations of thrombotic thrombocytopenic purpura: A review. J Clin Apher, 24, 47–52.Google Scholar

Sarode, R., Bandarenko, N., Brecher, M.E., et al. 2014. Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research. J Clin Apher, 29, 148–67.Google Scholar

Sayani, F. A. and Abrams, C. S. 2015. How I treat refractory thrombotic thrombocytopenic purpura. Blood, 125, 3860–67.Google Scholar

Scheid, R., Hegenbart, U., Ballaschke, O., and von Cramon, D. Y. 2004. Major stroke in thrombotic-thrombocytopenic purpura (Moschkowitz syndrome). Cerebrovasc Dis, 18, 83–5.Google Scholar

Scheiflinger, F., Knobl, P., Trattner, B., et al. 2003. Nonneutralizing IgM and IgG antibodies to von Willebrand factor-cleaving protease (ADAMTS-13) in a patient with thrombotic thrombocytopenic purpura. Blood, 102, 3241–43.CrossRefGoogle Scholar

Scully, M., Yarranton, H., Liesner, R., et al. 2008. Regional UK TTP Registry: Correlation with laboratory ADAMTS 13 analysis and clinical features. Br J Haematol, 142, 819–26.Google Scholar

Scully, M., McDonald, V., Cavenagh, J., et al. 2011. A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura. Blood, 118, 1746–53.Google Scholar

Scully, M., Hunt, B.J., Benjamin, S., et al. 2012. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol, 158, 323–35.CrossRefGoogle ScholarPubMed

Scully, M., Thomas, M., Underwood, M., et al. 2014. Thrombotic thrombocytopenic purpura and pregnancy: Presentation, management, and subsequent pregnancy outcomes. Blood, 124, 211–9.Google Scholar

Singer, K., Bornstein, F. P., and Wiles, S. A. 1947. Thrombotic thrombocytopenic purpura. Hemorrhagic diathesis with generalized platelet thrombosis. Blood, 2, 542–54.Google Scholar

Shekman, B. and Einav, Y. 2014. Thrombotic thrombocytopenic purpura and other thrombotic microangiopathic hemolytic anemias: Diagnosis and classification. Autoimmun Rev, 13, 584–6.Google Scholar

Sonneveld, M. A. H., De Maat, M. P. M., and Leebeek, F. W. G. 2014. Von Willebrand factor and ADAMTS13 in arterial thrombosis: A systematic review and meta-analysis. Blood Rev, 28, 167–78.Google Scholar

Sperati, C. J. and Moliterno, A. R. 2015. Thrombotic microangiopathy. Focus on atypical hemolytic uremic syndrome. Hematol Oncol Clin N Am, 29, 541–59.Google Scholar

Studt, J. D., Hovinga, J. A., Radonic, R., et al. 2004. Familial acquired thrombotic thrombocytopenic purpura: ADAMTS 13 inhibitory autoantibodies in identical twins. Blood, 103, 4195–7.CrossRefGoogle Scholar

Tardy, B., Page, Y., Convers, P., et al. 1993. Thrombotic thrombocytopenic purpura: MR findings. Am J Neuroradiol, 14, 489–90.Google Scholar

Terrel, D. R., Williams, L. A., Vesely, S. K., et al. 2005. The incidence of thrombotic thrombocytopenic purpura–hemolytic uremic syndrome: All patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost, 3, 1432–6.Google Scholar

Terrell, D. R., Vesely, S. K., Kremer Hovinga, J. A., Lämmle, B., and George, J. N., 2010. Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic–uremic syndromes. Am J Hematol, 85, 844–7.Google Scholar

Tsai, H. M. 1996. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood, 87, 4235–44.CrossRefGoogle ScholarPubMed

Tsai, H. M. 2000. High titers of inhibitors of von Willebrand factor-cleaving metalloproteinase in a fatal case of acute thrombotic thrombocytopenic purpura. Am J Hematol, 65, 251–5.Google Scholar

Tsai, H. M. 2002. Von Willebrand factor, ADAMTS 13, and thrombotic thrombocytopenic purpura. J Mol Med, 80, 639–47.Google Scholar

Tsai, H. M. 2003. Advances in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura. J Am Soc Nephrol, 14, 1072–81.Google Scholar

Tsai, H. M. and Lian, E. C. 1998. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med, 339, 1585–94.Google Scholar

Turner, N., Sartain, S., and Moake, J. 2015. Ultralarge von Willebrand factor-induced platelet clumping and activation of the alternative complement pathway in thrombotic thrombocytopenic purpura and the hemolytic–uremic syndromes. Hematol Oncol Clin North Am, 29, 509–24.Google Scholar

Uğur Bilgin, A., Karaselek, M. A., and Camlı, K. 2014. Successful management of thrombotic thrombocytopenic purpura associated with pregnancy. Transfus Apher Sci, 50, 433–7.Google Scholar

Vesely, S. K. 2015. Life after acquired thrombotic thrombocytopenic purpura: Morbidity, mortality, and risks during pregnancy. J Thromb Haemost, 13 (Suppl. 1): S216–22.CrossRefGoogle ScholarPubMed

Veyradier, A., Obert, B., Houllier, A., Meyer, D., and Dima, J. P. 2001. Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: A study of 111 cases. Blood, 98, 1765–72.CrossRefGoogle ScholarPubMed

Wada, H., Matsumoto, T., and Yamashita, Y. 2014. Natural history of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Semin Thromb Hemost, 40, 866–73.Google Scholar

Westwood, J. P., Webster, H., McGuckin, S., et al. 2013. Rituximab for thrombotic thrombocytopenic purpura: Benefit of early administration during acute episodes and use of prophylaxis to prevent relapse. J Thromb Haemost, 11, 481–90.CrossRefGoogle ScholarPubMed

Wijdicks, E. F. M. 1994. Silent brain infarct in thrombotic thrombocytopenic purpura. Stroke, 25, 1297–8.Google Scholar

Willis, M. S. and Bandarenko, N. 2005. Relapse of thrombotic thrombocytopenic purpura: Is it a continuum of disease? Semin Thromb Hemost, 31, 700–8.Google Scholar

Yu, W. L., Leung, T., Soo, Y., Lee, J., and Wong, K. S. 2015. Thrombotic thrombocytopenic purpura with concomitant small- and large-vessel thrombosis, atypical posterior reversible encephalopathy syndrome and cerebral microbleeds. Oxf Med Case Reports, 179–182.Google Scholar

Zheng, X. L. 2015. ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura. Annu Rev Med, 66, 211–25.Google Scholar

Zheng, X. L., Chung, D., Takayama, T. K., et al. 2001. Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem, 276, 41059–63.Google Scholar