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ISRN Hematology
Volume 2012 (2012), Article ID 310596, 8 pages
http://dx.doi.org/10.5402/2012/310596
Review Article

Thrombotic Microangiopathies

Pediatric Hematology/Oncology/Stem Cell Transplant, Children’s Mercy Hospital, Kansas City, MO 64108, USA

Received 9 April 2012; Accepted 10 June 2012

Academic Editors: G. Lucarelli and B. Wachowicz

Copyright © 2012 Mohamed Radhi and Shannon L. Carpenter. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Linked References

  1. G. W. Hall, “Kasabach-Merritt syndrome: pathogenesis and management,” British Journal of Haematology, vol. 112, no. 4, pp. 851–862, 2001. View at Publisher · View at Google Scholar · View at Scopus
  2. M. Sarkar, J. B. Mulliken, H. P. W. Kozakewich, R. L. Robertson, and P. E. Burrows, “Thrombocytopenic coagulopathy (Kasabach-Merritt Phenomenon) is associated with Kaposiform hemangioendothelioma and not with common infantile hemangioma,” Plastic and Reconstructive Surgery, vol. 100, no. 6, pp. 1377–1386, 1997. View at Scopus
  3. O. Enjolras, M. Wassef, E. Mazoyer et al., “Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas,” Journal of Pediatrics, vol. 130, no. 4, pp. 631–640, 1997. View at Publisher · View at Google Scholar · View at Scopus
  4. M. El-Dessouky, A. F. Azmy, P. A. M. Raine, and D. G. Young, “Kasabach-Merritt syndrome,” Journal of Pediatric Surgery, vol. 23, no. 2, pp. 109–111, 1988. View at Scopus
  5. C. Haisley-Royster, O. Enjolras, I. J. Frieden et al., “Kasabach-Merritt phenomenon: a retrospective study of treatment with vincristine,” Journal of Pediatric Hematology/Oncology, vol. 24, no. 6, pp. 459–462, 2002. View at Publisher · View at Google Scholar · View at Scopus
  6. F. Fahrtash, E. McCahon, and S. Arbuckle, “Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine,” Journal of Pediatric Hematology/Oncology, vol. 32, no. 6, pp. 506–510, 2010. View at Publisher · View at Google Scholar · View at Scopus
  7. J. Hauer, U. Graubner, N. Konstantopoulos, S. Schmidt, T. Pfluger, and I. Schmid, “Effective treatment of kaposiform hemangioendotheliomas associated with Kasabach-Merritt phenomenon using four-drug regimen,” Pediatric Blood and Cancer, vol. 49, no. 6, pp. 852–854, 2007. View at Publisher · View at Google Scholar · View at Scopus
  8. V. LÓpez, N. MartÍ, C. Pereda et al., “Successful management of kaposiform hemangioendothelioma with kasabach-merritt phenomenon using vincristine and ticlopidine,” Pediatric Dermatology, vol. 26, no. 3, pp. 365–366, 2009. View at Publisher · View at Google Scholar · View at Scopus
  9. S. Q. Wolfe, H. Farhat, M. S. Elhammady, R. Moftakhar, and M. A. Aziz-Sultan, “Transarterial embolization of a scalp hemangioma presenting with Kasabach-Merritt syndrome: case report,” Journal of Neurosurgery, vol. 4, no. 5, pp. 453–457, 2009. View at Publisher · View at Google Scholar · View at Scopus
  10. C. Ryan, V. Price, P. John et al., “Kasabach-Merritt phenomenon: a single centre experience,” European Journal of Haematology, vol. 84, no. 2, pp. 97–104, 2010. View at Publisher · View at Google Scholar · View at Scopus
  11. M. Galbusera, M. Noris, and G. Remuzzi, “Inherited thrombotic thrombocytopenic purpura,” Haematologica, vol. 94, no. 2, pp. 166–170, 2009. View at Publisher · View at Google Scholar · View at Scopus
  12. F. Peyvandi, S. Lavoretano, R. Palla et al., “ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission,” Haematologica, vol. 93, no. 2, pp. 232–239, 2008. View at Publisher · View at Google Scholar · View at Scopus
  13. L. Copelovitch and B. S. Kaplan, “The thrombotic microangiopathies,” Pediatric Nephrology, vol. 23, no. 10, pp. 1761–1767, 2008. View at Publisher · View at Google Scholar · View at Scopus
  14. C. Loirat, J. P. Girma, C. Desconclois, P. Coppo, and A. Veyradier, “Thrombotic thrombocytopenic purpura related to severe ADAMTS13 deficiency in children,” Pediatric Nephrology, vol. 24, no. 1, pp. 19–29, 2009. View at Publisher · View at Google Scholar · View at Scopus
  15. H. M. Tsai and E. C. Y. Lian, “Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura,” The New England Journal of Medicine, vol. 339, no. 22, pp. 1585–1594, 1998. View at Publisher · View at Google Scholar · View at Scopus
  16. M. Furlan, R. Robles, M. Galbusera et al., “Von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome,” The New England Journal of Medicine, vol. 339, no. 22, pp. 1578–1584, 1998. View at Publisher · View at Google Scholar · View at Scopus
  17. M. Franchini and P. M. Mannucci, “Advantages and limits of ADAMTS13 testing in thrombotic thrombocytopenic purpura,” Blood Transfusion, vol. 6, no. 3, pp. 127–135, 2008. View at Publisher · View at Google Scholar · View at Scopus
  18. X. Long Zheng, H. M. Wu, D. Shang et al., “Multiple domains of ADAMTS13 are targeted by autoantibodies against ADAMTS13 in patients with acquired idiopathic thrombotic thrombocytopenic purpura,” Haematologica, vol. 95, no. 9, pp. 1555–1562, 2010. View at Publisher · View at Google Scholar · View at Scopus
  19. J. A. K. Hovinga, S. K. Vesely, D. R. Terrell, B. Lämmle, and J. N. George, “Survival and relapse in patients with thrombotic thrombocytopenic purpura,” Blood, vol. 115, no. 8, pp. 1500–1511, 2010. View at Publisher · View at Google Scholar · View at Scopus
  20. S. Ferrari, F. Scheiflinger, M. Rieger et al., “Prognostic value of anti-ADAMTS13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS13 activity,” Blood, vol. 109, no. 7, pp. 2815–2822, 2007. View at Publisher · View at Google Scholar · View at Scopus
  21. M. Michael, E. J. Elliott, J. C. Craig, G. Ridley, and E. M. Hodson, “Interventions for hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: a systematic review of randomized controlled trials,” American Journal of Kidney Diseases, vol. 53, no. 2, pp. 259–272, 2009. View at Publisher · View at Google Scholar · View at Scopus
  22. H. Yarranton and S. J. Machin, “An update on the pathogenesis and management of acquired thrombotic thrombocytopenic purpura,” Current Opinion in Neurology, vol. 16, no. 3, pp. 367–373, 2003. View at Publisher · View at Google Scholar · View at Scopus
  23. G. Antoine, K. Zimmermann, B. Plaimauer et al., “ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13,” British Journal of Haematology, vol. 120, no. 5, pp. 821–824, 2003. View at Publisher · View at Google Scholar · View at Scopus
  24. F. B. Mayr, P. Knöbl, B. Jilma et al., “The aptamer ARC1779 blocks von Willebrand factor-dependent platelet function in patients with thrombotic thrombocytopenic purpura ex vivo,” Transfusion, vol. 50, no. 5, pp. 1079–1087, 2010. View at Publisher · View at Google Scholar · View at Scopus
  25. J. C. Gilbert, T. DeFeo-Fraulini, R. M. Hutabarat et al., “First-in-human evaluation of anti-von Willebrand factor therapeutic aptamer ARC1779 in healthy volunteers,” Circulation, vol. 116, no. 23, pp. 2678–2686, 2007. View at Publisher · View at Google Scholar · View at Scopus
  26. M. Niiya, M. Endo, D. Shang et al., “Correction of ADAMTS13 deficiency by in utero gene transfer of lentiviral vector encoding ADAMTS13 genes,” Molecular Therapy, vol. 17, no. 1, pp. 34–41, 2009. View at Publisher · View at Google Scholar · View at Scopus
  27. G. Remuzzi and P. Ruggenenti, “The hemolytic uremic syndrome,” Kidney International, Supplement, vol. 53, supplement 66, pp. S54–S57, 1998. View at Scopus
  28. M. Noris and G. Remuzzi, “Atypical hemolytic-uremic syndrome,” The New England Journal of Medicine, vol. 361, no. 17, pp. 1675–1687, 2009. View at Scopus
  29. L. H. Gould, L. Demma, T. F. Jones et al., “Hemolytic uremic syndrome and death in persons with Escherichia coli O157:H7 infection, foodborne diseases active surveillance network sites, 2000–2006,” Clinical Infectious Diseases, vol. 49, no. 10, pp. 1480–1485, 2009. View at Publisher · View at Google Scholar · View at Scopus
  30. E. Espié, F. Grimont, P. Mariani-Kurkdjian et al., “Surveillance of hemolytic uremic syndrome in children less than 15 years of age, a system to monitor o157 and non-o157 shiga toxin-producing Escherichia coli infections in France, 1996–2006,” Pediatric Infectious Disease Journal, vol. 27, no. 7, pp. 595–601, 2008. View at Publisher · View at Google Scholar · View at Scopus
  31. D. Karpman, M. Manea, F. Vaziri-Sani, A. L. Ståhl, and A. C. Kristoffersson, “Platelet activation in hemolytic uremic syndrome,” Seminars in Thrombosis and Hemostasis, vol. 32, no. 2, pp. 128–145, 2006. View at Scopus
  32. S. P. Andreoli, H. Trachtman, D. W. K. Acheson, R. L. Siegler, and T. G. Obrig, “Hemolytic Uremic Syndrome: epidemiology, pathophysiology and therapy,” Pediatric Nephrology, vol. 17, no. 4, pp. 293–298, 2002. View at Publisher · View at Google Scholar · View at Scopus
  33. K. I. Jeong, S. Chapman-Bonofiglio, P. Singh, J. Lee, S. Tzipori, and A. S. Sheoran, “In vitro and in vivo protective efficacies of antibodies that neutralize the RNA N-glycosidase activity of Shiga toxin 2,” BMC Immunology, vol. 11, article 16, 2010. View at Publisher · View at Google Scholar · View at Scopus
  34. M. Bitzan, R. Poole, M. Mehran et al., “Safety and pharmacokinetics of chimeric anti-shiga toxin 1 and anti-shiga toxin 2 monoclonal antibodies in healthy volunteers,” Antimicrobial Agents and Chemotherapy, vol. 53, no. 7, pp. 3081–3087, 2009. View at Publisher · View at Google Scholar · View at Scopus
  35. A. M. Waters, L. Kerecuk, D. Luk et al., “Hemolytic uremic syndrome associated with invasive pneumococcal disease: the United Kingdom experience,” Journal of Pediatrics, vol. 151, no. 2, pp. 140–144, 2007. View at Publisher · View at Google Scholar · View at Scopus
  36. C. Prestidge and W. Wong, “Ten years of pneumococcal-associated haemolytic uraemic syndrome in New Zealand children,” Journal of Paediatrics and Child Health, vol. 45, no. 12, pp. 731–735, 2009. View at Publisher · View at Google Scholar · View at Scopus
  37. Ö. Köse, L. B. Zimmerhackl, T. Jungraithmayr, C. MacHe, and J. Nürnberger, “New treatment options for atypical hemolytic uremic syndrome with the complement inhibitor eculizumab,” Seminars in Thrombosis and Hemostasis, vol. 36, no. 6, pp. 669–672, 2010. View at Publisher · View at Google Scholar · View at Scopus
  38. V. Frémeaux-Bacchi, E. C. Miller, M. K. Liszewski et al., “Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome,” Blood, vol. 112, no. 13, pp. 4948–4952, 2008. View at Publisher · View at Google Scholar · View at Scopus
  39. D. K. Lhotta, A. R. Janecke, J. Scheiring et al., “A large family with a gain-of-function mutation of complement C3 predisposing to atypical hemolytic uremic syndrome, microhematuria, hypertension and chronic renal failure,” Clinical Journal of the American Society of Nephrology, vol. 4, no. 8, pp. 1356–1362, 2009. View at Publisher · View at Google Scholar · View at Scopus
  40. E. Goicoechea De Jorge, C. L. Harris, J. Esparza-Gordillo et al., “Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome,” Proceedings of the National Academy of Sciences of the United States of America, vol. 104, no. 1, pp. 240–245, 2007. View at Publisher · View at Google Scholar · View at Scopus
  41. C. Skerka, M. Józsi, P. F. Zipfel, M. A. Dragon-Durey, and V. Fremeaux-Bacchi, “Autoantibodies in haemolytic uraemic syndrome (HUS),” Thrombosis and Haemostasis, vol. 101, no. 2, pp. 227–232, 2009. View at Publisher · View at Google Scholar · View at Scopus
  42. J. Dervenoulas, P. Tsirigotis, G. Bollas et al., “Thrombotic thrombocytopenic purpura/hemolytic uremic syndrome (TTP/HUS]: treatment outcome, relapses, prognostic factors. A single-center experience of 48 cases,” Annals of Hematology, vol. 79, no. 2, pp. 66–72, 2000. View at Scopus
  43. J. L. Moake and J. J. Byrnes, “Thrombotic microangiopathies associated with drugs and bone marrow transplantation,” Hematology/Oncology Clinics of North America, vol. 10, no. 2, pp. 485–497, 1996. View at Publisher · View at Google Scholar · View at Scopus
  44. K. Ramasubbu, T. Mullick, A. Koo et al., “Thrombotic microangiopathy and cytomegalovirus in liver transplant recipients: a case-based review,” Transplant Infectious Disease, vol. 5, no. 2, pp. 98–103, 2003. View at Publisher · View at Google Scholar · View at Scopus
  45. R. L. Paquette, L. Tran, and E. M. Landaw, “Thrombotic microangiopathy following allogeneic bone marrow transplantation is associated with intensive graft-versus-host disease prophylaxis,” Bone Marrow Transplantation, vol. 22, no. 4, pp. 351–357, 1998. View at Scopus
  46. R. Kato, A. Shinohara, and J. Sato, “ADAMTS13 deficiency, an important cause of thrombocytopenia during pregnancy,” International Journal of Obstetric Anesthesia, vol. 18, no. 1, pp. 73–77, 2009. View at Publisher · View at Google Scholar · View at Scopus
  47. J. Gerth, E. Schleussner, K. Kentouche, M. Busch, M. Seifert, and G. Wolf, “Pregnancy-associated thrombotic thrombocytopenic purpura,” Thrombosis and Haemostasis, vol. 101, no. 2, pp. 248–251, 2009. View at Publisher · View at Google Scholar · View at Scopus
  48. F. A. Niaz and A. Aleem, “Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus,” Saudi Journal of Kidney Diseases and Transplantation, vol. 21, no. 1, pp. 109–112, 2010. View at Scopus
  49. S. Changsirikulchai, D. Myerson, K. A. Guthrie, G. B. McDonald, C. E. Alpers, and S. R. Hingorani, “Renal thrombotic microangiopathy after hematopoietic cell transplant: role of GVHD in pathogenesis,” Clinical Journal of the American Society of Nephrology, vol. 4, no. 2, pp. 345–353, 2009. View at Publisher · View at Google Scholar · View at Scopus
  50. D. Iwami, H. Harada, K. Hotta et al., “A case of pregnancy-induced thrombotic thrombocytopenic purpura with a kidney allograft recipient,” Clinical Transplantation, vol. 24, no. 22, pp. 66–69, 2010. View at Publisher · View at Google Scholar · View at Scopus
  51. T. Zheng, L. Chunlei, W. Zhen et al., “Clinical-pathological features and prognosis of thrombotic thrombocytopenic purpura in patients with lupus nephritis,” American Journal of the Medical Sciences, vol. 338, no. 5, pp. 343–347, 2009. View at Publisher · View at Google Scholar · View at Scopus
  52. P. Iacopino, G. Pucci, W. Arcese et al., “Severe thrombotic microangiopathy: an infrequent complication of bone marrow transplantation,” Bone Marrow Transplantation, vol. 24, no. 1, pp. 47–51, 1999. View at Scopus
  53. J. N. Martin Jr., A. P. Bailey, J. F. Rehberg, M. T. Owens, S. D. Keiser, and W. L. May, “Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955–2006,” American Journal of Obstetrics and Gynecology, vol. 199, no. 2, pp. 98–104, 2008. View at Publisher · View at Google Scholar · View at Scopus
  54. A. Belford, O. Myles, A. Magill, J. Wang, R. C. Myhand, and J. K. Waselenko, “Thrombotic microangiopathy (TMA) and stroke due to human herpesvirus-6 (HHV-6) reactivation in an adult receiving high-dose melphalan with autologous peripheral stem cell transplantation,” American Journal of Hematology, vol. 76, no. 2, pp. 156–162, 2004. View at Publisher · View at Google Scholar · View at Scopus
  55. T. Nishida, M. Hamaguchi, N. Hirabayashi et al., “Intestinal thrombotic microangiopathy after allogeneic bone marrow transplantation: a clinical imitator of acute enteric graft-versus-host disease,” Bone Marrow Transplantation, vol. 33, no. 11, pp. 1143–1150, 2004. View at Publisher · View at Google Scholar · View at Scopus
  56. M. Davies, S. Maiti, P. H. B. Bolton-Maggs, and L. Byrd, “Successful vaginal delivery in a patient with extreme thrombotic thrombocytopenic purpura at term,” Journal of Obstetrics and Gynaecology, vol. 29, no. 8, pp. 765–766, 2009. View at Publisher · View at Google Scholar · View at Scopus
  57. J. N. George, “Thrombotic thrombocytopenic purpura,” The New England Journal of Medicine, vol. 354, no. 18, pp. 1927–1935, 2006. View at Publisher · View at Google Scholar · View at Scopus
  58. M. C. Bouw, N. Dors, H. Van Ommen, and N. L. Ramakers-van Woerden, “Thrombotic thrombocytopenic purpura in childhood,” Pediatric Blood and Cancer, vol. 53, no. 4, pp. 537–542, 2009. View at Publisher · View at Google Scholar · View at Scopus
  59. L. Murer, G. Zacchello, D. Bianchi et al., “Thrombotic microangiopathy associated with parvovirus B19 infection after renal transplantation,” Journal of the American Society of Nephrology, vol. 11, no. 6, pp. 1132–1137, 2000. View at Scopus
  60. D. R. Terrell, S. K. Vesely, J. A. K. Hovinga, B. Lämmle, and J. N. George, “Different disparities of gender and race among the thrombotic thrombocytopenic purpura and hemolytic-uremic syndromes,” American Journal of Hematology, vol. 85, no. 11, pp. 844–847, 2010. View at Publisher · View at Google Scholar · View at Scopus
  61. M. R. Ardalan, “Review of thrombotic microangiopathy (TMA), and post-renal transplant TMA,” Saudi Journal of Kidney Diseases and Transplantation, vol. 17, no. 2, pp. 235–244, 2006. View at Scopus
  62. C. L. Stella, J. Dacus, E. Guzman et al., “The diagnostic dilemma of thrombotic thrombocytopenic purpura/hemolytic uremic syndrome in the obstetric triage and emergency department: lessons from 4 tertiary hospitals,” American Journal of Obstetrics and Gynecology, vol. 200, no. 4, pp. 381–e1, 2009. View at Publisher · View at Google Scholar · View at Scopus
  63. F. Fakhouri, L. Roumenina, F. Provot et al., “Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations,” Journal of the American Society of Nephrology, vol. 21, no. 5, pp. 859–867, 2010. View at Publisher · View at Google Scholar · View at Scopus
  64. Y. Fujimura, M. Matsumoto, K. Kokame et al., “Pregnancy-induced thrombocytopenia and TTP, and the risk of fetal death, in Upshaw-Schulman syndrome: a series of 15 pregnancies in 9 genotyped patients,” British Journal of Haematology, vol. 144, no. 5, pp. 742–754, 2009. View at Publisher · View at Google Scholar · View at Scopus
  65. C. N. Ross, H. Reuter, D. Scott, and D. V. Hamilton, “Microangiopathic haemolytic anaemia and systemic vasculitis,” British Journal of Rheumatology, vol. 35, no. 4, pp. 377–379, 1996. View at Scopus
  66. E. Rozdzinski, B. Hertenstein, T. Schmeiser, E. Seifried, E. Kurrle, and H. Heimpel, “Thrombotic thrombocytopenic purpura in early pregnancy with maternal and fetal survival,” Annals of Hematology, vol. 64, no. 5, pp. 245–248, 1992. View at Publisher · View at Google Scholar · View at Scopus
  67. S. Tamura, Y. Sugawara, Y. Matsui et al., “Thrombotic microangiopathy in living-donor liver transplantation,” Transplantation, vol. 80, no. 2, pp. 169–175, 2005. View at Publisher · View at Google Scholar · View at Scopus
  68. A. Zarifian, S. Meleg-Smith, R. O'Donovan, R. J. Tesi, and V. Batuman, “Cyclosporine-associated thrombotic microangiopathy in renal allografts,” Kidney International, vol. 55, no. 6, pp. 2457–2466, 1999. View at Publisher · View at Google Scholar · View at Scopus
  69. S. K. Kwok, J. H. Ju, C. S. Cho, H. Y. Kim, and S. H. Park, “Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study,” Lupus, vol. 18, no. 1, pp. 16–21, 2009. View at Scopus
  70. A. Sakarcan and J. Stallworth, “Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case and review,” Pediatric Nephrology, vol. 16, no. 8, pp. 672–674, 2001. View at Publisher · View at Google Scholar · View at Scopus
  71. W. K. Chak, D. S. Y. Lam, W. H. Lo, C. M. Hui, and S. N. Wong, “Thrombotic thrombocytopenic purpura as a rare complication in childhood systemic lupus erythematosus: case report and literature review,” Hong Kong Medical Journal, vol. 9, no. 5, pp. 363–368, 2003. View at Scopus