Thrombotic Microangiopathies

Overview

Journal ISRN Hematol

Specialty Hematology

Date 2012 Aug 14

PMID 22888446

Citations 10

Authors

Mohamed Radhi

Shannon L Carpenter

Affiliations

Soon will be listed here.

Abstract

Thrombotic microangiopathy results from thrombotic occlusion of the microvasculature leading to fragmentation of red blood cells, profound thrombocytopenia, and a microangiopathic hemolytic anemia with elevation of lactate dehydrogenase and negative direct Coomb's test. This constellation of clinical and laboratory findings is not due to one disease entity; rather, it represents a variety of underlying diagnoses. Among the major disease entities are TTP/HUS, which can be congenital or acquired, bacterial infections, medications, vascular or endothelial pathology like Kasabach-Merritt phenomenon, and stem cell transplantation. In this paper, we offer a review of some of the major causes of thrombotic microangiopathy.

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References

Espie E, Grimont F, Mariani-Kurkdjian P, Bouvet P, Haeghebaert S, Filliol I . 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. Pediatr Infect Dis J. 2008; 27(7):595-601. DOI: 10.1097/INF.0b013e31816a062f. View

Zheng T, Chunlei L, Zhen W, Ping L, Haitao Z, Weixin H . Clinical-pathological features and prognosis of thrombotic thrombocytopenic purpura in patients with lupus nephritis. Am J Med Sci. 2009; 338(5):343-7. DOI: 10.1097/MAJ.0b013e3181b0c872. View

Kwok S, Ju J, Cho C, Kim H, Park S . Thrombotic thrombocytopenic purpura in systemic lupus erythematosus: risk factors and clinical outcome: a single centre study. Lupus. 2008; 18(1):16-21. DOI: 10.1177/0961203308094360. View

Bouw M, Dors N, van Ommen H, Ramakers-van Woerden N . Thrombotic thrombocytopenic purpura in childhood. Pediatr Blood Cancer. 2009; 53(4):537-42. DOI: 10.1002/pbc.22094. View

Skerka C, Jozsi M, Zipfel P, Dragon-Durey M, Fremeaux-Bacchi V . Autoantibodies in haemolytic uraemic syndrome (HUS). Thromb Haemost. 2009; 101(2):227-32. View

Moake J, Byrnes J . Thrombotic microangiopathies associated with drugs and bone marrow transplantation. Hematol Oncol Clin North Am. 1996; 10(2):485-97. DOI: 10.1016/s0889-8588(05)70348-8. View

Noris M, Remuzzi G . Atypical hemolytic-uremic syndrome. N Engl J Med. 2009; 361(17):1676-87. DOI: 10.1056/NEJMra0902814. View

Mayr F, Knobl P, Jilma B, Siller-Matula J, Wagner P, Schaub R . The aptamer ARC1779 blocks von Willebrand factor-dependent platelet function in patients with thrombotic thrombocytopenic purpura ex vivo. Transfusion. 2010; 50(5):1079-87. DOI: 10.1111/j.1537-2995.2009.02554.x. View

Andreoli S, Trachtman H, Acheson D, Siegler R, Obrig T . Hemolytic uremic syndrome: epidemiology, pathophysiology, and therapy. Pediatr Nephrol. 2002; 17(4):293-8. DOI: 10.1007/s00467-001-0783-0. View

10.

Fahrtash F, McCahon E, Arbuckle S . Successful treatment of kaposiform hemangioendothelioma and tufted angioma with vincristine. J Pediatr Hematol Oncol. 2010; 32(6):506-10. DOI: 10.1097/MPH.0b013e3181e001a9. View

11.

Niaz F, Aleem A . Response to rituximab in a refractory case of thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus. Saudi J Kidney Dis Transpl. 2010; 21(1):109-12. View

12.

Copelovitch L, Kaplan B . The thrombotic microangiopathies. Pediatr Nephrol. 2007; 23(10):1761-7. PMC: 6901414. DOI: 10.1007/s00467-007-0616-x. View

13.

Chak W, Lam D, Lo W, Hui C, Wong S . Thrombotic thrombocytopenic purpura as a rare complication in childhood systemic lupus erythematosus: case report and literature review. Hong Kong Med J. 2003; 9(5):363-8. View

14.

Gilbert J, DeFeo-Fraulini T, Hutabarat R, Horvath C, Merlino P, Marsh H . First-in-human evaluation of anti von Willebrand factor therapeutic aptamer ARC1779 in healthy volunteers. Circulation. 2007; 116(23):2678-86. DOI: 10.1161/CIRCULATIONAHA.107.724864. View

15.

Ryan C, Price V, John P, Mahant S, Baruchel S, Brandao L . Kasabach-Merritt phenomenon: a single centre experience. Eur J Haematol. 2009; 84(2):97-104. DOI: 10.1111/j.1600-0609.2009.01370.x. View

16.

Sakarcan A, Stallworth J . Systemic lupus erythematosus and thrombotic thrombocytopenic purpura: a case and review. Pediatr Nephrol. 2001; 16(8):672-4. DOI: 10.1007/s004670100625. View

17.

Martin Jr J, Bailey A, Rehberg J, Owens M, Keiser S, May W . Thrombotic thrombocytopenic purpura in 166 pregnancies: 1955-2006. Am J Obstet Gynecol. 2008; 199(2):98-104. DOI: 10.1016/j.ajog.2008.03.011. View

18.

Peyvandi F, Lavoretano S, Palla R, Feys H, Vanhoorelbeke K, Battaglioli T . ADAMTS13 and anti-ADAMTS13 antibodies as markers for recurrence of acquired thrombotic thrombocytopenic purpura during remission. Haematologica. 2008; 93(2):232-9. DOI: 10.3324/haematol.11739. View

19.

Kremer Hovinga J, Vesely S, Terrell D, Lammle B, George J . Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood. 2009; 115(8):1500-11. DOI: 10.1182/blood-2009-09-243790. View

20.

Changsirikulchai S, Myerson D, Guthrie K, McDonald G, Alpers C, Hingorani S . Renal thrombotic microangiopathy after hematopoietic cell transplant: role of GVHD in pathogenesis. Clin J Am Soc Nephrol. 2009; 4(2):345-53. PMC: 2637592. DOI: 10.2215/CJN.02070508. View