How I treat catastrophic thrombotic syndromes

Blood

Volumn 126, Issue 11, 2015, Pages 1285-1293

  Ortel, Thomas L ^a   Erkan, Doruk ^b   Kitchens, Craig S ^c  

^a DUKE UNIVERSITY MEDICAL CENTER   (United States)

^b WEILL CORNELL MEDICAL COLLEGE   (United States)

^c UNIVERSITY OF FLORIDA   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ANTICOAGULANT AGENT; BLOOD CLOTTING FACTOR 10A; HEPARIN;

ANTICOAGULANT THERAPY; ANTICOAGULATION; ANTIPHOSPHOLIPID SYNDROME; HEPARIN INDUCED THROMBOCYTOPENIA; HUMAN; IMMUNOSUPPRESSIVE TREATMENT; PARTIAL THROMBOPLASTIN TIME; PHENOTYPE; PLASMAPHERESIS; PREGNANCY; PRIORITY JOURNAL; RECURRENCE RISK; REVIEW; THROMBOEMBOLISM; THROMBOTIC THROMBOCYTOPENIC PURPURA; TREATMENT INDICATION; TUMOR THROMBUS; VENA CAVA FILTER; ADOLESCENT; CASE REPORT; CHEMICALLY INDUCED; FEMALE; MALE; PURPURA, THROMBOTIC THROMBOCYTOPENIC; SYNDROME; TERMINAL DISEASE; THROMBOCYTOPENIA; THROMBOSIS;

ADOLESCENT; ANTICOAGULANTS; ANTIPHOSPHOLIPID SYNDROME; CATASTROPHIC ILLNESS; FEMALE; HEPARIN; HUMANS; MALE; PLASMA EXCHANGE; PREGNANCY; PURPURA, THROMBOTIC THROMBOCYTOPENIC; SYNDROME; THROMBOCYTOPENIA; THROMBOSIS;

EID: 84942474644     PISSN: 00064971     EISSN: 15280020     Source Type: Journal    
DOI: 10.1182/blood-2014-09-551978     Document Type: Review

References (86)

1. Kitchens CS. Thrombotic storm: when thrombosis begets thrombosis. Am J Med. 1998;104(4):381-385.
2. Kitchens CS, Erkan D, Brandão LR, et al. Thrombotic storm revisited: preliminary diagnostic criteria suggested by the thrombotic storm study group. Am J Med. 2011;124(4):290-296.
3. Manco-Johnson MJ, Wang M, Goldenberg NA, et al. Treatment, survival, and thromboembolic outcomes of thrombotic storm in children. J Pediatr. 2012;161(4):682.e1-688.e1.
4. Asherson RA. The catastrophic antiphospholipid syndrome. J Rheumatol. 1992;19(4):508-512.
5. Asherson RA, Cervera R, Piette JC, et al. Catastrophic antiphospholipid syndrome. Clinical and laboratory features of 50 patients. Medicine (Baltimore). 1998;77(3):195-207.
6. Cervera R, Serrano R, Pons-Estel GJ, et al; Euro-Phospholipid Project Group (European Forum on Antiphospholipid Antibodies). Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: a multicentre prospective study of 1000 patients. Ann Rheum Dis. 2015;74(6):1011-1018.
7. Asherson RA, Cervera R, de Groot PG, et al; Catastrophic Antiphospholipid Syndrome Registry Project Group. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus. 2003;12(7):530-534.
8. Berman H, Rodríguez-Pintó I, Cervera R, et al; Catastrophic Registry Project Group (European Forum on Antiphospholipid Antibodies). Pediatric catastrophic antiphospholipid syndrome: descriptive analysis of 45 patients from the "CAPS Registry". Autoimmun Rev. 2014;13(2):157-162.
9. Cervera R; CAPS Registry Project Group. Catastrophic antiphospholipid syndrome (CAPS): update from the 'CAPS Registry'. Lupus. 2010;19(4):412-418.
10. Cervera R, Bucciarelli S, Plasín MA, et al; Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum On Antiphospholipid Antibodies). Catastrophic antiphospholipid syndrome (CAPS): descriptive analysis of a series of 280 patients from the "CAPS Registry". J Autoimmun. 2009;32(3-4):240-245.
11. Erkan D, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: updated diagnostic algorithms. Autoimmun Rev. 2010;10(2):74-79.
12. Aguiar CL, Erkan D. Catastrophic antiphospholipid syndrome: how to diagnose a rare but highly fatal disease. Ther Adv Musculoskelet Dis. 2013;5(6):305-314.
13. Espinosa G, Berman H, Cervera R. Management of refractory cases of catastrophic antiphospholipid syndrome. Autoimmun Rev. 2011;10(11):664-668.
14. Kronbichler A, Frank R, Kirschfink M, et al. Efficacy of eculizumab in a patient with immunoadsorption-dependent catastrophic antiphospholipid syndrome: a case report. Medicine (Baltimore). 2014;93(26):e143.
15. Strakhan M, Hurtado-Sbordoni M, Galeas N, Bakirhan K, Alexis K, Elrafei T. 36-year-old female with catastrophic antiphospholipid syndrome treated with eculizumab: a case report and review of literature. Case Rep Hematol. 2014;2014:704371.
16. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(7):654-666.
17. Idowu M, Reddy P. Atypical thrombotic thrombocytopenic purpura in a middle-aged woman who presented with a recurrent stroke. Am J Hematol. 2013;88(3):237-239.
18. Sarode R. Atypical presentations of thrombotic thrombocytopenic purpura: a review. J Clin Apher. 2009;24(1):47-52.
19. Yu WL, Leung T, Soo Y, Lee J, Wong KS. Thrombotic thrombocytopenic purpura with concomitant small- and large-vessel thrombosis, atypical posterior reversible encephalopathy syndrome and cerebral microbleeds. Oxf Med Case Reports. 2015;2015(2):179-182.
20. George JN, Chen Q, Deford CC, Al-Nouri Z. Ten patient stories illustrating the extraordinarily diverse clinical features of patients with thrombotic thrombocytopenic purpura and severe ADAMTS13 deficiency. J Clin Apher. 2012;27(6):302-311.
21. Imanirad I, Rajasekhar A, Zumberg M. A case series of atypical presentations of thrombotic thrombocytopenic purpura. J Clin Apher. 2012;27(4):221-226.
22. Musio F, Bohen EM, Yuan CM, Welch PG. Review of thrombotic thrombocytopenic purpura in the setting of systemic lupus erythematosus. Semin Arthritis Rheum. 1998;28(1):1-19.
23. Douglas KW, Pollock KG, Young D, Catlow J, Green R. Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors: a single-institution experience. J Clin Apher. 2010;25(2):47-53.
24. Yenigun EC, Bardak S, Piskinpasa SV, et al. Acute thrombotic thrombocytopenic purpura following orthopedic surgery: case report and review of the literature. Ren Fail. 2012;34(7):937-939.
25. McDonald V, Laffan M, Benjamin S, Bevan D, Machin S, Scully MA. Thrombotic thrombocytopenic purpura precipitated by acute pancreatitis: a report of seven cases from a regional UK TTP registry. Br J Haematol. 2009;144(3):430-433.
26. Arepally GM, Ortel TL. Heparin-induced thrombocytopenia. Annu Rev Med. 2010;61:77-90.
27. Greinacher A, Farner B, Kroll H, Kohlmann T, Warkentin TE, Eichler P. Clinical features of heparin-induced thrombocytopenia including risk factors for thrombosis. A retrospective analysis of 408 patients. Thromb Haemost. 2005;94(1):132-135.
28. Pantula NR, Vedula K. Catastrophic complication following heparin therapy. Asian Cardiovasc Thorac Ann. 2014;22(9):1096-1098.
29. Omran AS, Karimi A, Ahmadi H, Yazdanifard P. Delayed-onset heparin-induced thrombocytopenia presenting with multiple arteriovenous thromboses: case report. J Med Case Reports. 2007;1:131.
30. Warkentin TE. Agents for the treatment of heparin-induced thrombocytopenia. Hematol Oncol Clin North Am. 2010;24(4):755-775.
31. Linkins LA, Warkentin TE. Heparin-induced thrombocytopenia: real-world issues. Semin Thromb Hemost. 2011;37(6):653-663.
32. Warkentin TE, Sikov WM, Lillicrap DP. Multicentric warfarin-induced skin necrosis complicating heparin-induced thrombocytopenia. Am J Hematol. 1999;62(1):44-48.
33. Warkentin TE, Elavathil LJ, Hayward CP, Johnston MA, Russett JI, Kelton JG. The pathogenesis of venous limb gangrene associated with heparin-induced thrombocytopenia. Ann Intern Med. 1997;127(9):804-812.
34. Mallik A, Carlson KB, DeSancho MT. A patient with 'spontaneous' heparin-induced thrombocytopenia and thrombosis after undergoing knee replacement. Blood Coagul Fibrinolysis. 2011;22(1):73-75.
35. Warkentin TE, Makris M, Jay RM, Kelton JG. A spontaneous prothrombotic disorder resembling heparin-induced thrombocytopenia. Am J Med. 2008;121(7):632-636.
36. Jay RM, Warkentin TE. Fatal heparin-induced thrombocytopenia (HIT) during warfarin thromboprophylaxis following orthopedic surgery: another example of 'spontaneous' HIT? J Thromb Haemost. 2008;6(9):1598-1600.
37. Pruthi RK, Daniels PR, Nambudiri GS, Warkentin TE. Heparin-induced thrombocytopenia (HIT) during postoperative warfarin thromboprophylaxis: a second example of postorthopedic surgery 'spontaneous' HIT. J Thromb Haemost. 2009;7(3):499-501.
38. Okata T, Miyata S, Miyashita F, Maeda T, Toyoda K. Spontaneous heparin-induced thrombocytopenia syndrome without any proximate heparin exposure, infection, or inflammatory condition: atypical clinical features with heparin-dependent platelet activating antibodies [published online ahead of print November 10, 2014]. Platelets.
39. Warkentin TE, Basciano PA, Knopman J, Bernstein RA. Spontaneous heparin-induced thrombocytopenia syndrome: 2 new cases and a proposal for defining this disorder. Blood. 2014;123(23):3651-3654.
40. Perrin J, Barraud D, Toussaint-Hacquard M, Bollaert PE, Lecompte T. Rapid onset heparin-induced thrombocytopenia (HIT) without history of heparin exposure: a new case of so-called 'spontaneous' HIT. Thromb Haemost. 2012;107(4):795-797.
41. Schell AM, Petras M, Szczepiorkowski ZM, Ornstein DL. Refractory heparin induced thrombocytopenia with thrombosis (HITT) treated with therapeutic plasma exchange and rituximab as adjuvant therapy. Transfus Apheresis Sci. 2013;49(2):185-188.
42. Blom JW, Doggen CJ, Osanto S, Rosendaal FR. Malignancies, prothrombotic mutations, and the risk of venous thrombosis. JAMA. 2005;293(6):715-722.
43. Prandoni P, Lensing AW, Piccioli A, et al. Recurrent venous thromboembolism and bleeding complications during anticoagulant treatment in patients with cancer and venous thrombosis. Blood. 2002;100(10):3484-3488.
44. Kuderer NM, Ortel TL, Francis CW. Impact of venous thromboembolism and anticoagulation on cancer and cancer survival. J Clin Oncol. 2009;27(29):4902-4911.
45. Varki A. Trousseau's syndrome: multiple definitions and multiple mechanisms. Blood. 2007;110(6):1723-1729.
46. Sack GH Jr, Levin J, Bell WR. Trousseau's syndrome and other manifestations of chronic disseminated coagulopathy in patients with neoplasms: clinical, pathophysiologic, and therapeutic features. Medicine (Baltimore). 1977;56(1):1-37.
47. Lee AY, Peterson EA. Treatment of cancer-associated thrombosis. Blood. 2013;122(14):2310-2317.
48. Bell WR, Starksen NF, Tong S, Porterfield JK. Trousseau's syndrome. Devastating coagulopathy in the absence of heparin. Am J Med. 1985;79(4):423-430.
49. Ng HJ, Crowther MA. Malignancy-associated venous thrombosis with concurrent warfarin-induced skin necrosis, venous limb gangrene and thrombotic microangiopathy. Thromb Haemost. 2006;95(6):1038-1039.
50. Warkentin TE. Venous limb gangrene during warfarin treatment of cancer-associated deep venous thrombosis. Ann Intern Med. 2001;135(8 Pt 1):589-593.
51. Warkentin TE, Cook RJ, Sarode R, Sloane DA, Crowther MA. Warfarin-induced venous limb ischemia/gangrene complicating cancer: a novel and clinically distinct syndrome [published online ahead of print May 15, 2015]. Blood. 2015;126(4):486-493. doi:10.1182/blood-2015-01-622787.
52. Rosenbaum AN, Yu RC, Rooke TW, Heit JA. Venous gangrene and intravascular coagulation and fibrinolysis in a patient treated with rivaroxaban. Am J Med. 2014;127(6):e7-e8.
53. Ideguchi H, Ohno S, Ueda A, Ishigatsubo Y. Catastrophic antiphospholipid syndrome associated with malignancies (case report and review of the literature). Lupus. 2007;16(1):59-64.
54. Miesbach W, Asherson RA, Cervera R, et al; Members of CAPS Registry Group. The catastrophic antiphospholipid (Asherson's) syndrome and malignancies. Autoimmun Rev. 2006;6(2):94-97.
55. Lechner K, Obermeier HL. Cancer-related microangiopathic hemolytic anemia: clinical and laboratory features in 168 reported cases. Medicine (Baltimore). 2012;91(4):195-205.
56. Todd S, Hemmaway C, Nagy Z. Catastrophic thrombosis in idiopathic hypereosinophilic syndrome. Br J Haematol. 2014;165(4):425.
57. Park SM, Park JW, Kim SM, et al. A case of hypereosinophilic syndrome presenting with multiorgan infarctions associated with disseminated intravascular coagulation. Allergy Asthma Immunol Res. 2012;4(3):161-164.
58. Liu H, Al-Quran SZ, Lottenberg R. Thrombotic storm in Kimura disease. J Thromb Thrombolysis. 2010;29(3):354-357.
59. Maggi U, Rossi G, Avesani EC, et al. Thrombotic storm in a teenager with previously undiagnosed ulcerative colitis. Pediatrics. 2013;131(4):e1288-e1291.
60. Bittencourt MJ, Dias CM, Lage TL, Barros RS, Paz OA, Vieira WB. Behçet disease in association with Budd-Chiari syndrome and multiple thrombosis - case report. An Bras Dermatol. 2013;88(3):448-451.
61. Celik G, Yildirm E, Narci H, Ozulku M. Diffuse thrombosis secondary to Behçet's disease: a case report. Am J Emerg Med. 2013;31(12):1723.e1-2.
62. Kuzu MA, Ozaslan C, Köksoy C, Gürler A, Tüzüner A. Vascular involvement in Behçet's disease: 8-year audit. World J Surg. 1994;18(6):948-953.
63. Tascilar K, Melikoglu M, Ugurlu S, Sut N, Caglar E, Yazici H. Vascular involvement in Behçet's syndrome: a retrospective analysis of associations and the time course. Rheumatology (Oxford). 2014;53(11):2018-2022.
64. Seligsohn U, Berger A, Abend M, et al. Homozygous protein C deficiency manifested by massive venous thrombosis in the newborn. N Engl J Med. 1984;310(9):559-562.
65. Mahasandana C, Suvatte V, Marlar RA, Manco-Johnson MJ, Jacobson LJ, Hathaway WE. Neonatal purpura fulminans associated with homozygous protein S deficiency. Lancet. 1990;335(8680):61-62.
66. Boey JP, Jolley A, Nicholls C, et al. Novel protein C gene mutation in a compound heterozygote resulting in catastrophic thrombosis in early adulthood: diagnosis and long-term treatment with subcutaneous protein C concentrate [June 24, 2015]. Br J Haematol.
67. Garcia DA, Baglin TP, Weitz JI, Samama MM. Parenteral anticoagulants: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012;141(suppl 2):e24S-e43S.
68. Uprichard J, Manning RA, Laffan MA. Monitoring heparin anticoagulation in the acute phase response. Br J Haematol. 2010;149(4):613-619.
69. Olson JD, Arkin CF, Brandt JT, et al. College of American Pathologists Conference XXXI on Laboratory Monitoring of Anticoagulant Therapy: laboratory monitoring of unfractionated heparin therapy. Arch Pathol Lab Med. 1998;122(9):782-798.
70. Levine MN, Hirsh J, Gent M, et al. A randomized trial comparing activated thromboplastin time with heparin assay in patients with acute venous thromboembolism requiring large daily doses of heparin. Arch Intern Med. 1994;154(1):49-56.
71. Avidan MS, Levy JH, van Aken H, et al. Recombinant human antithrombin III restores heparin responsiveness and decreases activation of coagulation in heparin-resistant patients during cardiopulmonary bypass. J Thorac Cardiovasc Surg. 2005;130(1):107-113.
72. Treichl B, Bachler M, Lorenz I, et al. Efficacy of argatroban in critically ill patients with heparin resistance: a retrospective analysis. Semin Thromb Hemost. 2015;41(1):61-67.
73. Kennedy DM, Alaniz C. Apparent argatroban resistance in a patient with elevated factor VIII levels. Ann Pharmacother. 2013;47(7-8):e29.
74. Park KM, Moon IS, Kim JI, et al. Mechanical thrombectomy with Trerotola compared with catheter-directed thrombolysis for treatment of acute iliofemoral deep vein thrombosis. Ann Vasc Surg. 2014;28(8):1853-1861.
75. Bozkaya H, Cinar C, Ertugay S, et al. Endovascular treatment of iliac vein compression (May-Thurner) syndrome: angioplasty and stenting with or without manual aspiration thrombectomy and catheter-directed thrombolysis. Ann Vasc Dis. 2015;8(1):21-28.
76. Andreoli JM, Lewandowski RJ, Vogelzang RL, Ryu RK. Comparison of complication rates associated with permanent and retrievable inferior vena cava filters: a review of the MAUDE database. J Vasc Interv Radiol. 2014;25(8):1181-1185.
77. Ishibashi H, Takashi O, Hosaka M, et al. Heparin-induced thrombocytopenia complicated with massive thrombosis of the inferior vena cava after filter placement. Int Angiol. 2005;24(4):387-390.
78. Martinez LA, Burnett AE. Catheter-directed thrombolysis with alteplase and bivalirudin in a patient with heparin-induced thrombocytopenia. Am J Health Syst Pharm. 2015;72(9):707-710.
79. Cervera R, Rodríguez-Pintó I, Colafrancesco S, et al. 14th International Congress on Antiphospholipid Antibodies Task Force Report on Catastrophic Antiphospholipid Syndrome. Autoimmun Rev. 2014;13(7):699-707.
80. Berman H, Rodríguez-Pintó I, Cervera R, et al; Catastrophic Antiphospholipid Syndrome (CAPS) Registry Project Group (European Forum on Antiphospholipid Antibodies). Rituximab use in the catastrophic antiphospholipid syndrome: descriptive analysis of the CAPS registry patients receiving rituximab. Autoimmun Rev. 2013;12(11):1085-1090.
81. Saunders KH, Erkan D, Lockshin MD. Perioperative management of antiphospholipid antibody-positive patients. Curr Rheumatol Rep. 2014;16(7):426.
82. Gómez-Puerta JA, Espinosa G, Cervera R. Catastrophic antiphospholipid syndrome: diagnosis and management in pregnancy. Clin Lab Med. 2013;33(2):391-400.
83. Hanouna G, Morel N, Le Thi Huong D, et al. Catastrophic antiphospholipid syndrome and pregnancy: an experience of 13 cases. Rheumatology (Oxford). 2013;52(9):1635-1641.
84. Aryal MR, Badal M, Bhandari N, Bhatt VR. Accelerated arterial and venous clots in a young pregnant woman: a saga of thrombotic storm [published online ahead of print May 27, 2013]. BMJ Case Rep.
85. Tanimura K, Ebina Y, Sonoyama A, Morita H, Miyata S, Yamada H. Argatroban therapy for heparin-induced thrombocytopenia during pregnancy in a woman with hereditary antithrombin deficiency. J Obstet Gynaecol Res. 2012;38(4):749-752.
86. Nagler M, Haslauer M, Wuillemin WA. Fondaparinux - data on efficacy and safety in special situations. Thromb Res. 2012;129(4):407-417.