#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Overlap of the symptoms of thrombotic thrombocytopenic purpura and antiphospholipid syndrome in a patient with juvenile systemic lupus erythematosus – case report


Authors: M. Bělobrádková 1;  M. Cermanová 2;  L. Minxová 3;  Z. Bělobrádek 4;  Z. Vavera 5;  M. Tošovský 1;  T. Soukup 1
Authors place of work: Lékařská fakulta Univerzity Karlovy a Fakultní nemocnice v Hradci Králové, Česká republika ;  II. interní gastroenterologická klinika 1;  IV. interní hematologická klinika 2;  Dětská klinika 3;  Radiologická klinika 4;  I. kardioangiologická klinika 5
Published in the journal: Čes. Revmatol., 25, 2017, No. 4, p. 190-198.
Category: Case Report

Summary

Thrombocytopenia accompanied by thrombosis is often observed in patients with systemic lupus erythematosus. The underlying process may be associated with more frequent secondary antiphospholipid syndrome characterized by the presence of antiphospholipid antibodies and thromboses, or with associated thrombotic thrombocytopenic purpura linked with insufficient ADAMTS13 protease activity (A Disintegrin-like And Metalloproteinase with Thrombospondin Type 1 motif 13). In our article we demonstrate a case of a fourteen-year-old girl without a serious pre-existing medical history, who was hospitalized for a stroke accompanied by severe thrombocytopenia and microangiopathic haemolytic anemia. The condition was evaluated as a thrombotic thrombocytopenic purpura, and the patient was treated with plasma exchange therapy (plasmapheresis), glucocorticoids, and then anticoagulation and antiaggregation therapy. Within 6 weeks of onset of the disease, thrombocytopenia has relapsed after tapering of the glucocorticoid dose. Additional plasmaphereses were initiated and rituximab was successfully administered at the same time. Based on further developments in the disease, imaging and laboratory tests, it is possible to admit that it was most likely a coincidence of thrombotic thrombocytopenic purpura and antiphospholipid syndrome. The clinical picture was later completed by a manifestation of systemic lupus erythematosus. The pathogenesis and the current prevalence of these three diseases are discussed.

Key words:
Systemic lupus erythematosus, thrombotic microangiopathy, thrombotic thrombocytopenic purpura, antiphospholipid syndrome


Zdroje

1. Thakral A, Klein-Gitelman MS. An Update on Treatment and Management of Pediatric Systemic Lupus Erythematosus. Rheumatology and Therapy 2016; 3(2): 209–219.

2. Fonseca R, Aguiar F, Rodrigues M, Brito I. Clinical phenotype and outcome in lupus according to age: a comparison between juvenile and adult onset. Reumatologia clinica, 2016.

3. Velo-Garcia A, Castro SG, Isenberg DA. The diagnosis and management of the haematologic manifestations of lupus. Journal of Autoimmunity 2016; 74: 139–160.

4. Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R, Reber G. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). Journal of Thrombosis and Haemostasis 2006; 4(2), 295–306.

5. Negrini S, Pappalardo F, Murdaca G, Indiveri F, Puppo F. The antiphospholipid syndrome: from pathophysiology to treatment. Clinical and Experimental Medicine 2016; 1-11.

6. Asherson RA, Cervera R, De Groot PG, Erkan D, Boffa MC, Piette JC. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12(7): 530–534.

7. Nayer A, Ortega LM. Journal of Nephropathology. Journal of nephropathology 2014; 3(1): 9.

8. Fayyaz A, Igoe A, Kurien BT, Danda D, James JA, Stafford HA, Scofield RH. Haematological manifestations of lupus. Lupus science & medicine 2015; 2(1): e000078.

9. Scully M, Hunt BJ, Benjamin S, Liesner R, Rose P, Peyvandi F, Machin SJ. Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. British journal of haematology 2012; 158(3): 323–335.

10. Moore JC, Hayward CP, Warkentin TE, Kelton JG. Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood 2001; 98(6): 1842–1846.

11. Bianchi V, Robles R, Alberio L, Furlan M, Lämmle B. Von Willebrand factor–cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura. Blood 2002; 100(2): 710-713.

12. Noris M, Remuzzi G. Thrombotic thrombocytopenic purpura-then and now. In: Seminars in thrombosis and hemostasis. Copyright© 2006 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA; 2006. p. 081–089.

13. Asherson RA, Espinosa G, Cervera R, Font J, Reverter JC. Catastrophic antiphospholipid syndrome: proposed guidelines for diagnosis and treatment. JCR: Journal of Clinical Rheumatology 2002; 8(3): 157–165.

14. Aby ES, Rosolo Z, Simegn MA. Mitral valve perforation in Libman–Sacks endocarditis: a heart-wrenching case of lupus. Journal of general internal medicine 2016; 31(8): 964–969.

15. Khan RM, Namas R, Parikh S, Rubin B. Embolic stroke as the initial manifestation of systemic lupus erythematosus. Case reports in rheumatology 2015.

16. Paran D, Caspi D, Levartovsky D, Elkayam O, Kaufman I, Litinsky I, Koifman B. Cardiac dysfunction in patients with systemic lupus erythematosus and antiphospholipid syndrome. Annals of the rheumatic diseases 2007; 66(4): 506–510.

17. Hojnik M, George J, Ziporen L, Shoenfeld Y. Heart valve involvement (Libman-Sacks endocarditis) in the antiphospholipid syndrome. Circulation 1996; 93(8): 1579–1587.

18. Tan EM, Cohen AS, Fries JF, Masi AT, Mcshane DJ, Rothfield NF, Winchester RJ. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis & Rheumatology 1982; 25(11): 1271–1277.

19. Petri M, Orbai AM, Alarcon GS, Gordon C, Merrill JT, Fortin PR, Sturfelt G. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria for systemic lupus erythematosus. Arthritis & Rheumatology 2012; 64(8): 2677–2686.

20. Muscal E, Edwards RM, Kearney DL, Hicks JM, Myones BL, Teruya J. Thrombotic microangiopathic hemolytic anemia with reduction of ADAMTS13 activity. American journal of clinical pathology 2011; 135(3): 406-416.

21. Amoura Z, Costedoat–Chalumeau N, Veyradier A, Wolf M, Ghillani–Dalbin P, Cacoub P, Piette JC. Thrombotic thrombocytopenic purpura with severe ADAMTS13 deficiency in two patients with primary antiphospholipid syndrome. Arthritis & Rheumatism 2004; 50(10): 3260–3264.

22. Brunner HI, Freedman M, Silverman ED. Close relationship between systemic lupus erythematosus and thrombotic thrombocytopenic purpura in childhood. Arthritis & Rheumatism 1999; 42(11): 2346–2355.

23. Mannucci PM, Vanoli M, Forza I, Canciani MT, Scorza R. Von Willebrand factor cleaving protease (ADAMTS13) in 123 patients with connective tissue diseases (systemic lupus erythematosus and systemic sclerosis). Haematologica 2003; 88(8): 914–918.

24. Lee SJ, Kim JE. Han KS, Kim HK. Thrombotic risk of reduced ADAMTS13 activity in patients with antiphospholipid antibodies. Blood Coagulation & Fibrinolysis 2016; 27(8): 907–912.

25. Martin-Rodriguez S, Reverter JC, Tàssies D, Espinosa G, Heras M, Pino M, Diaz-Ricart M. Reduced ADAMTS13 activity is associated with thrombotic risk in systemic lupus erythematosus. Lupus 2015; 24(11): 1143–1149.

26. Yamazaki S, Taki M, Yasumuro Y, Takayama S, Miyake F, Fujimura Y, Suzuki Y. Von Willebrand factor-cleaving protease activity in patients of collagen disease with antiphospholipid antibodies. Rinsho byori. The Japanese journal of clinical pathology 2002; 50(3): 301–307.

27. Espinosa G, Bucciarelli S, Cervera R, Lozano M, Reverter JC, De la Red G, Asherson RA. Thrombotic microangiopathic haemolytic anaemia and antiphospholipid antibodies. Annals of the rheumatic diseases 2004; 63(6): 730–736.

28. Rodriguez-Pinto I, Espinosa G, Cervera R. Catastrophic APS in the context of other thrombotic microangiopathies. Current rheumatology reports 2015; 17(1): 1–10.

29. Campistol JM, Arias M, Ariceta G, Blasco M, Espinosa M, Grinyó JM, Rodriguez de Cordoba S. An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. A consensus document. Nefrologia 2013; 33(1): 27–45.

30. Bu F, Maga T, Meyer NC, Wang K, Thomas CP, Nester CM, Smith RJ. Comprehensive genetic analysis of complement and coagulation genes in atypical hemolytic uremic syndrome. Journal of the American Society of Nephrology 2014; 25(1): 55–64.

31. Groot N, de Graeff N, Aviin T, et al. European evidence-based recommendations for diagnosis and treatment of pediatric antiphospholipid syndrome: the SHARE initiative. Ann Rheum Dis Published Online 4.5.2016.

Štítky
Dermatology & STDs Paediatric rheumatology Physiotherapist, university degree Internal medicine Cardiology Orthopaedics Rheumatology
Prihlásenie
Zabudnuté heslo

Zadajte e-mailovú adresu, s ktorou ste vytvárali účet. Budú Vám na ňu zasielané informácie k nastaveniu nového hesla.

Prihlásenie

Nemáte účet?  Registrujte sa

#ADS_BOTTOM_SCRIPTS#