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The Significance and Management of Thrombocytopenia in Antiphospholipid Syndrome

  • Antiphospholipid Syndrome (D Erkan, Section Editor)
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Abstract

The association between antiphospholipid antibodies (aPL) and clinical problems goes beyond what is stated in the antiphospholipid syndrome (APS) classification criteria, namely thrombosis and pregnancy morbidity, and thrombocytopenia is the most common non-criteria hematologic manifestation of aPL with a frequency ranging from 20 to 50 %. Thrombocytopenia is rarely severe, and hemorrhage is far less common than thrombosis. However, when anticoagulation is considered, it may constitute a clinical problem with increased bleeding risk. Furthermore, thrombocytopenia represents a risk factor for thrombosis in aPL-positive patients. Therefore, it is important to understand the pathogenesis and the clinical associations of thrombocytopenia to build the right medical approach in aPL-positive patients. In this paper, we review the literature on aPL/APS-associated thrombocytopenia and briefly discuss the other conditions that can result in thrombocytopenia as they have commonalities with APS and their recognition is important to establish the most appropriate treatment strategy.

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References

  1. Petri M. Epidemiology of the antiphospholipid antibody syndrome. J Autoimmun. 2000;15(2):145–51.

    Article  CAS  PubMed  Google Scholar 

  2. Rai RS. Antiphospholipid syndrome and recurrent miscarriage. J Postgrad Med. 2002;48(1):3–4.

    CAS  PubMed  Google Scholar 

  3. Wilson WA, Gharavi AE, Koike T, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum. 1999;42(7):1309–11.

    Article  CAS  PubMed  Google Scholar 

  4. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295–306.

    Article  CAS  PubMed  Google Scholar 

  5. Cervera R, Piette JC, Font J, et al. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum. 2002;46(4):1019–27.

    Article  PubMed  Google Scholar 

  6. Cervera R, Tektonidou MG, Espinosa G, et al. Task Force on Catastrophic Antiphospholipid Syndrome (APS) and Non-criteria APS Manifestations (II): thrombocytopenia and skin manifestations. Lupus. 2011;20(2):174–81.

    Article  CAS  PubMed  Google Scholar 

  7. Alarcón-Segovia D, Pérez-Vázquez ME, Villa AR, et al. Preliminary classification criteria for the antiphospholipid syndrome within systemic lupus erythematosus. Semin Arthritis Rheum. 1992;21(5):275–86.

    Article  PubMed  Google Scholar 

  8. Morrell CN, Aggrey AA, Chapman LM, et al. Emerging roles for platelets as immune and inflammatory cells. Blood. 2014;123(18):2579–767.

    Article  Google Scholar 

  9. Stasi R, Amadori S, Osborn J, et al. Long-term outcome of otherwise healthy individuals with incidentally discovered borderline thrombocytopenia. PLoS Med. 2006;3(3):e24.

    Article  PubMed Central  PubMed  Google Scholar 

  10. Bain BJ. Ethnic and sex differences in the total and differential white cell count and platelet count. J Clin Pathol. 1996;49(8):664–6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  11. Cuadrado MJ, Mujic F, Munoz E, et al. Thrombocytopenia in the antiphospholipid syndrome. Ann Rheum Dis. 1997;56(3):194–6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  12. Petri M, Orbai AM, Alarcon GS, et al. Derivation and validation of the systemic lupus international collaborating clinics classification criteria for systemic lupus erythematosus. Arthritis Rheum. 2012;64(8):2677–86.

    Article  PubMed Central  PubMed  Google Scholar 

  13. Krause I, Blank M, Fraser A, et al. The association of thrombocytopenia with systemic manifestations in the antiphospholipid syndrome. Immunobiology. 2005;210:749–54.

    Article  CAS  PubMed  Google Scholar 

  14. Galli M, Finazzi G, Barbui T. Thrombocytopenia in the antiphospholipid syndrome. Br J Haematol. 1996;93:1–5.

    Article  CAS  PubMed  Google Scholar 

  15. Italian Registry of Antiphospholipid Antibodies. Thrombosis and thrombocytopenia in antiphospholipid syndrome (idiopathic and secondary to SLE): first report from Italian Registry. Haematologica. 1993;78:313–8.

    Google Scholar 

  16. Vianna JL, Khamashta MA, Ordi Ros J, et al. Comparison of the primary and secondary antiphospholipid syndrome: a European multicenter study of 114 patients. Am J Med. 1994;96:3–9.

    Article  CAS  PubMed  Google Scholar 

  17. McClain MT, Arbuckle MR, Heinlen LD, et al. The prevalence, onset, and clinical significance of antiphospholipid antibodies prior to diagnosis of systemic lupus erythematosus. Arthritis Rheum. 2004;50(4):1226–32.

    Article  PubMed  Google Scholar 

  18. Gómez-Puerta J, Martin H, Amigo MC, et al. Long-term follow-up in 128 patients with primary antiphospholipid syndrome. Medicine. 2005;84(4):225–30.

    Article  PubMed  Google Scholar 

  19. Rodeghiero F, Stasi R, Gernsheimer T, et al. Standardization of terminology, definitions and outcome criteria in immune thrombocytopenic purpura of adults and children: report from an international working group. Blood. 2009;113(11):2386–93.

    Article  CAS  PubMed  Google Scholar 

  20. Asherson RA. New subsets of the antiphospholipid syndrome in 2006: ‘PRE-APS’ (probable APS) and microangiopathic antiphospholipid syndromes (‘MAPS’). Autoimmun Rev. 2006;6(2):76–80.

    Article  PubMed  Google Scholar 

  21. Artim-Esen B, Çene E, Şahinkaya Y, et al. Cluster analysis of autoantibodies in 852 patients with systemic lupus erythematosus from a single center. J Rheumatol. 2014;41(7):1304–10.

    Article  PubMed  Google Scholar 

  22. Espinola RG, Pierangeli SS, Gharavi AE, et al. Hydroxychloroquine reverses platelet activation induced by human IgG antiphospholipid antibodies. Thromb Haemost. 2002;87(3):518–22.

    CAS  PubMed  Google Scholar 

  23. Campbell AL, Pierangeli SS, Wellhausen S, et al. Comparison of the effects of anticardiolipin antibodies from patients with the antiphospholipid syndrome and with syphilis on platelet activation and aggregation. Thromb Haemost. 1995;73(3):529–34.

    CAS  PubMed  Google Scholar 

  24. Martinuzzo ME, Maclouf J, Carreras LO, et al. Antiphospholipid antibodies enhance thrombin-induced platelet activation and thromboxane formation. Thromb Haemostasis. 1993;70(4):667–71.

    CAS  Google Scholar 

  25. Lutters BC, Meijers JC, Derksen RH, et al. Dimers of beta 2-glycoprotein I mimic the in vitro effects of beta 2-glycoprotein I-anti-beta 2-glycoprotein I antibody complexes. J Biol Chem. 2001;276(5):3060–7.

    Article  CAS  PubMed  Google Scholar 

  26. Proulle V, Furie RA, Merrill-Skoloff G, et al. Platelets are required for enhanced activation of endothelium and fibrinogen in a mouse thrombosis model of APS. Blood. 2014;124(4):611–22.

    Article  CAS  PubMed  Google Scholar 

  27. Lutters BC, Derksen RH, Tekelenburg WL, et al. Dimers of beta 2-glycoprotein I increase platelet deposition to collagen via interaction with phospholipids and the apolipoprotein E receptor 2′. J Biol Chem. 2003;278(36):33831–8.

    Article  CAS  PubMed  Google Scholar 

  28. Pennings MT, van Lummel M, Derksen RH, et al. Interaction of beta2-glycoprotein I with members of the low density lipoprotein receptor family. J Thromb Haemost. 2006;4(8):1680–90.

    Article  CAS  PubMed  Google Scholar 

  29. Shi T, Giannakopoulos B, Yan X, et al. Anti-beta2-glycoprotein I antibodies in complex with beta2-glycoprotein I can activate platelets in a dysregulated manner via glycoprotein Ib-IX-V. Arthritis Rheum. 2006;54(8):2558–67.

    Article  CAS  PubMed  Google Scholar 

  30. Pennings MT, Derksen RH, van Lummel M, et al. Platelet adhesion to dimeric beta-glycoprotein I under conditions of flow is mediated by at least two receptors: glycoprotein Ibalpha and apolipoprotein E receptor 2′. J Thromb Haemost. 2007;5(2):369–77.

    Article  CAS  PubMed  Google Scholar 

  31. Herz J, Strickland DK. LRP: a multifunctional scavenger and signaling receptor. J Clin Invest. 2001;108(6):779–84.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  32. Laman JD, Claassen E, Noelle RJ. Functions of CD40 and its ligand, gp39 (CD40L). Crit Rev Immunol. 1996;16(1):59–108.

    Article  CAS  PubMed  Google Scholar 

  33. Nakamura M, Tanaka Y, Satoh T, et al. Autoantibody to CD40 ligand in systemic lupus erythematosus: association with thrombocytopenia but not thromboembolism. Rheumatology (Oxford). 2006;45(2):150–6.

    Article  CAS  Google Scholar 

  34. Vlachoyiannopoulos PG, Mavragani CP, Bourazopoulou E, et al. Anti-CD40 antibodies in antiphospholipid syndrome and systemic lupus erythematosus. Thromb Haemost. 2004;92(6):1303–11.

    CAS  PubMed  Google Scholar 

  35. Galli M, Daldossi M, Barbui T. Anti-glycoprotein Ib/IX and IIb/IIIa antibodies in patients with antiphospholipid antibodies. Thromb Haemost. 1994;71(5):571–5.

    CAS  PubMed  Google Scholar 

  36. Godeau B, Piette JC, Fromont P, et al. Specific antiplatelet glycoprotein autoantibodies are associated with the thrombocytopenia of primary antiphospholipid syndrome. Br J Haematol. 1997;98(4):873–9.

    Article  CAS  PubMed  Google Scholar 

  37. Macchi L, Rispal P, Clofent-Sanchez G, et al. Anti-platelet antibodies in patients with systemic lupus erythematosus and the primary antiphospholipid antibody syndrome: their relationship with the observed thrombocytopenia. Br J Haematol. 1997;98(2):336–41.

    Article  CAS  PubMed  Google Scholar 

  38. Panzer S, Gschwandtner ME, Hutter D, et al. Specificities of platelet autoantibodies in patients with lupus anticoagulants in primary antiphospholipid syndrome. Ann Hematol. 1997;74(5):239–42.

    Article  CAS  PubMed  Google Scholar 

  39. Fabris F, Steffan A, Cordiano I, et al. Specific antiplatelet autoantibodies in patients with antiphospholipid antibodies and thrombocytopenia. Eur J Haematol. 1994;53(4):232–6.

    Article  CAS  PubMed  Google Scholar 

  40. Stasi R, Stipa E, Masi M, et al. Prevalence and clinical significance of elevated antiphospholipid antibodies in patients with idiopatic thrombocytopenic purpura. Blood. 1994;84(12):4203–8.

    CAS  PubMed  Google Scholar 

  41. Provan D, Stasi R, Newland AC, et al. International consensus report on the investigation and management of primary immune thrombocytopenia. Blood. 2010;115:168–86.

    Article  CAS  PubMed  Google Scholar 

  42. Neunert C, Lim W, Crowther M, et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011;117:4190–207.

    Article  CAS  PubMed  Google Scholar 

  43. Diz-Küçükkaya R, Junmei C, Amy G. Thrombocytopenia, Chapter 119. In: Lichtman MA, Beutler E, Kipps TJ, Selighson U, Kaushansky K, Prchol JT, editors. Williams hematology, eight edition. USA: Mc Graw Hill Company; 2010.

    Google Scholar 

  44. Verschoor A, Langer HF. Crosstalk between platelets and the complement system in immune protection and disease. Thromb Haemost. 2013;110(5):910–9.

    Article  CAS  PubMed  Google Scholar 

  45. McKenzie CG, Guo L, Freedman J. Cellular immune dysfunction in immune thrombocytopenia (ITP). Br J Haematol. 2013;163(1):10–23.

    Article  CAS  PubMed  Google Scholar 

  46. Kuter DJ. Milestones in understanding platelet production: a historical overview. Br J Haematol. 2014;165(2):248–58.

    Article  CAS  PubMed  Google Scholar 

  47. Pierrot-Deseilligny C, Michel M, Khellaf M, et al. Antiphospholipid antibodies in adults with immune thrombocytopenic purpura. Br J Haematol. 2008;142(4):638–43.

    Article  Google Scholar 

  48. Diz-Kucukkaya R, Hacihanefioglu A, Yenerel M, et al. Antiphospholipid antibodies and antiphospholipid syndrome in patients presenting with immune thrombocytopenic purpura: a prospective cohort study. Blood. 2001;98(6):1760–4.

    Article  CAS  PubMed  Google Scholar 

  49. Funauchi M, Hamada K, Enomoto H, et al. Characteristics of the clinical findings in patients with idiopathic thrombocytopenic purpura who are positive for antiphospholipid antibodies. Intern Med. 1997;36(12):882–5.

    Article  CAS  PubMed  Google Scholar 

  50. Bidot CJ, Jy W, Horstman LL, et al. Antiphospholipid antibodies in immune thrombocytopenic purpura tend to emerge in exacerbation and decline in remission. Br J Haematol. 2005;128(3):366–72.

    Article  CAS  PubMed  Google Scholar 

  51. George JN, Nester CM. Syndromes of thrombotic microangiopathy. N Engl J Med. 2014;371(19):1847–8.

    PubMed  Google Scholar 

  52. Asherson RA, Cervera R. Microvascular and microangiopathic antiphospholipid-associated syndromes (‘MAPS’): semantic or antisemantic? Autoimmun Rev. 2008;7:164–67.

    Article  CAS  PubMed  Google Scholar 

  53. Asherson RA, Pierangeli SS, Cervera R. Is there a microangiopathic antiphospholipid syndrome? Ann Rheum Dis. 2007;66(4):429–32.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  54. Blombery P, Scully M. Management of thrombotic thrombocytopenic purpura: current perspectives. J Blood Med. 2014;5:15–23.

    PubMed Central  PubMed  Google Scholar 

  55. Jain R, Chartash E, Susin M, et al. Systemic lupus erythematosus complicated by thrombotic microangiopathy. Semin Arthritis Rheum. 1994;24:173–82.

    Article  CAS  PubMed  Google Scholar 

  56. Durand JM, Lefevre P, Kaplanski G, et al. Thrombotic microangiopathy and the antiphospholipid antibody syndrome. J Rheumatol. 1991;18:1916–8.

    CAS  PubMed  Google Scholar 

  57. Amoura Z, Costedoat-Chalumeau N, Veyradier A, et al. Thrombotic thrombocytopenic purpura with severe ADAMTS-13 deficiency in two patients with primary antiphospholipid syndrome. Arthritis Rheum. 2004;50(10):3260–4.

    Article  PubMed  Google Scholar 

  58. Austen SK, Starke RD, Lawrie AS, et al. The VWF/ADAMTS13 axis in the antiphospholipid syndrome: ADAMTS13 antibodies and ADAMTS13 dysfunction. Br J Haematol. 2008;141(4):536–44.

    Article  Google Scholar 

  59. Hancer VS, Diz Kucukkaya R, Topal-Sarıkaya A. ADAMTS-13 gene expression in antiphospholipid syndrome. Turk J Hematol. 2011;28:213–8.

    Article  CAS  Google Scholar 

  60. Ardiles LG, Olavarria F, Elgueta M, et al. Anticardiolipin antibodies in classic pediatric hemolytic-uremic syndrome: a possible pathogenic role. Nephron. 1998;78(3):278–83.

    Article  CAS  PubMed  Google Scholar 

  61. Goldman-Wohl D, Yagel S. Regulation of trophoblast invasion: from normal implantation to pre-eclampsia. Mol Cell Endocrinol. 2002;187(1–2):233–8.

    Article  CAS  PubMed  Google Scholar 

  62. McCrae KR. Thrombocytopenia in pregnancy: differential diagnosis, pathogenesis, and management. Blood Rev. 2003;17:7–14.

    Article  PubMed  Google Scholar 

  63. Kirkpatrick CA. The HELLP syndrome. Acta Clin Belg. 2010;65(2):91–7.

    Article  CAS  PubMed  Google Scholar 

  64. Thi Thuong DL, Tieuliè N, Costedoat N, et al. The HELLP syndrome in the antiphospholipid syndrome: retrospective study of 16 cases in 15 women. Ann Rheum Dis. 2005;64:273–8.

    Article  PubMed Central  PubMed  Google Scholar 

  65. Tsirigotis P, Mantzios G, Pappa V, et al. Antiphospholipid syndrome: a predisposing factor for early onset HELLP syndrome. Rheumatol Int. 2007;28(2):171–4.

    Article  PubMed  Google Scholar 

  66. Veres K, Papp K, Lakos G, et al. Association of HELLP syndrome with primary antiphospholipid syndrome—a case report. Clin Rheumatol. 2008;27(1):111–3.

    Article  PubMed  Google Scholar 

  67. Abildgaard U, Heimdal K. Pathogenesis of the syndrome of hemolysis, elevated liver enzymes, and low platelet count (HELLP): a review. Eur J Obstet Gynecol Reprod Biol. 2013;166(2):117–23.

    Article  CAS  PubMed  Google Scholar 

  68. Hulstein JJ, van Runnard Heimel PJ, Franx A, et al. Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. J Thromb Haemost. 2006;4(12):2569–75.

    Article  CAS  PubMed  Google Scholar 

  69. Hoppensteadt DA, Walenga JM. The relationship between the antiphospholipid syndrome and heparin-induced thrombocytopenia. Hematol Oncol Clin N Am. 2008;22:1–18.

    Article  Google Scholar 

  70. Warkentin TE. How I diagnose and manage HIT. Hematol Am Soc Hematol Educ Prog. 2011;2011:143–9.

    Article  Google Scholar 

  71. McKenzie SE, Sachais BS. Advances in the pathophysiology and treatment of heparin-induced thrombocytopenia. Cur Opin Hematol. 2014;21(5):380–7.

    Article  CAS  Google Scholar 

  72. Marques MB. Thrombotic thrombocytopenic purpura and heparin-induced thrombocytopenia: two unique causes of life-threatening thrombocytopenia. Clin Lab Med. 2009;29:321–38.

    Article  PubMed  Google Scholar 

  73. Greinacher A. Heparin-induced thrombocytopenia. J Thromb Haemost. 2009;7:9–12.

    Article  CAS  PubMed  Google Scholar 

  74. Kelton JG. The pathophysiology of heparin-induced thrombocytopenia. Chest. 2005;127(2):9S–20S.

    Article  PubMed  Google Scholar 

  75. Shibata S, Harpel PC, Gharavi A, et al. Autoantibodies to heparin from patients with antiphospholipid antibody syndrome inhibit formation of antithrombin III-thrombin complexes. Blood. 1994;83(9):2532–40.

    CAS  PubMed  Google Scholar 

  76. Alpert D, Mandl LA, Erkan D, et al. Anti-heparin platelet factor 4 antibodies in systemic lupus erythematosus are associated with IgM antiphospholipid antibodies and the antiphospholipid syndrome. Ann Rheum Dis. 2008;67(3):395–401.

    Article  CAS  PubMed  Google Scholar 

  77. Ikeda K, Nawata M, Ando S, Koike M, Sekigawa I, Iida N. Antiphospholipid antibody-associated haemophagocytic syndrome. Rheumatology (Oxford). 2000;39(5):564–5.

    Article  CAS  Google Scholar 

  78. Resende LS, Mendes RP, Bacchi MM, Marques SA, Barraviera B, Souza LR, et al. Bone marrow necrosis related to paracoccidioidomycosis: the first eight cases identified at autopsy. Histopathology. 2009;54(4):486–9.

    Article  PubMed Central  PubMed  Google Scholar 

  79. Paydas S, Kocak R, Zorludemir S, Baslamisli F. Bone marrow necrosis in antiphospholipid syndrome. J Clin Pathol. 1997;50(3):261–2.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  80. Bulvik S, Aronson I, Ress S, Jacobs P. Extensive bone marrow necrosis associated with antiphospholipid antibodies. Am J Med. 1995;98(6):572–4.

    Article  CAS  PubMed  Google Scholar 

  81. Moore J, Ma DD, Concannon A. Non-malignant bone marrow necrosis: a report of two cases. Pathology. 1998;30(3):318–20.

    Article  CAS  PubMed  Google Scholar 

  82. Cikrikcioglu MA, Hursitoglu M, Erkal H, Inan B, Ozturk T, Cakirca M, et al. Splenomegaly in primary antiphospholipid syndrome without accompanying portal hypertension or comorbidity. Pathophysiol Haemost Thromb. 2010;37(2–4):104–9.

    PubMed  Google Scholar 

  83. Bizzaro N, Brandalise M. EDTA-dependent pseudothrombocytopenia. Association with antiplatelet and antiphospholipid antibodies. Am J Clin Pathol. 1995;103(1):103–7.

    CAS  PubMed  Google Scholar 

  84. Comellas-Kirkerup L, Hernandez-Molina G, Cabral AR. Antiphospholipid-associated thrombocytopenia or autoimmune hemolytic anemia in patients with or without definite primary antiphospholipid syndrome according to the Sapporo revised classification criteria: a 6-year follow-up study. Blood. 2010;116(16):3058–63.

    Article  CAS  PubMed  Google Scholar 

  85. Khattri S, Zandman-Goddard G, Peeva E. B-cell directed therapies in antiphospholipid antibody syndrome—new directions based on murine and human data. Autoimmun Rev. 2012;11(10):717–22.

    Article  CAS  PubMed  Google Scholar 

  86. Kumar D, Roubey RA. Use of rituximab in the antiphospholipid syndrome. Curr Rheumatol Rep. 2010;12(1):40–4.

    Article  CAS  PubMed  Google Scholar 

  87. Godeau B. B-cell depletion in immune thrombocytopenia. Semin Hematol. 2013;50 suppl 1:S75–82.

    Article  CAS  PubMed  Google Scholar 

  88. Mahevas M, Patin P, Huetz F, et al. B cell deletion in immune thrombocytopenia reveals splenic long-lived plasma cells. J Clin Invest. 2013;123(1):432–42.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  89. Ramos-Casals M, Garcia-Hernandez FJ, de Ramon E, et al. Off-label use of rituximab in 196 patients with severe, refractory systemic autoimmune diseases. Clin Exp Rheumatol. 2010;28(4):468–76.

    CAS  PubMed  Google Scholar 

  90. Erkan D, Vega J, Ramon G, et al. A pilot open-label phase II trial of rituximab for non-criteria manifestations of antiphospholipid syndrome. Arthritis Rheum. 2013;65(2):464–71.

    Article  CAS  PubMed  Google Scholar 

  91. Erkan D, Aguiar CL, Andrade D, et al. International Congress on Antiphospholipid Antibodies: task force report on antiphospholipid syndrome treatment trends. Autoimmun Rev. 2014;13(6):685–96.

    Article  CAS  PubMed  Google Scholar 

  92. Berman H, Rodriguez-Pinto I, Cervera R, et al. Rituximab use in the catastrophic antiphospholipid syndrome: descriptive analysis of the CAPS registry patients receiving rituximab. Autoimmun Rev. 2013;12(11):1085–90.

    Article  CAS  PubMed  Google Scholar 

  93. Galindo M, Khamashta MA, Hughes GR. Splenectomy for refractory thrombocytopenia in the antiphospholipid syndrome. Rheumatology (Oxford). 1999;38(9):848–53.

    Article  CAS  Google Scholar 

  94. Hakim AJ, Machin SJ, Isenberg DA. Autoimmune thrombocytopenia in primary antiphospholipid syndrome and systemic lupus erythematosus: the response to splenectomy. Semin Arthritis Rheum. 1998;28(1):20–5.

    Article  CAS  PubMed  Google Scholar 

  95. Delgado Alves J, Inanc M, Diz-Kucukkaya R, et al. Thrombotic risk in patients submitted to splenectomy for systemic lupus erythematosus and antiphospholipid antibody syndrome-related thrombocytopenia. Eur J intern Med. 2004;15(3):162–7.

    Article  PubMed  Google Scholar 

  96. Cervera R. Catastrophic antiphospholipid syndrome (CAPS): update from the ‘CAPS Registry’. Lupus. 2010;19(4):412–8.

    Article  CAS  PubMed  Google Scholar 

  97. Sperati CJ, Streiff MB. Acute renal failure in a patient with antiphospholipid syndrome and immune thrombocytopenic purpura treated with eltrombopag. Am J Hematol. 2010;85(9):724–6.

    Article  PubMed  Google Scholar 

  98. Jansen AJ, Swart RM, te Boekhorst PA. Thrombopoietin receptor agonists for immune thrombocytopenia. N Eng J Med. 2011;365(23):2240–1.

    Article  Google Scholar 

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Bahar Artim-Esen, Reyhan Diz-Küçükkaya, Murat İnanç declare that they have no conflict of interest.

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Artim-Esen, B., Diz-Küçükkaya, R. & İnanç, M. The Significance and Management of Thrombocytopenia in Antiphospholipid Syndrome. Curr Rheumatol Rep 17, 14 (2015). https://doi.org/10.1007/s11926-014-0494-8

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