Management of patient with immune thrombocytopenia with antiphospholipid syndrome and monoclonal gammopathy of undetermined significance Case report

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Wiktoria Ryżewska
Malwina Zarzycka
Michał Witkowski
Magdalena Witkowska
Tadeusz Robak


Immune thrombocytopenia with antiphospholipid syndrome and monoclonal gammopathy of undetermined significance poses therapeutic dilemmas – whether we should modify the immune thrombocytopenia treatment in antiphospholipid syndrome, what is the influence of monoclonal gammopathy of undetermined significance on the course of immune thrombocytopenia and whether we should and how to prevent the progression of monoclonal gammopathy of undetermined significance to multiple myeloma.


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Ryżewska W, Zarzycka M, Witkowski M, Witkowska M, Robak T. Management of patient with immune thrombocytopenia with antiphospholipid syndrome and monoclonal gammopathy of undetermined significance. OncoReview [Internet]. 2021Nov.8 [cited 2022Aug.16];11(4 (44):106-11. Available from:


1. Neunert C, Lim W, Crowther M et al. The American Society of Hematology 2011 evidence-based practice guideline for immune thrombocytopenia. Blood. 2011; 117(16): 4190-207.
2. Liebman HA. Recognizing and treating secondary immune thrombocytopenic purpura associated with lymphoproliferative disorders. Semin Hematol. 2009; 46: S33-S6.
3. Kim KJ, Baek IW, Yoon CH et al. Thrombotic risk in patients with immune thrombocytopenia and its association with antiphospholipid antibodies. Br J Haematol. 2013; 161(5): 706-14.
4. Chayoua W, Kelchtermans H, Moore GW et al. Identification of high thrombotic risk triple-positive antiphospholipid syndrome patients is dependent on anti-cardiolipin and anti-β2glycoprotein I antibody detection assays. J Thromb Haemost. 2018; 16(10): 2016-23.
5. Vreede AP, Bockenstedt PL, McCune WJ et al. Cryptic conspirators: a conversation about thrombocytopenia and antiphospholipid syndrome. Curr Opin Rheumatol. 2019; 31(3): 231-40.
6. Witkowski M, Witkowska M, Robak T. Autoimmune thrombocytopenia: Current treatment options in adults with a focus on novel drugs. Eur J Haematol. 2019; 103(6): 531-41.
7. Rossi D, Paoli LD, Franceschetti S et al. Prevalence and clinical characteristics of immune thrombocytopenic purpura in a cohort of monoclonal gammopathy of uncertain significance. Br J Haematol. 2007; 138(2): 249-52.
8. The International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group. Br J Haematol. 2003; 121(5): 749-57.
9. Sikara MP, Grika EP, Vlachoyiannopoulos PG. Pathogenic mechanisms of thrombosis in antiphospholipid syndrome (Aps). In: Tranquilli A ed. Thrombophilia. InTech. 2011.
10. Galli M, Luciani D, Bertolini G et al. Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature. Blood. 2003; 101(5): 1827-32.
11. Stasi R, Stipa E, Masi M et al. Prevalence and clinical significance of elevated antiphospholipid antibodies in patients with idiopathic thrombocytopenic purpura. Blood. 1994; 84(12): 4203-8.
12. Neunert C, Terrell DR, Arnold DM et al. American Society of Hematology 2019 guidelines for immune thrombocytopenia. Blood Adv. 2019; 3(23): 3829-66.
13. Arachchillage DJ, Cohen H. Use of new oral anticoagulants in antiphospholipid syndrome. Curr Rheumatol Rep. 2013; 15(6): 331.
14. Ghembaza A, Saadoun D. Management of antiphospholipid syndrome. Biomedicines. 2020; 8(11): 508.
15. Ordi-Ros J, Sáez-Comet L, Pérez-Conesa M et al. Rivaroxaban versus vitamin k antagonist in antiphospholipid syndrome. Ann Intern Med. 2019; 171(10): 685-94.
16. Dufrost V, Risse J, Reshetnyak T et al. Increased risk of thrombosis in antiphospholipid syndrome patients treated with direct oral anticoagulants. Results from an international patient-level data meta-analysis. Autoimmun Rev. 2018; 17(10): 1011-21.
17. Woller SC, Stevens SM, Kaplan DA et al. Apixaban for the secondary prevention of thrombosis among patients with antiphospholipid syndrome: study rationale and design (Astro-aps). Clin Appl Thromb Hemost. 2016; 22(3): 239-47.
18. Zangari M, Elice F, Fink L et al. Hemostatic dysfunction in paraproteinemias and amyloidosis. Semin Thromb Hemost. 2007; 33(4): 339-49.
19. Eby C. Pathogenesis and management of bleeding and thrombosis in plasma cell dyscrasias. Br J Haematol. 2009; 145(2): 151-63.
20. Crawford J, Cox EB, Cohen HJ. Evaluation of hyperviscosity in monoclonal gammopathies. Am J Med. 1985; 79(1): 13-22.
21. Djunic I, Elezovic I, Vucic M et al. Specific binding of paraprotein to platelet receptors as a cause of platelet dysfunction in monoclonal gammopathies. Acta Haematol. 2013; 130(2): 101-7.
22. Robert F, Mignucci M, McCurdy SA et al. Hemostatic abnormalities associated with monoclonal gammopathies. Am J Med Sci. 1993; 306(6): 359-66.
23. Dührsen U, Paar D, Kölbel C et al. Lupus anticoagulant associated syndrome in benign and malignant systemic disease – analysis of ten observations. Klin Wochenschr. 1987; 65(18): 852-9.
24. Ganhão S, Silva B, Rodrigues M et al. P3 Is there a relationship between lupus anticoagulant autoantibody and fatigue in jSLE? In: Poster Presentations. Lupus Foundation of America 2020: A30-A1.
25. Friedriksen H, Schmidt K. The incidence of idiopathic thrombocytopenic purpura in adults increases with age. Blood. 1999; 94(3): 909-13.
26. Neylon AJ, Saunders PWG, Howard MR et al; on behalf of the Northern Region Haematology Group. Clinically significant newly presenting autoimmune thrombocytopenic purpura in adults: a prospective study of a population-based cohort of 245 patients: ITP in Adults. Br J Haematol. 2003; 122(6): 966-74.
27. Merlini G, Stone MJ. Dangerous small B-cell clones. Blood. 2006; 108(8): 2520-30.
28. Ho M, Patel A, Goh CY et al. Changing paradigms in diagnosis and treatment of monoclonal gammopathy of undetermined significance (Mgus) and smoldering multiple myeloma (Smm). Leukemia. 2020; 34(12): 3111-25.
29. Phipps C, Chen Y, Gopalakrishnan S et al. Daratumumab and its potential in the treatment of multiple myeloma: overview of the preclinical and clinical development. Ther Adv Hematol. 2015; 6(3): 120-7.
30. Nadeem O, Redd R, Stampleman LV et al. A phase II study of daratumumab in patients with high-risk MGUS and low-risk smoldering multiple myeloma: First report of efficacy and safety. Blood. 2019; 134(suppl 1): 1898.
31. Mouhieddine TH, Weeks LD, Ghobrial IM. Monoclonal gammopathy of undetermined significance. Blood. 2019; 133(23): 2484-94.
32. Dendritic Cell DKK1 Vaccine for Monoclonal Gammopathy and Stable or Smoldering Myeloma. (access: 5.06.2021).
33. Landgren O, Hofmann JN, McShane CM et al. Association of immune marker changes with progression of monoclonal gammopathy of undetermined significance to multiple myeloma. JAMA Oncol. 2019; 5(9): 1293.
34. Lomas OC, Mouhieddine TH, Tahri S et al. Monoclonal gammopathy of undetermined significance (Mgus) – not so asymptomatic after all. Cancers (Basel). 2020; 12(6).

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