Necrose de medula óssea em paciente portadora de lúpus eritematoso sistêmico

Autores

  • Cláudia Hilbig PUC-SP/FCMS
  • José Victor Martinez PUC-SP/FCMS
  • José Eduardo Martinez PUC-SP/FCMS

DOI:

https://doi.org/10.5327/Z1984-4840201625947

Palavras-chave:

lúpus eritematoso sistêmico, pancitopenia, necrose, medula óssea

Resumo

As manifestações hematológicas do lúpus eritematoso sistêmico (LES) são causas de morbidade e risco aumentado de mortalidade global. Paciente jovem, mulher, com LES e síndrome antifosfolípide (SAF) apresentou pancitopenia severa após infecção urinária. A biópsia de medula óssea (MO) mostrou necrose e fibrose reticulínica. O mecanismo mais comum de pancitopenia é a produção de anticorpos periféricos. No entanto, pode ocorrer raramente com aplasia ou necrose de MO. Necrose de MO é mais frequentemente associada a doenças neoplásicas, infecções severas e anemia falciforme, mas também é descrita em pacientes com LES. Tal necrose é vista, mais raramente, em pacientes com SAF primária. Alterações na microcirculação da MO levam a isquemia necrose. As principais complicações na necrose de MO são pancitopenia e embolia. Poucos estudos da literatura apontam a necrose de medula óssea como causa de pancitopenia em pacientes lúpicos. A necrose de MO é uma entidade relativamente rara e de mau prognóstico.

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Biografia do Autor

Cláudia Hilbig, PUC-SP/FCMS

Especializanda em Reumatologia

José Victor Martinez, PUC-SP/FCMS

Estudante de Medicina

José Eduardo Martinez, PUC-SP/FCMS

Titular do Departamento de Medicina

Referências

Voulgarelis M, Giannouli S, Tasidou A, Anagnostou D, Ziakas PD, Tzioufas AG. Bone marrow histological findings in systemic lupus erythematosus with hematologic abnormalities: a clinicopathological study. Am J Hematol. 2006;81(8):590-7.

Palanichamy A, Bauer JW, Yalavarthi S, Meednu N, Barnard J, Owen T, et al. Neutrophil-mediated IFN activation in the bone marrow alters B cell development in human and murine systemic lupus erythematosus. J Immunol. 2014;192(3):906-18.

Zhuang H, Han S, Xu Y, Li Y, Wang H, Yang LJ, et al. Toll-like receptor 7-stimulated tumor necrosis factor α causes bone marrow damage in systemic lupus erythematosus. Arthritis Rheumatol. 2014;66(1):140-51.

Pereira RM, Velloso ER, Menezes Y, Gualandro S, Vassalo J, Yoshinari NH. Bone marrow findings in systemic lupus erythematosus patients with peripheral cytopenias. Clin Rheumatol. 1998;17(3):219-22.

Chu VT, Enghard P, Schürer S, Steinhauser G, Rudolph B, Riemekasten G, et al. Systemic activation of the immune system induces aberrant BAFF and APRIL expression in B cells in patients with systemic lupus erythematosus. Arthritis Rheum. 2009;60(7):2083-93.

Vasudevan AR, Ginzler EM. Clinical features of systemic lupus erythematosus. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (Eds.). Rheumatology. 5th ed. Philadelphia: Mosby Elsevier; 2010. p. 1229-46.

Newman K, Owlia MB, El-Hemaidi I, Akhtari M. Management of immune cytopenias in patients with systemic lupus erythematosus: old and new. Autoimmun Rev. 2013;12(7):784-91.

Sarkar RN, Banerjee S, Dey S, Saha A, Bhattacharjee P, Banerjee TK, et al. Haematological presentation of systemic lupus erythematosus. J Assoc Physicians India. 2009;57:767-8.

Ahmad SQ, Khan O, Zafar SI, Zafar SN. A case of systemic lupus erythematosus with aplastic anaemia. J Pak Med Assoc. 2011;61(8):817-9.

Levine AB, Erkan D. Clinical assessment and management of cytopenias in lupus patients. Curr Rheumatol Rep. 2011;13(4):291-9.

Lanna CCD, Ferreira GA, Telles RW. Lúpus eritematoso sistêmico. In: Carvalho MAP, Lanna CCD, Bertolo MB, Ferreira GA. Reumatologia: diagnóstico e tratamento. 4ª ed. São Paulo: AC Farmacêutica; 2013. p. 378-410.

Hochberg MC. Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1997;40(9):1725.

Petri M, Orbai AM, Alarcón GS, Gordon C, Merrill JT, Fortin PR, et al. Derivation and validation of the Systemic Lupus International Collaborating Clinics classification criteria of systemic lupus erythematosus. Arthritis Rheum. 2012;64(8):2677-86. doi: 10.1002/art.34473.

Dall’Era M. Lúpus eritematoso sistêmico. In: Imboden JB, Hellmann DB, Stone JH. Current Reumatologia: diagnóstico e tratamento. 3ª ed. Porto Alegre: AMGH; 2014. p. 187-97.

Young NS. Anemia aplásica, mielodisplasia e síndromes relacionadas à insuficiência da medula óssea. In: Longo DL, Jameson JL, Fauci AS, Hauser SL, Loscalzo J. Medicina interna de Harrison. 18ª ed. Porto Alegre: AMGH; 2013. p. 887-97.

Kuwama M, Kaburaki J, Okazaki Y, Miyazaki H, Ikeda Y. Two types of autoantibody-mediated thrombocytopenia in patients with systemic lupus erythematosus. Rheumatology (Oxford). 2006;45(7):851-4. Epub 2006 Jan 17.

Miller MH, Urowitz MB, Gladman DD. The significance of thrombocytopenia in systemic lupus erythematosus. Arthritis Rheum. 1983;26(10):1181-6.

Itoh Y, Sekine H, Hosono O, Takeuchi T, Koide J, Takano M, et al. Thrombotic thrombocytopenic purpura in two patients with systemic lupus erythematosus: clinical significance of anti-platelet antibodies. Clin Immunol Immunopathol. 1990;57(1):125-36.

Rangaswamy M, Prabhu, Nandini NM, Manjunath GV. Bone marrow examination in pancytopenia. J Indian Med Assoc. 2012;110(8):560-2, 566.

Tagoe C, Shah A, Yee H, Belmont HM. Aplastic anemia in systemic lupus erythematosus: a distinct presentation of acquired aplastic anemia? J Clin Rheumatol. 2001;7(6):377-83.

Li X, Liu L, Meng D, Wang D, Zhang J, Shi D, et al. Enhanced apoptosis and senescence of bone-marrow-derived mesenchymal stem cells in patients with systemic lupus erythematosus. Stem Cells Dev. 2012;21(13):2387-94. doi: 10.1089/scd.2011.0447. Epub 2012 Apr 20.

Papo T, Andre MH, Amoura Z, Lortholary O, Tribout B, Guillevin L, et al. The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus. J Rheumatol. 1999;26(4):927-30.

Nossent JC, Swaak AJ. Pancytopenia in systemic lupus erythematosus related to azathioprine. J Intern Med. 1990;227(1):69-72.

Funauchi M, Ohno M, Yamagata T, Nozaki Y, Kinoshita K, Kanamaru A. Effects of liposteroid on the hemophagocytic syndrome in systemic lupus erythematosus. Lupus. 2003;12(6):483-5.

Arnez MAC, Azevedo MNL, Bica BERG. Reactive haemophagocytic syndrome in a systemic lupus erythematosus patient: case report. Rev Bras Reumatol. 2012;52(5):790-5.

Nawata M, Suzuki J, Ikeda K, Ando S, Koike M, Sekigawa I, et al. Haemophagocytic syndrome in a systemic lupus erythematosus patient with antiphospholipid antibodies. Rheumatology (Oxford). 2001;40(7):828-9.

Oliveira LR, Ferreira TC, Neves FF, Meneses ACO. Aplastic anemia associated to systemic lupus erythematosus in an AIDS patient: a case report. Rev Bras Hematol Hemoter. 2013;35(5):366-8. doi: 10.5581/1516-8484.20130100.

Khamashta MA, Pierangeli S, Harris EN. Antiphospholipid syndrome: overview of pathogenesis, diagnosis, and management. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (Eds.). Rheumatology. 5th ed. Philadelphia: Mosby Elsevier; 2010. p. 1351-9.

Cervera R, Piette JC, Font J, Khamashta MA, Shoenfeld Y, Camps MT, 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.

Uthman I, Godeau B, Taher A, Khamashta M. The hematologic manifestations of the antiphospholipid syndrome. Blood Rev. 2008;22(4):187-94. doi: 10.1016/j.blre.2008.03.005.Epub 2008 Apr 15.

Fong KY, Loizou S, Boey ML, Walport MJ. Anticardiolipin antibodies, haemolytic anaemia and thrombocytopenia in systemic lupus erythematosus. Br J Rheumatol. 1992;31(7):453-5.

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

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

Norgard MJ, Carpenter JT Jr, Conrad ME. Bone marrow necrosis and degeneration. Arch Intern Med. 1979;139(8):905-11.

Kiraly JF, Munsey SW. Bone marrow necrosis. Am J Med. 1976;60(3):361-8. doi: http://dx.doi.org/10.1016/0002-9343(76)90752-X

Feng CS, Ng MH, Szeto RS, Li EK. Bone marrow findings in lupus patients with pancytopenia. Pathology. 1991;23(1):5-7.

Janssens AM, Offner FC, Van Hove WZ. Bone marrow necrosis. Cancer. 2000;88(8):1769-80.

Sinha J, Chowdhry I, Sedan S, Barland P. Bone marrow necrosis and refractory HELLP syndrome in a patient with catastrophic antiphospholipid antibody syndrome. J Rheumatol. 2002;29(1):195-7.

Schaar CG, Ronday KH, Boets EP, van der Lubbe PA, Breedveld FC. Catastrophic manifestation of the antiphospholipid syndrome. J Rheumatol. 1999;26(10):2261-4.

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

Sultan SM, Begum S, Isenberg DA. Prevalence, patterns of disease and outcome in patients with systemic lupus erythematosus who develop severe haematological problems. Rheumatology (Oxford). 2003;42(2):230-4.

Cervera R, Khamashta MA, Font J, Sebastiani GD, Gil A, Lavilla P, et al. Morbidity and mortality in systemic lupus erythematosus during a 5-year period. A multicenter prospective study of 1,000 patients. European Working Party on Systemic Lupus Erythematosus. Medicine (Baltimore). 1999;78(3):167-75.

Beyan E, Beyan C, Turan M. Hematological presentation in systemic lupus erythematosus and its relationship with disease activity. Hematology. 2007;12(3):257-61.

Kiss E, Gál I, Simkovics E, Kiss A, Bányai A, Szakáll S, et al. Myelofibrosis in systemic lupus erythematosus. Leuk Lymphoma. 2000;39(5-6):661-5.

Konstantopoulos K, Terpos E, Prinolakis H, Kanta A, Variami E, Kanellopoulou G, et al. Systemic lupus erythematosus presenting as myelofibrosis. Haematologia (Budap). 1998;29(2):153-6.

Inoue Y, Matsubara A, Okuya S, Okafuji K, Kaku K, Kaneko T. Myelofibrosis and systemic lupus erythematosus: reversal of fibrosis with high-dose corticosteroid therapy. Acta Haematol. 1992;88(1):32-6.

Dall’Era M, Wofsy D. Tratamento do lúpus eritematoso sistêmico. In: Imboden JB, Hellmann DB, Stone JH. Current Reumatologia: diagnóstico e tratamento. 3ª ed. Porto Alegre: AMGH; 2014. p. 198-203.

McCune WJ, Cagnoli PC, Gonzalez-Rivera T. Treatment of non-renal lupus. In: Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH (Eds.). Rheumatology. 5th ed. Philadelphia: Mosby Elsevier; 2010. p. 1307-16.

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2017-01-23

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1.
Hilbig C, Martinez JV, Martinez JE. Necrose de medula óssea em paciente portadora de lúpus eritematoso sistêmico. Rev. Fac. Ciênc. Méd. Sorocaba [Internet]. 23º de janeiro de 2017 [citado 28º de março de 2024];18(4):187-92. Disponível em: https://revistas.pucsp.br/index.php/RFCMS/article/view/25947

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