Autoimmune myelofibrosis revealing a systemic lupus erythematosus
  • Fatimaezzahra Bensalek
    Department of Internal Medicine, University Hospital Centre Mohammed VI, Marrakech, Morocco
  • Hajar Joulal
    Department of Internal Medicine, University Hospital Centre Mohammed VI, Marrakech, Morocco
  • Jaouad Yousfi
    Department of Internal Medicine, University Hospital Centre Mohammed VI, Marrakech, Morocco
  • Mouna Zahlane
    Department of Internal Medicine, University Hospital Centre Mohammed VI, Marrakech, Morocco
  • Leila Benjilali
    Department of Internal Medicine, University Hospital Centre Mohammed VI, Marrakech, Morocco
  • Lamiaa Essaadouni
    Department of Internal Medicine, University Hospital Centre Mohammed VI, Marrakech, Morocco

Keywords

Autoimmune myelofibrosis, systemic lupus erythematosus, corticosteroid therapy

Abstract

Background: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, characterised by multi-organ affections. Haematological involvement is a common manifestation of SLE, consisting of autoimmune peripheral cytopenia. Autoimmune myelofibrosis (AIMF) is a rare cause of cytopenia in SLE; it could precede or be concurrent with the diagnosis of SLE. There are few studies that describe this association.
Case description: We report a case of AIMF revealing the diagnosis of SLE in a 34-year-old female, presented with episodes of gingival bleeding associated with peripheral inflammatory polyarthralgia, photosensitivity and deterioration of general condition. Clinical examination revealed a soft pitting oedema in the lower limbs. Laboratory investigations showed a pancytopenia, inflammatory biological syndrome, with positive 24-hour proteinuria and anti-native DNA antibodies. A bone marrow biopsy showed diffuse myelofibrosis associated with maturation disorders and no tumour infiltrate. Renal biopsy revealed proliferative glomerulonephritis class III with immune deposits.
Conclusion: The association of AIMF with SLE has been rarely reported, and it could be another cause for cytopenia in SLE.

VIEW THE ENTIRE ARTICLE

References

  • 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 for systemic lupus erythematosus. Arthritis Rheum 2012;64:2677–2686.
  • Wanitpongpun C, Teawtrakul N, Mahakkanukrauh A, Siritunyaporn S, Sirijerachai C, Chansung K. Bone marrow abnormalities in systemic lupus erythematosus with peripheral cytopenia. Clin Exp Rheumatol 2012;30:825–829.
  • Vergara-Lluri ME, Piatek CI, Pullarkat V, Siddiqi IN, O’Connell C, Feinstein DI, et al. Autoimmune myelofibrosis: an update on morphologic features in 29 cases and review of the literature. Hum Pathol 2014;45:2183–2191.
  • Fayyaz A, Igoe A, Kurien BT, Danda D, James JA, Stafford HA, et al. Haematological manifestations of lupus. Lupus Sci Med 2015;2:e000078.
  • Paquette RL, Meshkinpour A, Rosen PJ. Autoimmune myelofibrosis. A steroid-responsive cause of bone marrow fibrosis associated with systemic lupus erythematosus. Medicine (Baltimore) 1994;73:145–152.
  • Tefferi A. Primary myelofibrosis: 2017 update on diagnosis, risk-stratification, and management. Am J Hematol 2016;91:1262–1271.
  • Chalayer E, Ffrench M, Cathébras P. Bone marrow fibrosis as a feature of systemic lupus erythematosus: a case report and literature review. Springerplus 2014;3:349.
  • Del Porto F, Tatarelli C, Di Napoli A, Proietta M. Systemic lupus erythematosus and myelofibrosis: a case report and revision of literature. Leuk Res Rep 2018;9:58–64.
  • Ungprasert P, Chowdhary VR, Davis MD, Makol A. Autoimmune myelofibrosis with pancytopenia as a presenting manifestation of systemic lupus erythematosus responsive to mycophenolate mofetil. Lupus 2016;25:427–430.
  • Views: 285
    HTML downloads: 14
    PDF downloads: 139


    Published: 2024-05-13
    Issue: 2024: Vol 11 No 6 (view)


    How to cite:
    1.
    Bensalek F, Joulal H, Yousfi J, Zahlane M, Benjilali L, Essaadouni L. Autoimmune myelofibrosis revealing a systemic lupus erythematosus. EJCRIM 2024;11 doi:10.12890/2024_004511.