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Case Study








ABSTRACT: Macrophage activation syndrome (MAS) belongs to the hemophagocytic lymphohistiocytosis group of diseases. It is an anatomo-clinical condition resulting from the inappropriate proliferation and activation of macrophagic cells. This rare but potentially fatal syndrome can be primary or secondary to certain pathologies dominated by infections and neoplasia. In adults, MAS is rarely associated with systemic lupus erythematosus, but it arises as a complication of several systemic autoimmune diseases. Here we report the case of 30-year-old woman who presented with a pruritic rush. She met the Systemic Lupus International Collaborating Clinics (CLICC) criteria for the diagnosis of Systemic Lupus Erythematosus (SLE). The bone marrow showed the presence of abundant hemosiderophages with focal hemophagocytosis. Due to the overlap in clinical findings, SLE-associated MAS might be underdiagnosed. This case represents the importance of prompt diagnosis and treatment of such a potentially fatal clinical syndrome.

KEYWORD:  Macrophage activation syndrome, lymphohistiocytosis, Systemic Lupus International Collaborating Clinics, SLE-associated MAS


  1. [1]. Bain B, Clark D, Wilkins B. Bone marrow pathology. International Journal of Hematology 2019;12: 157-159.

  2. [2]. Granata G, Didona D, Stifano G, Feola A, Granata M. Macrophage Activation Syndrome as Onset of Systemic Lupus Erythematosus: A Case Report and a Review of the Literature. Case Reports in Medicine. 2015; 20:178-181.

  3. [3]. Tsuda H.Serum lipids in adult patients with hemophagocytic syndrome. hematology KS-A journal 1996; 53:1096-8652

  4. [4]. Emmenegger U, Reimers A, Frey U, Fux C, Bihl F, Semela D.  Reactive Macrophage Activation Syndrome: a simple screening strategy and its potential in early treatment initiation. Annales françaises de biologie, 12 ; 85 : 130-132.

  5. [5]. Kumakura S, Ishikura H, Kondo M. Autoimmune-associated hemophagocytic syndrome. Rhumato Rev. 2004, 25: 152-155

  6. [6]. Berrady R, Bono W. Le syndrome d’activation lympho-histiocytaire (SALH). Annales Françaises d’Anesthésie et de Réanimation. 2014 Jan 1;33,1:26–32.

  7. [7]. Sharmeen S, Hussain N. An Unusual Case of Systemic Lupus Erythematosus and Hemophagocytic Syndrome. Case Reports in Rheumatology. 2016:1–5.

  8. [8]. Henter JI, Elinder G, Soder O, Ost A. Incidence in Sweden and clinical features of familial hemophagocytic lymphohistiocytosis. Acta Paediatrica Scandinavica. 1991;80,4:428–35.

  9. [9]. Nadir Z, T. Luna. Differential diagnosis of hemophagocytic syndrome: underlying disorders and selection of the most effective treatment. Blood. 1997;90,4:439-442.

  10. [10]. Papo T, Andre M, Amoura Z. The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus. Rheumatology. 1999; 72,3: 422-428

  11. [11]. Dhote R, Simon J, Papo T, Detournay B, Sailler L, Andre M-H. Reactive hemophagocytic syndrome in adult systemic disease: report of twenty‐six cases and literature review. Blood.2003;80,5:712-715.

  12. [12]. Lu M, Yu S, Xu W, Gao B, Xiong S. HMGB1 Promotes Systemic Lupus Erythematosus by Enhancing Macrophage Inflammatory Response. Journal of Immunology Research.2015; 12,2:551-554.

  13. [13]. Kumakura S. Clinical characteristics and treatment outcomes of autoimmune‐associated hemophagocytic syndrome in adults. Journal of Rheumatology Research.2014;66,8:2297–307.

  14. [14]. Wong K. Reactive hemophagocytic syndrome—a clinicopathologic study of 40 patients in an Oriental population. Medicine JC-TA Journal. 1992;70,2:1198-206.

  15. [15]. Tiab M., Mechinaud F., Hamidou M., Gaillard F., Raffi. diagnosis of hemophagocytic syndrome. Blood. 2003;55,6:1095-103.

  16. [16]. Imashuku S. Differential diagnosis of hemophagocytic syndrome: underlying disorders and selection of the most effective treatment. International Journal of Hematology.1997;147:116-120.

  17. [17]. Berrady R. Le syndrome d’activation lympho-histiocytaire (SALH). Ann françaises d’anesthésie.2014 ;139 :240-243

  18. [18]. Kaito K, Kobayashi M, Katayama T, Otsubo H, Ogasawara Y, Sekita T. Prognostic factors of hemophagocytic syndrome in adults: Analysis of 34 cases. European Journal of Haematology. 1997;59,4:247–53.

  19. [19]. Cerny A, Bihl F, Emmenegger U, Reimers A, Frey U, Fux C. Reactive macrophage activation syndrome: a simple screening strategy and its potential in early treatment initiation. Swss med WKLS,2002;132:2030-236.

  20. [20]. Mecha B, Ketatto S. Hepatic manifestations of hemophagocytic syndrome: a study of 30 cases. International Journal of Hematology.2011;80,2:155-159.

 To cite this article:

ET-TAHOURIZ, OUSSAGA J, Belmekki A, EL YAHYAOUI H, AMEUR MA, CHAKOUR M Macrophage activation syndrome as onset of systemic lupus erythematosus: a case report. Int. J. Med. Lab. Res. 2022; 7,1:44-48.

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