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Colegio de Medicina Interna de México.

2022, Number 5

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Med Int Mex 2022; 38 (5)

Systemic lupus erythematosus and its association with hemophagocytic syndrome as an initial manifestation

Medina-Castillo J, Becerra-Márquez AM, Borjon-Cabada IA

Language: Spanish
References: 10
Page: 1090-1095
PDF size: 209.47 Kb.


ABSTRACT

Background: Hemophagocytic syndrome is a hyperinflammatory state, potentially fatal, characterized by excessive activation of macrophages and T cells. Systemic lupus erythematosus is an autoimmune condition that predisposes to hemophagocytic syndrome. The appearance of systemic lupus erythematosus and hemophagocytic syndrome is rare.
Clinical case: A 16-year-old male patient who presented fever for one month and lymphadenopathy prior to admission. During his evaluation he fulfilled 10 points for EULAR/ACR 2019, classifying as systemic lupus erythematosus. Hemophagocytosis was observed in the bone marrow aspirate. The diagnosis of hemophagocytic syndrome secondary to systemic lupus erythematosus was concluded. Treatment was started with intravenous methylprednisolone and mycophenolic acid, showing improvement, so he was subsequently discharged.
Conclusions: Hemophagocytic syndrome is not one of the frequent manifestations of systemic lupus erythematosus, so, we must maintain a high suspicion of its association for timely treatment.


REFERENCES

  1. Ramos-Casals M, Brito-Zerón P, López-Guillermo A,Khamashta MA, Bosch X. Adult haemophagocytic syndrome.Lancet 2014; 383 (9927): 1503-16. https://doi.org/10.1016/S0140-6736(13)61048-X.

  2. Bode S, Lehmberg K, Maul-Pavicic A, Vraetz T, Janka G, StadtU, et al. Recent advances in the diagnosis and treatment ofhemophagocytic lymphohistiocytosis. Arthr Res Ther 2012;14 (3): 213. https://doi.org/10.1186/ar3843.

  3. Stevens M, Frobisher C, Hawkins M, Jenney M, Lancashire E,Reulen R, et al. The British Childhood Cancer Survivor Study:Objectives, methods, population structure, response ratesand initial descriptive information. Pediatr Blood Cancer2008; 50 (5): 1018-25. https://doi.org/10.1002/pbc.21335.

  4. Wang HY, Yang CF, Chiou TJ, Yang SH, Gau JP, Yu Y Bin, etal. Risk of hemophagocytic lymphohistiocytosis in adultswith fevers of unknown origin: the clinical utility of a newscoring system on early detection. Hematol Oncol 2017; 35(4): 835-44. https://doi.org/10.1002/hon.2333.

  5. Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, HoritaT, et al. Clinical features of haemophagocytic syndrome inpatients with systemic autoimmune diseases: Analysis of30 cases. Rheumatology 2008; 47 (11): 1686-91. https://doi.org/10.1093/rheumatology/ken342.

  6. Kalra S, Aggarwal A. Hemophagocytic lymphohistiocytosisas presenting feature of lupus. Indian Pediatr 2012; 49 (12):993. https://doi.org/10.1007/s13312-012-0227-x.

  7. Gupta D, Mohanty S, Thakral D, Bagga A, Wig N, Mitra DK.Unusual association of hemophagocytic lymphohistiocytosisin systemic lupus erythematosus: Cases reportedat tertiary care center. Am J Case Rep 2016; 17: 739-44.https://doi.org/10.12659/ajcr.899433.

  8. Dubuc CAE, Ecenarro MU, Villalba CM, Cáceres VA, RubioIH, Otano JB. Hemophagocytic syndrome as the initialmanifestation of systemic lupus erythematosus. ReumatologíaClínica 2014; 10 (5): 321-324. DOI: 10.1016/j.reumae.2013.09.004.

  9. Vilaiyuk S, Sirachainan N, Wanitkun S, Pirojsakul K,Vaewpanich J. Recurrent macrophage activation syndromeas the primary manifestation in systemic lupus erythematosusand the benefit of serial ferritin measurements: Acase-based review. Clin Rheumatol 2013; 32 (6): 899-904.https://doi.org/10.1007/s10067-013-2227-1.

  10. Thomas M, Robert A, Kuruvilla N, C U. An unusual presentationof systemic lupus erythematosus as hemophagocyticlymphohistiocytosis in a male. Cureus 2019; 11 (8). https://doi.org/10.7759/cureus.5427.




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