Hemophagocytic syndrome: Report of 4 cases

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Published Jan 18, 2011
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Vol. 89 No. 1 (2011): Issue Jan 2011

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Besma Ben Dhaou Hmaidi
Fatma Derbali
Fatma Boussema
Sonia Ketari Jamoussi
Lilia Baili
Samir Kochbati
Ouahida Cherif
Lilia Rokbani

Abstract

Background: Hemophagocytic syndrome (HPS) is a rare but potentially fatal disease. The diagnosis is based on the combination of clinical and biological signs, requiring histological or cytological research hemophagocytosis and exhaustive etiological investigation.
Aim: To report four cases of the HPS in an internal medicine department.
Cases report: We report four cases of HPS associated with Still's disease in two cases, with Sjogren syndrome in one case and a severe sepsis in one case. There are three women and one man. The mean age was 34.75 years, with extremes of 21 to 50 years. In all cases, patients were hospitalized for fever of unknown origin. The etiologic research of this fever remained negative. In all cases, patients validated criteria of HPS confirmed by cytological studies. The treatment was based on corticosteroids in all cases and immunosuppressant in one case. The evolution was favorable in two cases and fatal in two cases.
Conclusion: HPS is a serious, often overlooked, may affect the prognosis and complicating various infectious, neoplastic or autoimmune diseases.

Keywords:

Hemophagocytosis, Hemophagocytic syndrome, Still's disease, sepsis

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References

  1. Risdall RJ, McKenna RW, Nesbit ME, Krivit W, et al. Virusassociated hemophagocytic syndrome: a benign histiocytic proliferation distinct from malignant histiocytosis. Cancer 1979; 44: 993-1002.
  2. Reiner A, Spivak J. Hemophagocytic histiocytosis: a report of 23 new patients and a review of the literature. Medicine 1988; 67: 369-88.
  3. Rostaing L, Fillola G, Baron E, Cisterne JM, Durand D. Course of hemophagocytic syndrome in renal transplant patients. Transplantation 1995; 60: 506-9.
  4. Imashuku S, Ikushima S, Hibi S, Todo S. Langherans cell histiocytosis and hemophagocytic syndrome in Japan: epidemiological studies. Int J Pediatr Hematol Oncol 1994; 1: 241-6.
  5. Fisman DN. Hemophagocytic syndromes and infection. Emerg Infect Dis 2000; 6: 601-8.
  6. Karras A, Hermine O. Hemophagocytic syndrome. Rev Med Interne 2002; 23:768-78
  7. Dalle J.H. Syndrome d'activation macrophagqiue chez l'enfant inféctés par le HIV. A proopos de trois cas. Arch Pédiatr 1995 ; 2 : 442- 6.
  8. Tiab M, Mechinaud F, Hamidou M, Gaillard F, Raffi F, Harousseau JL. Syndromes hémophagocytaires, une série de 23 observations. Ann Med Interne 1996; 147:138-44.
  9. Sailler L, Duchayne E, Marchou B, et al. Aspects étiologiques des hémophagocytoses réactionnelles : étude rétrospective chez 99 patients. Rev Med Interne 1997; 18: 855-64.
  10. Kaito K, Kobayashi M, Katayama T, et al. Prognostic factors of hemophagocytic syndrome in adults: analysis of 34 cases. Eur J Haematol 1997; 59: 247-53.
  11. Debatty D et al. Syndrome d'activation macrophagique lié à une légionellose. Med Mal Infest 2002 ; 32 : 251-2.
  12. Henter JI, Horne A, Aricó M, et al. HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124-31
  13. Wong KF, Hui PK, Chan J, Chan YW, Ha SY. The acute lupus hemophagocytic syndrome. Ann Int Med 1991; 114: 387-90
  14. Fukaya S, Yasuda S, Hashimoto T, et al. Rheumatology 2008; 47: 1686-91
  15. Albert A, Azgui Z, Buisine J, et al. Macrophage activation syndromes. Nouv Rev Fr Hematol 1992; 34: 435-41.
  16. Tsuda H. Hemophagocytic syndrome in children and adults. Int J Hematol 1997; 65:215-26.
  17. Abdelkafi A, Ben Jamil W, Torjman L, et al. Hemophagocytic syndrome after hematopoietic stem cell transplantation : a prospective observational study. Int J Hematol 2009 ; 89 : 368 - 373.
  18. Gadhouma H, Aslangul E, Szwebel T, Perrot S, Romnicianu S, Le Jeunne C. Spectre étiologique du syndrome d'activation macrophagique en médecine interne : à propos de sept cas monocentriques. Rev Méd Int 2006 ; 27 : S394
  19. Veerakul G, Sanpakit K, Tanphaichitr V, Mahasandana C, Jirarattanosopa N. Secondary hemophagocytic lymphohistiocytosis in children — an analysis of etiology and outcome. J Med Ass Thailand 2002; 85: S530-41