Hémophagocytic syndrome: A study of 11 patients in tunisian population.

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Published Feb 25, 2015
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Vol. 92 No. 11 (2014): Issue Nov 2014

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Boukhris Imen
Rachdi Imene
Chérif Eya
Azzabi Samira
Ben Hassine Lamia
Kéchaou Iness
Kaouech Zouleikha
Kooli Chékib
Khalfallah Narjess

Abstract

Background: Hemophagocytic syndromes are mostly associated with underlying pathology, they can reveal: immunodeficiency, infections, hemopathies, cancers and auto-immune diseases. Aim: to investigate clinical, biological features, outcome characteristics and underlying pathology of Tunisian patients with hemophagocytic syndromes.

Methods: A retrospective study of patients with hemophagocytic syndromes admitted in an internal medicine department in Tunis over the period 2009-2012.

Results: There were 11 patients included, 4 men and and 7 women. Mean age was 47,3years. Purpura was observed in 3 cases, hepatosplenomegaly in 6 cases and peripheral lymph nodes in 3 cases. Hemorrhagic complication was noted in 2 cases. There were an increase of inflammatory indices in all the cases. Eight patients had pancytopenia and 3 had bicytopenia. Six patients developed intravascular dissiminated coagulation. High levels of triglyceridemia was noted in 5 cases and an hyperferritinaemia in all cases. Cytological examination of bone marrow confirmed hemophagocytosis in ten cases. Hemophagocytosis was associated to infectious diseases in 6 cases, there were 2 cases of leishmaniasis, septicemia to E.Coli and staphylococcus aureus and lymph nodes tuberculosis with HIV. We also noted a case of lymphoma, myelodysplasic syndrome, rheumatoid polyarthritis, adult onset still’s disease. In one patient, the origin of hemophagocytosis remained indefinite. Visceral leishmaniose were treated by Glucantime®, adult Still’s disease by corticosteroids and méthrotrexate®, lymphoma by chemotherapy. In lymph nodes tuberculosis, there were good evolution. Six patients died.

Conclusion: Hemophagocytic syndrome is a rare and life-threatening disease. Pejorative prognosis requires an early therapy with etiological treatment.

Keywords:

Hemophagocytic syndrome; Hemophagocytosis; Cytopenia

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References

  1. Henter JI, Tondini C, Pritchard J. Histiocyte disorders. Crit Rev Oncol Hematol 2004; 50:157-74
  2. Menasche G, Feldmann J, Fischer A, Saint Basile G. Primary hemophagocytic syndromes point to a direct link between lymphocyte cytotoxicity and homeostasis. Immunol Rev 2005; 203:165-79
  3. Larroche C. Le syndrome d'activation macrophagique acquis de l'adulte : comment le reconnaître et le traiter ? Joint Bone Spine 2012; 79(4):356- 61
  4. Reiner A, Spivak J. Hemophagocytic histiocytosis: a report of 23 new patients and a review of the literature. Medicine 1988; 67:369-88.
  5. 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.
  6. 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
  7. Risdall R, Brunning R, Hernadez J, Gordon D. Bacteria-associated hemophagocytic syndrome. Cancer 1984; 54:2968-72.
  8. Wong K, Chan J. Reactive hémophagocytic syndrome: a clinicopathologic study of 40 patients in an Oriental population. Am J Med 1992; 93:177- 80
  9. Karras A, Hermine O. Syndrome d'activation macrophagique. Rev Med Interne 2002;23: 768-78.
  10. Haddad E, Sulis ML, Jabado N, Blanche S, Fischer A, Tardieu M. Frequency and severity of central nervous system lesions in hemophagocytic lymphohistiocytosis. Blood 1997;89:794-800.
  11. Debatty D, Asdrubal J, Menecier-Ossia L, Clavier M, Latrasse J. Syndrome d'activation macrophagique lié à une légionellose. Med Mal Infect 2002; 32: 251-2
  12. Fisman DN. Hemophagocytic syndromes and infection. Emerg Infect Dis 2000; 6: 601-8
  13. Tsuda H. Hemophagocytic syndrome in children and adults. Int J Hematol 1997;65 : 215-26
  14. Majluf-Cruz A, Sosa-Camas R, Perez-Ramirez O, Rosas-Cabral A, Vargas-Vorackova F, Labardini-Mendez J. Hemophagocytic syndrome associated with haematological neoplasias. Leuk Res 1998; 22:893-8.
  15. . Wong KF, Chan JKC. Hemophagocytic disorders: a review. Hematol Rev 1991;5:5-37.
  16. Henter JI, Horne A, Arico M, et al. Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007; 48:124-31.
  17. Miyahara M, Sano M, Shibata K, et al. B-cell lymphoma-associated hemophagocytic syndrome: clinicopathological characteristics. Ann Hematol 2000;79:378-88.
  18. Grateau G, Bachmeyer C, Blanche P, et al. Haemophagocytic syndrome in patients infected with HIV: 9 cases and a review. J Infect 1997;34:219-25.
  19. Bourquelot P, Oksenhendler E, Wolff M, et al. Syndrome d'hémophagocytose au cours de l'infection par le VIH. Presse Med 1993; 22:1217-20.
  20. Brastianos PK, Swanson JW, Torbenson M, Sperati J, Karakousis PC. Tuberculosis-associated haemophagocytic syndrome. Lancet Infect Dis 2006;6:447-54
  21. Nau GJ, Richmond JFL, Schlesinger A, Jennings EG, Lander ES, Young RA. Human macrophage activation programs induced by bacterial pathogens. Proc Natl Acad Sci U S A 2002;99:1503-8
  22. Stéphan F, Thiolière B, Verdy E, Tulliez M. Role of hemophagocytic histiocytosis in the etiology of thrombocytopenia in patients with sepsis syndrome or septic shock. Clin Infect Dis 1997;25:1159-64
  23. . El Khoury N, Lassoued K, Pellé G, Foucher A, Costa MA, Rondeau E. Syndrome d'activation macrophagique associé à une septicémie à Escherichia coli : à propos d'un cas. Rev Med Interne 2003;24:688-91.
  24. Rajagopala S, Dutta U, Chandra KS, Bhatia P, Varma N, Kochhar R. Visceral leishmaniasis associated haemophagocytic lymphohistiocytosis - case report and systematic review. J Infect 2008;56:381-8.
  25. Kontopoulou T, Tsaousis G, Vaidakis E, et al. Hemophagocytic syndrome in association with visceral leishmaniasis. Am J Med 2002;113: 439-40.
  26. Kilani B, Ammari L, Kanoun F, Ben Chaabane T, Abdellatif S, Chaker E. Hemophagocytic syndrome associated with visceral leishmaniasis. Int J Infect Dis 2006; 10:85-6
  27. Marthur P, Samantaray JC, Samanta P. Fatal haemophagocytic syndrome and hepatitis associated with visceral leishmaniasis. Indian J Med Microbiol 2007;25:416
  28. Rajagopala S, Dutta U, Chandra KS, Bhatia P, Varma N, Kochhar R. Visceral leishmaniasis associated hemophagocytic lymphohistiocytosis- case report and systematic review. J Infect 2008;56:381-8
  29. Emmenegger U, Reimers A, Frey U, et al. Reactive macrophage activation syndrome: a simple screening strategy and its potential in early treatment initiation. Swiss Med Wkly 2002;132:230-6.
  30. Wong KF, Hui PK, Chan J, Chan YW, Ha SY. The acute lupus hemophagocytic syndrome. Ann Int Med 1991;114:387-90.
  31. Katoh N, Gono T, Mitsuhashi S, et al. Haemophagocytic syndrome associated with rheumatoid arthritis. Intern Med 2007;46:1809-13
  32. Dhote R, Simon J, Papo T, et al. Reactive hemophagocytic syndrome in adult systemic disease: report of twenty-six cases and literature review. Arthritis care and research 2003;49:633-9.
  33. Ravelli A, Caria MC, Buratti S, Malattia C, Temporini F, Martini A. Methotrexate as a possible trigger of macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol 2001;28:865-7.
  34. ajluf-Cruz A, Sosa-Camas R, Perez-Ramirez O, Rosas-Cabral A, Vargas-Vorackova F, Labardini-Mendez J. Hemophagocytic syndrome associated with haematological neoplasias. Leuk Res 1998;22:893-8.
  35. Créput C, Galicier L, Buyse S, Azoulay E. Understanding organ dysfunction in hemophagocytic lymphohistiocytosis. Intensive Care Med 2008;34:1177-87.
  36. Rouphael NG, Talati NJ, Vaughan C, Cunningham K, Moreira R, Gould C. Infections associated with haemophagocytic syndrome. Lancet Infect Dis 2007;7:814-22
  37. Imashuku S. Advances in the management of hemophagocytic lymphohistiocytosis. Int J Hematol 2000;72:1-11
  38. Prahalad S, Bove KE, Dickens D, Lovell DJ, Grom AA. Etanercept in the treatment of macrophage activation syndrome. J Rheumatol 2001;28:2120-4.
  39. Veerakul G, Sanpakit K, Tanphaichitr V, Mahasandana C, Jirarattanosopa N. Secondary hemophagocytic lymphohistiocytosis in children - an analysis of etiology and outcome. J Med Assoc Thai 2002;85:530-41.