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Revista de la Asociación Médica del Centro Médico ABC

2001, Number 3

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An Med Asoc Med Hosp ABC 2001; 46 (3)

Hemophagocytic syndrome in pediatrics

Frenkel-Salamón M, Bolea-Murga V, Durán-Padilla MA

Language: Spanish
References: 23
Page: 137-141
PDF size: 60.20 Kb.


ABSTRACT

The hemophagocytic syndrome, also known as macrophage activation syndrome or reactive histiocitosis, is a heterogeneous nosological entity that was described more than 60 years ago. Its etiology is still unknown, but it has been related to a decontrol and exaggerated response of the immunological system. It has a 1-2 incidence for every million children without predominance of age or sex. The clinical manifestations include the presence of a febrile syndrome, hepatosplenomegaly, coagulation alterations, hepatic dysfunction, neurological disorders and multiple organic failure. Paraclinical studies show pancitopenia and a clear hemophagocytosis especially in bone marrow aspirate smear. The treatment can vary, depending on the triggering cause, and the prognosis also will depend directly upon the kind of hemophagocytic syndrome involved. Average death rate can be 60%.


REFERENCES

  1. Herrero-Hernández A, Ramírez-Jiménez S, García-Martín F, Martínez-Valverde A. Síndromes hemofagocíticos. An Esp Pediatr 1998; 49: 230-236.

  2. Chan JK, Ng CS, Law CK, Ng WF, Wong KF. Reactive hemophagocytic syndrome: A study of 7 fatal cases. Pathology 1987; 19: 43-50.

  3. Scott RB, Robb-Smith AHT. Histiocitica medullar reticulosis. Lancet 1939; 2: 194-198.

  4. Ladisch S. Histiocitosis syndromes of childhood. In: Nelson WE, Behrman RE, Kliegman RM, Arvin AN (eds). Nelson textbook of paediatrics. 16th ed. Philadelphia PA: WB Saunders, 2000: 1570-1572.

  5. Komp DM. Historical perspectives of Langerhans cell histiocitosis. Hematol Oncol Clin North Am 1987; 1: 9-21.

  6. Cline MJ. Histiocyte and histiocitosis. Blood 1994: 2840-2853.

  7. Rappaport H. Tumors of the hematopoietic system. Atlas of tumour pathology. Section III, Fascicle 8. Washington DC: AFIP, 1966: 49-63.

  8. Risdall RJ, McKenna RW, Nesbit ME et al. Virus-associated hemophagocytic syndrome, a benign histiocytic proliferation distinct from malignant histiocitosis. Cancer 1979; 44: 993-1002.

  9. Hesseling PB, Wessels G, Egeler RM, Rossow DJ. Simultaneous occurrence of viral-associated hemophagocytic syndrome and Langerhans cell histiocitosis: A case report. Pediatr Hematol Oncol l995; 12: 135-141.

  10. Blanche S. Lymphohistiocytose familiale et autres syndromes d’activation macrophagique. Edition Techniques-Encycl. Méd Chir (Paris-France), Pédiatrie, 4-082-J-40, 1995: 2.

  11. Ladisch S, Jaffe E. The histiocytoses. In: Pizo PA, Poplack DG (eds). Principles and practice of paediatric oncology. 3rd ed. Philadelphia PA: Lippincott-Raven Publishers, 1997: 615-631.

  12. Baumann MA, Libnoch JA. Hemophagocytosis. JAMA 1984; 252: 1340.

  13. Astigarra-Aguirre I, Fernández-Teijeiro A, García-Pérez N, Piñan-Francés MA, Navajas-Gutiérrez. Síndrome hemofagocítico en la infancia: a propósito de 4 casos. An Esp Pediatr 1998; 49: 289-293.

  14. Janka G, Imashuku S, Elinder G, Schneider M, Hunter JI. Infection and malignancy associated hemophagocytic syndromes. Hematol Oncol Clin North Am 1998; 12: 435-443.

  15. Rohrlich P, Et Garel C. Diagnostic d’une splénomegalie. Edition Techniques-Encycl. Méd Chir (Paris-France), Pédiatrie, 4-080-C-10, 1995: 5.

  16. Eakle JF, Bressoud PF. Hemophagocytic syndrome following an Epstein-Barr virus infection: a case report and literature review. Ky Med Assoc 2000; 98: 161-165.

  17. García-Díaz JD, Guijarro-Herraiz C, Abarca-Costalago M, Guerra-Vales JM. Histiocitosis reactiva, hemofagocitosis histiocítica y síndromes hemofagocíticos. Rev Clin Esp 1990; 186: 193-194.

  18. Osugi Y, Hara J, Tagawa S, Takai K, Hosoi G, Matsuda Y et al. Cytokine production regulating Th1 and Th2 cytokines in hemophagocytic lymphohistiocytosis. Blood 1997; 89: 4100-4103.

  19. Wong KF, Chan JFC, Ha SY, Wong HW. Reactive hemophagocytic syndrome in childhood-frequent occurrence of atypical mononuclear cells. Hematol Oncol 1994; 12: 67-74.

  20. Gogusev J, Nezelof C. Malignant histiocitosis. Hematol Oncol Clin North Am 1998; 12: 445-459.

  21. Duval M, Fenneteau O, Doireau V, Faye A, Emilie D, Yotnda P et al. Intermittent hemophagocytic lymphohistiocytosis as a regular feature of lysinuric protein intolerance. J Pediatr 1999; 134: 236-239.

  22. Henter JI, Aricò M, Egeler RM, Elinder G, Fabra BE, Filipovich AH et al. HLH-94: a treatment protocol for hemophagocytic lymphohistiocytosis. Med Pediatr Oncol 1997; 28: 342-347.

  23. Henter J-I, Elinder G, Öst A. The FHL study group of the Histiocyte Society. Diagnostic guidelines for hemophagocytic lymphohistiocytosis. Semin Oncol 1991; 18: 29-33.




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