HAEMOPHAGOCYTIC SYNDROME

Abstract
The histyocitic disorders are a group of illnesses involving benign and malignant proliferation of macrophages in various organs and tissues. The haemophagocytic syndrome (HPS) is a rare disorder of immune regulation due to activation and uncontrolled production of non-malignant cells of the monocyte-macrophage-histiocyte lineage, that causes an overproduction of cytokines and clinical features, like fever, pancitopenia, hepatoesplenomegaly, lymph nodes enlargement, hypetriglyceridaemia, hipofibrinogenemia and coagulopaty. It may be familial or associated with many diseases, including infections, connective tissues disorders and lymphoma, particularly cutaneous T-cell lymphoma. Haemopoietic cells are actively ingested by monocites and macrophages in lymph nodes, bone marrow, liver and spleen. This is a diagnostic criteria for haemophagocytic syndrome. Treatment of haemophagocytic syndrome is very difficult and there are no guidelines of treatment, requires intensive supportive therapy, treatment of coagulopaty disorders, high dose corticosteroids, immunosuppressive therapy, and treatment of associated diseases, although in most cases there is no response to treatment and the evolution is fatal.

Key words
haemophagocytic syndrome, fever, hepatoesplenomegaly

Bibliografía del artículo
1. Seçmeer G, Sakalli H, Gök F, et. al. Fatal cytophagic histiocytic panniculitis. Pediatr Dermatol 2005; 21(3):246-249.
2. Kenneth L, McClain. Up to Date. Haemophagocytic lymphohistiocytosis. 2005.
3. Reiner AP, Spivak JL. Haemophagocytic lymphohistiocytosis: a report of 23 new patients and a review of the literature. Medicine (Baltimore) 1998; 67(6):369-388.
4. Wong KF, Hui PK, Chan JK, Chan YW, Ha SY. The acute lupus haemophagocytic syndrome. Ann Intern Med 1991 ; 114 B(5):387-390.
5. Dhote R, Simon J, Papo T, et al. Reactive haemophagocytic syndrome in adult systemic disease: report of 26 cases and literature review. Arthritis Rheum 2003; 49(5):633-639.
6. Karras A, Thervet E, Legendre C. Haemophagocytic syndrome in renal transplant recipients: report of 17 cases and review of literature. Transplantation 2004; 77(2):238-243.
7. De Kerguenec C, Hillaire S, Molinie V, et al. Hepatic manifestations of haemophagocytic syndrome: a study of 30 cases. Am J Gastroenterol 2001; 96(3):852-857.
8. Esumi N, Ikshima S, Hibi S, Todo S, Imashuku S. High serum ferritin level as a marker of malignant histiocytosis and virus-associated haemophagocytic syndrome. Cancer 1988; 61(10):2071-2076.
9. Huilgol SC, Fenton D, Pambakian H, Cerio R, McGibbon DM, Black MM. Fatal cytophagic paniculitis and haemophagocytic syndrome. Clinical & Experimental Dermatology 1998; 23:51-55.
10. Cheng FY, Tsui WMS, Yeung WTC, Ip LS, Ng CS. Intravascular Iymphomatosis: a case presenting with encephalomyelitis and reactive haemophagocytic syndrome diagnosed by renal biopsy. Histopathology 1997; 31:552-554.
11. Stephan JL, Kone-Paut 1, Galambrun C, Mouy R, Bader-Meunier B, Prieur AM. Reactive haemophagocytic syndrome in children with inflammatory disorders. A retrospective study of 24 patients. Rheumatology 2001; 40:1285-1292.
12. Cho K, Kim C, Yang S, Kim B, Kim J. Angiocentric T celllymphoma of the skin presentin as inflarnmatory nodules of the leg. Clinical & Expe Pathol 2000; 53:636-638.
13. Kotz EA, Anderson D, Thiers BH. Cutaneous T-cell lymphoma. J Eur Acad Dermatol Venereol 2003; 17:131-137.
14. Artigues Barceló A, Ferragut Reus M, Sánchez C, Amenguall, Matanza 1, Sanz Parras MS. Síndrome hemofagocítico y !infoma cutáneo de células T. An Med Interna (Madrid) 2004; 21:131-134.
15. Mahadeva U, Allport T, Bain B, Chan WK. Haemophagocytic syndrome and histiocytic necrotising lymphadenitis (Kikuchi's disease). J Clin Path 2000; 53(8):636-638.
16. Iwasaki H, Hashimoto K, Takada N, Nakayama T, Ueda T, Nakamura T. Fulminant Rickettsia tsutsugamushi infection associated with haemophagocytic syndrome. The Lancet 1994, 343 (8907):1236.
17. Aouba A, De Bandt N, Aslangul E, Atkhen N, Patri B. Haemophagocytis syndrome in a rheumatoid artrhtitis patient treated with infliximab. Rheum 2003; 42(6):800-802.