Montiel LL, Posadas CJG, Domínguez CG

Language: Spanish
References: 24
Page: 75-81
PDF size: 65.53 Kb.

ABSTRACT

The hemophagocytic syndrome (hemophagocytic lymphohistiocytosis) is a rare entity which is most commonly seen in children, but can be found at any age. It consists of a combination of cytologically benign proliferation of activated macrophages in bone marrow, spleen, liver or lymph nodes in association with fever, cytopenia, splenomegaly, and hypertriglyceridemia or hypofibrinogenemia. This can be primary (which is caused by genetic disturbances like PRF1 gene mutations), or secondary (triggered by viral, bacterial, fungal or parasitic infections or in association with malignancies). The diagnosis of the hemophagocytic syndrome is complex and it is practically impossible to differentiate primary from secondary syndrome. In 1991 the Histiocytosis Society developed the diagnostic guidelines for hemophagocytic lymphohistiocytosis. Therapy for this syndrome has only three possibilities: chemotherapy, gamma-globulin therapy, or allogenic bone marrow transplantation. Finally, the prognosis in the primary variant is poor, and is little better for the secondary one, depending on the presence or absence of an underlying cause.

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