HEMOPHAGOCYTIC SYNDROMES

Hemophagocytic syndromes are the clinicobiological translation of an u nconnected macrophagic activity with hemophagocytosis. Their physiopat hology is related with a deregulation of the T lymphocytes and an exce ssive production of cytokines. Acquired hemophagocytic syndromes are m ostly associated with underlying pathology which they can reveal: immu nodeficiency, infections (mostly of viral origin), hemopathies and can cers, auto-immune diseases. The main clincobiological features are fev er, hepatosplenomegaly and peripheric bicytopeny. In the majority of c ases, the diagnosis is confirmed by a myelogram which shows the presen ce of benign histiocytes, actively phagocyting the hematopoietic cells . The pejorative prognosis of hemophagocytic syndromes (actual mortali ty rate 30 to 45%) requirs an early therapy which associates etiologic al treatment of the underlying affection with pathogenic treatment (pu lse of corticosteroids, immunoglobulins, immunosuppressors, or plasmap heresis).