Uribe-Franco RC, Milán-Salvatierra AI, Román-Bahena DA

Language: Spanish
References: 13
Page: 189-196
PDF size: 257.20 Kb.

ABSTRACT

Background: Hemophagocytic syndrome or macrophage activation syndrome is a disorder of the phagocytic-mononuclear system characterized by a generalized histiocytic proliferation, with hemophagocytosis; it may be primary (familial erythrophagocytic lymphohistiocytosis) or secondary. Primary usually is diagnosed in the first 2 years of life as a result of genetic abnormalities in NK cells, antigen-presenting cells and cytotoxic CD8 T lymphocytes, and secondary, also called reactive, is usually conditioned by an acute disease such as infections (hemophagocytic syndrome associated with infection or IAHS), neoplasms (malignancy associated hemophagocytic syndrome), or autoimmune diseases such as systemic lupus erythematosus and adult Still disease. Clinical presentation is characterized by fever, hepatomegaly, and splenomegaly, and can be associated with rash, lymphadenopathy, and neurological manifestations.
Clinical cases: Three cases of hemophagocytic lymphohistiocytosis are reported with ages from 17 to 64 years.
Conclusions: For the diagnosis of secondary hemophagocytic lymphohistiocytosis, a series of clinical signs based on the Histiocyte Society must be met.

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