Sao Paulo Medical Journal
versión impresa ISSN 1516-3180
versión On-line ISSN 1806-9460
SCHETTERT, Isolmar Tadeu et al. Hemophagocytic syndrome: pitfalls in its diagnosis. Sao Paulo Med. J. [online]. 1997, vol.115, n.5, pp.1548-1552. ISSN 1516-3180. http://dx.doi.org/10.1590/S1516-31801997000500007.
The hemophagocytic syndrome (HS) is characterized by a clinical picture of fever, hepatosplenomegaly, lymphadenopathy and peripheral pancytopenia. The morphologic hallmark of this syndrome is the phagocytosis of hematopoietic elements by morphologically normal macrophages. HS is considered rare and may be a primary disease or associated to viral, infection, neoplasias or autoimmune diseases. Treatment is controversial and its evolution is often fatal. Anatomo-pathological evaluation shows the phenomenon of hemophagocytosis in several organs, especially the hematopoietic tissues. We describe a case of HS, discuss its possible causes, its clinical and pathologic features, its pathophysiology and therapeutic possibilities.
Palabras clave : Hemophagocytic Syndrome; Lymphohistiocytosis; pancytopenia.