Bourlon CR, Sandia ZMA

Language: Spanish
References: 11
Page: 481-485
PDF size: 82.22 Kb.

ABSTRACT

The hemophagocytic syndrome is a disorder caractherized by a non malignant, generalized proliferation of histiocytes, with an increased hemophagocytic activity. This entity has been related to a defect in perforin-mediated cytotoxicity. The incidence is not well known since many cases are misdiagnosed. Lymphohistiocytosis can be either primary (hereditary or genetically determined) or secondary (acquired). It is associated to viral (EBV, CMV, HSV), bacterial (Mycobacterium, Brucella, Haemophilus, Staphylococcus), mycotic or parasitic infections, neoplasia, collagen-related diseases, primary immunodeficiencies and drugs. Clinical manifestations consist of: fever, esplenomegaly, hepatic dysfunction, lymph node enlargement, rash, neurological changes, anemia and cytopenia, with at least two affected series. The presence of hemophagocytosis in bone marrow, spleen and lymph nodes, without evidence of malignancy, and 2% of cells with hemophagocytic activity are quite enough for the diagnosis. The clinical features can be explained by the prolonged and sustained production of high levels of circulating cytokines, originated from activated histiocytes and T cells. The aims of treatment are: to exclude other causes of the group of symptoms; to identify potential infectious factors that require specific treatment; to establish the duration of the disease, and to collect material for further characterization of genetic factors. The use of agents that suspend the function of the activated macrophages, histiocytes and T cells has been proposed: etoposide, steroids, high doses of IV IgG, cyclosporine A, antithymocyte globulins, as well as support measurements.

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