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2020, Number 4

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Rev Hematol Mex 2020; 21 (4)

Hemophagocytic syndrome associated to infection due to the Epstein-Barr virus

Leija-Walle R, Osorno-Rodríguez KL, Pérez-Arredondo LA, Gómez-De León A

Language: Spanish
References: 32
Page: 236-246
PDF size: 351.82 Kb.


ABSTRACT

Background: Hemophagocytic lymphohistiocytosis is an entity with high morbidity and mortality, mainly caused by an impairment in the T-lymphocytes and NK-cells function which leads to an uncontrolled immune response with hyperactivation and excessive proliferation of macrophages. All these changes cause the typical clinical features of the syndrome including cytopenias, inflammation of the reticuloendothelial organs (lymph nodes, spleen and liver), fever, rash and malaise. It can be diagnosed in association with malignant, genetic, autoimmune or infectious entities, such as Epstein- Barr virus. Diagnosis is established by clinical and analytical criteria or by genetics. The backbone of treatment is eradicating or controlling the causal factor. The most common treatment protocol is based on dexamethasone, rituximab and etoposide, adding in some cases other agents such as cyclosporine and in the particular case of Epstein-Barr virus, nivolumab. These patients have poor prognosis with an overall survival probability at 6 years of 50-60%.
Clinical case: A 34-year-old female patient diagnosed with hemophagocytic lymphohistiocytosis associated to Epstein-Barr virus.
Conclusions: The diagnosis of hemophagocytic syndrome associated with the Epstein-Barr virus is based on the fulfillment of clinical and laboratory criteria, among documenwhich are fever, splenomegaly, jaundice and the finding of hemophagocytosis in the bone marrow and other tissues in combination with the confirmation of infection.


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