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2022, Number 1

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Rev Hematol Mex 2022; 23 (1)

Hemophagocytic lymphohistiocytosis treated with HLH-94 protocol: a single center experience in Mexico

Martínez-Villegas O, Canamejoy-Arciniegas MJ, Arias-León J, Sánchez-Jara B, Ortiz-Torres MG, Jiménez-Hernández E, Valdez-Garibay B

Language: Spanish
References: 33
Page: 1-10
PDF size: 328.73 Kb.


ABSTRACT

Objective: To assess the results in response and survival of a treatment scheme (HLH- 94) in a simple institution.
Patients and Method: Retrospective, observational and analytical study including patients who fulfilled five of eight clinical criteria proposed by Histiocyte Society of hemophagocytic lymphohistiocytosis.
Results: A total of 20 patients diagnosed with hemophagocytic lymphohistiocytosis were included, most cases were between 1–6-year group. All cases presented fever, hepatomegaly, and hemophagocytes in bone marrow. Splenomegaly and clinically severe forms predominated in patients with Epstein-Barr virus positive serology; 15 patients were associated with infections, 11 of them with Epstein-Barr virus. At 3.8 years follow-up, 15 patients remained alive, 4 of them had recurrence of disease, treatment began with favorable response and remission. Survival was lower when hemophagocytic lymphohistiocytosis was associated with Epstein-Barr virus.
Conclusions: Epstein-Barr virus plays an important role as responsible for triggering hemophagocytic lymphohistiocytosis; however, it is important to take into account factors such as age, early diagnosis and follow-up with laboratory tests to improve survival with HLH-94 protocol.


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