RESUMO
Introduction: Leishmaniasis is a tropical and subtropical disease highly reported in Southeast Asia, East Africa, Latin America, and the Mediterranean basin, with an incidence of two million new cases by year and 500,000 cases of visceral leishmaniasis. One of the more severe and rare complications of visceral leishmaniasis is hemophagocytic lymphohistiocytosis. Objective: To describe the clinical characteristics of hemophagocytic lymphohistiocytosis associated with visceral leishmaniasis Methods: We performed a literature review based on the case reports indexed in MEDLINE/PubMed. Results: Twenty-five cases were included; 52 percent under two years of age. All cases presented splenomegaly and 84 percent hepatomegaly. Cytopenias were described in all patients: 100 prcent thrombocytopenia, 96 percent anemia, and 84 percent leukopenia or neutropenia. Hypertriglyceridemia and hypofibrinogenemia were found in 68 percent and 32 percent, respectively, and hyperferritinemia in 80 percent. Additionally, hemophagocytosis was documented in 84 percent, with Leishmania detection in 92 percent. All patients were treated against Leishmania: 80% with liposomal amphotericin B. regarding the treatment for hemophagocytic lymphohistiocytosis; corticosteroid were used in 36 percent, endovenous immunoglobulin in 28 percent, cyclosporine in 28 prcent and etoposide in 16 percent The complications reported included gastrointestinal hemorrhage (8 percent), disseminated intravascular coagulation (8 percent), autoimmune hemolytic anemia (12 percent), multiple-organ dysfunction/septic shock (12 prcent), petechial rash (16 percent), and four patients deceased. Variables such as fever (p=0.031), hemoglobin level (p=0.031), platelet count (p=0.0048), and ferritin (p=0.0072) were associated with mortality Conclusions: During visceral leishmaniasis, the hemophagocytic syndrome is a rare condition that mainly affects pediatric patients, but with excellent outcomes using liposomal amphotericin B. However, there is a lack of strong evidence to make a recommendation(AU)
Introducción: La leishmaniasis es una enfermedad tropical y subtropical con una elevada incidencia, dos millones de casos nuevos por año y 500 000 de leishmaniasis visceral. La linfohistiocitosis hemofagocítica es una complicación grave y rara de la leishmaniasis visceral. Objetivo: Describir las características clínicas de la linfohistiocitosis hemofagocítica asociada con leishmaniasis visceral. Métodos: Se realizó una revisión bibliográfica basada en los informes de casos indexados en MEDLINE/PubMed. Se identificaron 34 publicaciones; después de analizarlas en función de los criterios de inclusión se trabajó con 22 trabajos. Resultados: En los trabajos incluidos se informaron 25 casos; el 52 por ciento fueron pacientes menores de 2 años. Todos presentaron esplenomegalia y 84 por ciento hepatomegalia. Se describieron citopenias en todos los pacientes: 100 por ciento trombocitopenia, 96 por ciento anemia y 84 por ciento leucopenia o neutropenia. Se encontró hipertrigliceridemia e hipofibrinogenemia en 68 por ciento y 32 por ciento, respectivamente, e hiperferritinemia en 80 por ciento. Todos los pacientes fueron tratados contra leishmania, 80 por ciento con anfotericina B liposomal. Las complicaciones incluyeron: hemorragia gastrointestinal, coagulación intravascular diseminada, anemia hemolítica autoinmune, falla multiorgánica/shock séptico, erupción petequial y cuatro pacientes fallecieron. Conclusiones: En la leishmaniasis visceral, el síndrome hemofagocítico es una afección poco frecuente que afecta principalmente a pacientes pediátricos. Para el tratamiento, usando la anfotericina B liposomal se obtienen excelentes resultados; sin embargo, la evidencia es insuficiente para hacer una recomendación(AU)