Hemophagocytic lymphohistiocytosis
Keywords:
Leishmaniasis, hemophagocytic lymphohistiocytosis, amphotericin BAbstract
Leishmaniasis is a tropical and subtropical disease that affects people, especially in Southeast Asia, East Africa, Latin America, and the Mediterranean basin, with an incidence of 2 million new cases by year, with 500,000 being visceral leishmaniasis.
One of the more severe and rare complications of visceral leishmaniasis is hemophagocytic lymphohistiocytosis. To describe the clinical characteristics of this syndrome, we performed a literature review based on the case reports published in PubMed.
Twenty-five cases were included; the majority of them (52%) were under 2 years of age. All cases presented splenomegaly and 84% hepatomegaly. Cytopenias were described in all patients: 100% presented with thrombocytopenia, 96% anemia, and 84% leukopenia or neutropenia. Hypertriglyceridemia and hypofibrinogenemia were found in 68% and 32% of patients, respectively, and hyperferritinemia in 80%. Additionally, the evidence of hemophagocytosis was documented in 21 patients (84%), with direct visualization of Leishmania in 92% of cases.
All patients were treated against Leishmania: 80% liposomal amphotericin B. Regarding the treatment for hemophagocytic lymphohistiocytosis; corticosteroid were used in 36%, endovenous immunoglobulin in 28%, cyclosporine in 28% and etoposide in 16%
The complications reported included gastrointestinal hemorrhage (8%), disseminated intravascular coagulation (8%), hemolytic immune anemia (12%), multiple-organ dysfunction/septic shock (12%), petechial rash (16%), and four patients deceased. variables such fever (p=0.031), hemoglobin (p=0.031), platelet count (p=0.0048), and ferritin (p=0.0072) were associated with mortality
In conclusion, hemophagocytic syndrome during visceral leishmaniasis is a rare condition that mainly affects pediatric patients, with good outcomes with the therapy based on liposomal amphotericin B.
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