Hemophagocytic lymphohistiocytosis

Authors

  • David Israel Garrido Universidad de la República
  • Michelle Fuseau Universidad de la República
  • Santiago Garrido Hospital General del IESS, Ibarra
  • Santiago Celi Clínica TOA

Keywords:

Leishmaniasis, hemophagocytic lymphohistiocytosis, amphotericin B

Abstract

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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Author Biographies

David Israel Garrido, Universidad de la República

Posgrado de hematología

Michelle Fuseau, Universidad de la República

Posgrado de reumatología

Published

2020-12-31

How to Cite

1.
Garrido DI, Fuseau M, Garrido S, Celi S. Hemophagocytic lymphohistiocytosis. Rev Cuba Med Tropical [Internet]. 2020 Dec. 31 [cited 2025 Apr. 2];72(3). Available from: https://revmedtropical.sld.cu/index.php/medtropical/article/view/524

Issue

Section

Artículos de revisión