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Review
. 2021 Sep 1:40:e2020269.
doi: 10.1590/1984-0462/2022/40/2020269. eCollection 2021.

Hemophagocytic lymphohistiocytosis and visceral leishmaniasis in children: a series of cases and literature review

[Article in English, Portuguese]
Natália Fernanda Ferreira Brum  1 , Julia Sampaio Coelho  2 , Laís Silva Carvalho  2 , Matheus Nascimento Otoni Vieira  2 , Aline Almeida Bentes  2 , Ericka Viana Machado Carellos  2 , Lilian Martins Oliveira Diniz  2 , Andrea Lucchesi de Carvalho  3 , Roberta Maia de Castro Romanelli  2
Affiliations
Review

Hemophagocytic lymphohistiocytosis and visceral leishmaniasis in children: a series of cases and literature review

[Article in English, Portuguese]
Natália Fernanda Ferreira Brum et al. Rev Paul Pediatr. .

Abstract
in English, Portuguese

Objective: Hemophagocytic lymphohistiocytosis syndrome (HLHS) is characterized by an immunological hyperactivation of cytotoxic T cells, natural killer cells, and macrophages, leading to the secretion of proinflammatory cytokines. HLHS associated with Visceral Leishmaniasis might be difficult to diagnose once symptoms are similar, resulting in the death of untreated patients. Our aim is to describe a series of cases of Visceral Leishmaniasis with HLHS admitted to a referral hospital for infectious diseases.

Case description: All 115 cases of Visceral Leishmaniasis referred to a referral center for pediatric infectious diseases were reviewed to identify the cases of HLHS. Five cases (4.5%) were confirmed with HLHS and they presented fever, splenomegaly, cytopenia, hypertriglyceridemia or hypofibrinogenemia, increased ferritin and hemophagocytosis in the bone marrow.

Comments: It important to rule out HLHS in children with infectious diseases that do not respond adequately to treatment or in patients with severe symptoms, especially in leishmaniasis endemic areas.

Objetivo:: Linfo-histiocitose hemofagocítica (LHH) é uma síndrome caracterizada pela hiperativação de linfócitos T citotóxicos, células natural killer e macrófagos, levando à secreção de citocinas pró-inflamatórias. Linfo-histiocitose hemofagocítica associada a Leishmaniose Visceral é um quadro de grande desafio para o diagnóstico, considerando que os sintomas são semelhantes, podendo levar ao óbito pacientes não tratados. Nosso objetivo é descrever uma série de casos de Leishmaniose Visceral com LHH admitidos em um hospital de referência para doenças infecciosas.

Descrição do caso:: Todos os 115 casos de Leishmaniose Visceral encaminhados ao centro de referência em doenças infecciosas pediátricas foram revistos para identificar os casos de LHH. Cinco casos (4,5%) foram confirmados com LHH e apresentaram febre, esplenomegalia, citopenia, hipertrigliceridemia ou hipofibrinogenemia, aumento de ferritina e hemafagocitose em medula óssea.

Comentários:: Deve-se suspeitar de LHH em crianças com doenças infecciosas que não respondem adequadamente ao tratamento ou em pacientes com sintomas graves, principalmente em áreas endêmicas da leishmaniose.%

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Conflict of interest statement

Conflict of interests

The authors declare there is no conflict of interests.

References

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