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

Abstract

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.%

Severe Visceral Leishmaniasis Associated With COVID-19 and Complicated by Hemophagocytic Syndrome

“…HPS associated with VL is well known. 1 The uniqueness of this case report is the pulmonary involvement of COVID-19, which increased its severity. Further, liposomal amphotericin B treatment was extended to 14 doses because of persistent clinical and laboratory signs of VL and symptoms of COVID-19; however, the Brazilian protocol prescribes 7 doses.…”

“…if neglected the disease could become systemic. 1 Coronavirus disease 2019 (COVID-19) is a viral disease that could complicate the management of several infectious diseases in different regions worldwide. 2 Managing severe pediatric VL associated with COVID-19 and complicated by hemophagocytic syndrome (HPS) as well as finding an efficient therapeutic protocol to reduce mortality is a challenge.…”

“…was originally classified under the genus Bacillus until 1993, when they were segregated based on phylogenetic analysis of 16S rRNA. 1 A few cases of Paenibacillus spp. infections have been described in pediatrics.…”

Non-Endemic Leishmaniases Reported Globally in Humans between 2000 and 2021—A Comprehensive Review

“…In 22 patients, laboratory findings were consistent with hemophagocytic lymphohistiocytosis (HLH) [17,26,44,49,50,52,59,68,206,214]. Although the association between leishmaniasis and HLH seems to be a rare finding in the pediatric population of endemic areas [288], cases occurring in infants have been reported in imported cases [44], so in children under 2 years of age traveling to endemic areas, leishmaniasis should be included in the differential diagnosis of secondary HLH.…”

A 10-Year Retrospective Study on Pediatric Visceral Leishmaniasis in a European Endemic Area: Diagnostic and Short-Course Therapeutic Strategies

“…An abdominal ultrasound is part of the initial diagnostic work-up in patients with suspected VL, identifying splenomegaly, sometimes with distinctive multifocal hypoechoic nodules in pediatric patients [ 14 ]. A differential diagnosis is critical to distinguish VL from other illnesses, such as typhoid fever, tuberculosis, brucellosis, malaria, or other hematologic disorders [ 15 ], and to ensure proper treatment.…”