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Case Reports
. 2018 Nov 27;15(12):2672.
doi: 10.3390/ijerph15122672.

Complex Investigation of a Pediatric Haematological Case: Haemophagocytic Syndrome Associated with Visceral Leishmaniasis and Epstein⁻Barr (EBV) Co-Infection

Giorgia Tascini  1 , Lucia Lanciotti  2 , Lisa Sebastiani  3 , Alessandra Paglino  4 , Susanna Esposito  5
Affiliations
Case Reports

Complex Investigation of a Pediatric Haematological Case: Haemophagocytic Syndrome Associated with Visceral Leishmaniasis and Epstein⁻Barr (EBV) Co-Infection

Giorgia Tascini et al. Int J Environ Res Public Health. .

Abstract

Background: Visceral leishmaniasis (VL) is an anthropozoonosis caused by an intracellular parasite belonging to the genus Leishmania. In the Mediterranean region, L. donovani and L. infantum are responsible for VL and dogs are the main reservoir. Haemophagocytic lymphohistiocytosis (HLH) represents a complication of VL and consists of unrestrained activation and proliferation of lymphocytes and macrophages, leading to uncontrolled immune activation. Haemophagocytic lymphohistiocytosis may also develop during viral infection, and Epstein⁻Barr virus (EBV) infection is one of the main HLH causes. Macrophage haemophagocytosis in the bone marrow aspirate is pathognomonic.

Case presentation: The case involves a 19-month-old male infant presenting with a high persistent fever with a fluctuating pattern, pancytopaenia, hepatosplenomegaly, and a high triglyceride level. Initial investigations showed an EBV infection. Considering the persistent signs and symptoms, bone marrow aspiration was performed and confirmed the suspicion of HLH. In addition, the presence of Leishmania infection was shown. The patient was treated with liposomal amphotericin B and had complete resolution of his symptoms.

Conclusion: Diagnosis of VL represents a demanding challenge in endemic and non-endemic areas. Our case demonstrates that leishmaniasis should always be considered in the differential diagnosis in patients presenting with hepatosplenomegaly and cytopaenia with a persistent fever, even in cases of infectious mononucleosis. Moreover, the execution of bone marrow aspiration should not be delayed in order to diagnose and treat at an early stage the potential occurrence of VL, especially if complicated with HLH.

Keywords: Epstein–Barr virus; Leishmania; haemophagocytic syndrome; mononucleosis; visceral leishmaniasis.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Body temperature and laboratory tests during the second hospitalization (nv: normal value; ac: absolute count; A: acetaminophen). CRP: C reactive protein; ESR: erythrocyte sedimentation rate; Hb: hemoglobin; L%: percentage of lymphocytes; M%: percentage of monocytes; MCV: mean corpuscular volume; N%: percentage of neutrophils; PLT: platelets; RBC: red blood cells; WBC: white blood cells.
Figure 2
Figure 2
Bone marrow aspiration: red blood cells phagocytized by a macrophage and Leishmaniae amastigotes (blue arrows).
Figure 3
Figure 3
On day 22 of fever, initiation of therapy with Amphotericin B and gradual normalization of laboratory tests.
See this image and copyright information in PMC

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