An acute presentation of haemophagocytic lymphohistiocytosis due to visceral leishmaniasis in a British adult returning traveller

Abstract

A 62-year old British Caucasian woman normally resident in Spain presented with fever and pancytopaenia after returning to the UK. Her symptoms persisted despite broad-spectrum antibiotics, and she gradually became confused, hypotensive and progressively more pancytopaenic. Imaging demonstrated hepatosplenomegaly, and a bone marrow aspirate confirmed a diagnosis of haemophagocytic lymphohistiocytosis (HLH). Bone marrow polymerase chain reaction (PCR) and blood serology were both positive for Leishmania donovani, consistent with visceral leishmaniasis (VL). Following treatment with dexamethasone and amphotericin, she improved clinically and biochemically, and was able to return to Spain. Fever in the returning traveller is a common acute medical presentation. Although HLH and VL are rare diagnoses, both carry a very high mortality rate if undiagnosed and untreated.

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

“…Among travelers, most people were tourists, traveling for work or visiting friends and relatives; only three VL cases were described as military personnel: two deployed in Afghanistan [21] and one in Bangladesh [19]. Refugees were from Somalia (n = 17; [16]) and Ethiopia (n = 1; [17]), while migrants with VL were from Europe and Central Asia (n = 19; [25,36,44,60,208,214,216]), Sub-Saharan Africa (n = 4; [20,28,29]), Latin America (n = 2; [55,199]) and Middle East (n = 2; [10,20]). In one case, a transplacental transmission was described [45].…”

“…Outside the European region, cases were diagnosed in Australia (n = 10, [28,36,45,57,64,211,251]), Kenya (n = 16, [16]), Kuwait (n = 36, [19]), Saudi Arabia (n = 35, [259]) and the USA (n = 8, [21,43,49,51,55,60,215]). The predominance of cases from the Mediterranean region likely reflects more intensive travel to southern Europe, especially in people from countries where most cases were diagnosed such as France (n = 107, [6,18,61,68]), the UK (n = 80, [22,38,50,52,58,201,204,207,210,214,219,252,253,261]) and Germany (n = 68, [8,20,25,27,33,35,37,40,44,46,47,53,56,…”

“…Similarly, laboratory findings of 125 patients translated the natural history of the disease well known in endemic countries [286]: most patients had single or multiple lineage cytopenias, while elevation of liver enzymes and renal failure were less commonly described (Table 2). 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.…”