Percorsi clinici
Quando la linfoistiocitosi emofagocitica è secondaria
Secondary Haemophagocytic lymphohistiocytosis: a case of visceral leishmaniasis
Silvia Ventresca1, Elena Fabbri2, Claudia Bracaglia3, Pietro Gasperini2, Beatrice Filippini2, Francesca Libertucci4, Barbara Bigucci4, Roberta Pericoli2, Gianluca Vergine4
1Specializzazione in Pediatria, Università di Ferrara
2SSD di Oncoematologia Pediatrica, Ospedale Infermi, Rimini, ASL della Romagna
3UOC di Reumatologia, Ospedale Pediatrico Bambino Gesù IRCCS, Roma
4UOC di Pediatria, Ospedale Infermi, Rimini, ASL della Romagna
Febbraio 2022 - pagg. 97 -102 | DOI: 10.53126/MEB41097
Abstract
The paper describes the case of a 1-year-old Caucasian boy with a 1-month history of fever and splenomegaly. Laboratory findings showed pancytopenia, hypertriglyceridemia and hyperferritinemia. Secondary haemophagocytic lymphohistiocytosis was suspected on the basis of clinical and laboratory features. Bone marrow aspirate revealed active haemophagocytosis without intra- or extracellular parasites. High levels of antibody title for leishmaniasis confirmed the final diagnosis of haemophagocytic syndrome secondary to visceral leishmaniasis. Amphotericin B treatment was started and showed good clinical response and improvement of laboratory parameters.
Riassunto
Riportiamo il caso di un lattante giunto alla nostra attenzione per febbre, splenomegalia e pancitopenia da circa un mese, associati a ipertrigliceridemia e iperferritinemia. La rachicentesi e la biopsia del midollo osseo hanno escluso natura neoplastica del quadro, evidenziando un quadro di emofagocitosi, per il quale il bambino è stato trattato con glucocorticoidi, immunoglobuline e ciclosporina senza significativo miglioramento clinico. Il pannello sierologico completo è risultato successivamente positivo per Leishmania, causa scatenante della linfoistiocitosi emofagocitica (HLH), per la quale è stato avviato trattamento con amfotericina B liposomiale con ottima risposta clinica.
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Bibliografia
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Corrispondenza: gianluca.vergine@auslromagna.it