Rivista di formazione e aggiornamento professionale del pediatra e del medico di base, fondata nel 1982. In collaborazione con l'Associazione Culturale Pediatri.
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Cosa deve sapere il pediatra della linfoistiocitosi emofagocitica attraverso i casi degli specializzandi

What tha paediatrician should know on hemophagocytic lymphohistiocytosis through the cases managed by residents

S. Lega, F. Dell’Acqua, M. Minute, F. Vendemini, C. Cattelani


Settembre 2015 - pagg. 431 -441

Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by severe hypercytokinemia due to a highly stimulated but ineffective immune response. HLH is not a disease by its own but is rather a common final consequence of an inherited or acquired inability of the immune system to cope with a trigger, which in most cases is an infection. HLH in autoinflammatory and autoimmune conditions is usually termed macrophage activation syndrome (MAS) and can be as severe as other forms of HLH. In absence of treatment HLH has a high mortality rate due to the overwhelming immune activation, and early recognition with prompt immunosuppressive treatment may be life saving. The paper presents four cases of HLH/MAS: each one has a different underlying cause and shows a possible presenting picture of the disease, helping paediatrician to recognize it.
Parole chiave
IT Sindrome da attivazione dei macrofagi
EN Macrophage activation syndrome Hemophagocytic lymphohistiocytosis Leishmaniasis
Suggerite dall'AI
IT pediatra pratica
EN pediatrician practice
Classificazione MeSH
Bone Marrow Examination (31) Cytokine Release Syndrome (5) Immunosuppressive Agents (41) Leishmaniasis (13) Lymphohistiocytosis, Hemophagocytic (21) Macrophage Activation Syndrome (11)
Leggi anche
EL I Poster degli specializzandi
Leishmania e linfoistiocitosi emofagocitica (HLH)
Bratta A, Arcuri L, Micalizzi C · 2025
Leishmaniasis/Leishmaniasis, Visceral, Macrophage Activation Syndrome, Lymphohistiocytosis, Hemophagocytic, Bone Marrow Examination
RI Focus
LA LINFOISTIOCITOSI EMOFAGOCITICA
· 2015
Cytokine Release Syndrome, Macrophage Activation Syndrome, Lymphohistiocytosis, Hemophagocytic
RI Percorsi clinici
Quando la linfoistiocitosi emofagocitica è secondaria
Ventresca S, Fabbri E, Bracaglia C, Gasperini P, F · 2022
Leishmaniasis/Leishmaniasis, Visceral, Lymphohistiocytosis, Hemophagocytic, Bone Marrow Examination
EL Caso contributivo
"Amici per la pelle": un caso di leishmaniosi
Fantasia M, Vecchi M, Cavalleri L, Bruni F, Proven · 2025
Leishmaniasis/Leishmaniasis, Visceral, Bone Marrow Examination
RI Editoriali
Malattia di Kawasaki e coronavirus: lo stato attuale delle cose
A. Taddio, A. Tommasini · 2020
Cytokine Release Syndrome, Macrophage Activation Syndrome
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Bibliografia

di riferimento • de Seta L, Sabatino MS, de Seta F, Siani P. Una febbre alta e persistente con epatosplenomegalia e citopenia (Focus). Medico e Bambino 2015;34:431-6. • Gagnaire MH, Galambrun C, Stéphan JL. Hemophagocytic syndrome: A misleading complica-tion of visceral leishmaniasis in children - a series of 12 cases. Pediatrics 2000;106 (4):E58. • Janka GE. Hemophagocytic syndromes. Blood Rev 2007;21(5):245-53. • Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL. How I treat hemophagocytic lymphohistiocytosis. Blood 2011;118(15):4041- 52. • Ravelli A, Magni-Manzoni S, Pistorio A, et al. Preliminary diagnostic guidelines for macrophage activation syndrome complicating systemic juvenile idiopathic arthritis. J Pediatr 2005;146(5):598-604. • Sieni E, Cetica V, Aricò M. Linfoistiocitosi emofagocitica: una sfida diagnostica per il pediatra. Medico e Bambino 2012;31:21-9.

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