Caso contributivo
Linfoistiocitosi emofagocitica: una complicanza che non ti aspetti
Hemophagocytic Lymphohistiocytosis: a complication you do not expect
Antonella Grigoli1, Piero Farruggia1, Giusi Bruno1, Sonia Cannella1, Desirée Caselli2,
Paolo D’Angelo1, Delia Russo1, Antonino Trizzino1, Serena Tropia1, Ottavio Ziino1,
Maurizio Aricò3
1UO di Oncoematologia Pediatrica, ARNAS Ospedali Civico, Di Cristina, Benfratelli, Palermo
2Dipartimento di Oncoematologia Pediatrica, AOU Meyer, Firenze
3Istituto Toscano Tumori, Firenze
Dicembre 2014
Abstract
Haemophagocytic lymphohistiocytosis (HLH) is a clinical syndrome caused by hypersecretion of cytokines secondary to temporary or persistent failure of the immune system. The sporadic form usually occurs in the first months of life as a result of an infection and affects about 1 out of 50.000 children. There is also a familial HLH, related to at least 6 genetic defects. The two forms are not differentiable from clinical and laboratory point of view. The paper reports the case of a child suffering from secondary HLH who was tre-ated at our department. After four years, the parents deci-ded, unexpectedly, to sue the medical staff.
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