Hemophagocytic Lymphohistiocytosis: a complication you do not expect
Key words: Hemophagocytic lymphohistiocytosis, Hypersecretion of cytokines, Failure of the immune system
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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