Clinica Pediatrica, Centro di Emato-Oncologia, IRCCS “Burlo Garofolo”, Trieste
Key words: Histiocytosis, Langerhans cell histiocytosis, Hemophagocytic lymphohistiocytosis, Malignant histiocytosis
Histiocyte disorders in childhood are caracterized by tissue infiltration with cells of the monocyte/ macrophage lineage. Two disorders, Langerhans cell histiocytosis (LCH), and hemophagocytic lymphohistiocytosis (HLH), account for the majority of cases in childhood and are now classified as class one and class two histiocytosis. Althought both LHC and HLH are considered reactive disorders, the prognosis is variable, ranging from self-limited disease to multisystem life-threatening disorder. Malignant histiocytosis is classified as class three, together with acute monocytic leukemia and histiocytic lymphoma. Treatment protocols vary along with the type and severity of the disease and should comprise treatment of the primary infection if an infectious agent is identified.
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