Hemophagocytic Lymphohistiocytosis in Pediatric Patients: A Review
Contino A, Trombatore G, Timeus F
1Specialization School in Pediatrics, University of Turin, Italy
2Division of Pediatric Onco-Hematology, Catholic University of Rome, Italy
3Pediatric Onco-Hematology, Regina Margherita Children Hospital, Turin, Italy
Correspondence Address :
piazza Polonia, 94-10126 Torino-
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Citation: Contino A, Trombatore G, Timeus F (2018). Hemophagocytic Lymphohistiocytosis in Pediatric Patients: A Review
Copyright: 2018 Fabio Timeus, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease due to a highly stimulated but ineffective immune response, with uncontrolled proliferation of activated lymphocytes and macrophages that may lead to multisystem failure. HLH can be caused by underlying genetic diseases (primary HLH or familial hemophagocytic lymphohistiocytosis, FHL), or it can be acquired, resulting after strong immunologic activation, such as that which can occur with infections, autoimmune/rheumatologic diseases, malignancies or others conditions. FHL is more frequent in children. Clinical signs include prolonged highgrade fever, multi-organ involvement including cytopenias, hepatosplenomegaly and liver dysfunction, skin rash, coagulopathy and variable neurologic symptoms. Diagnosis of HLH requires high level of suspicion and applying the diagnostic criteria as accepted by HLH Society. The aim of therapy is the suppression of the hyperactive T cells and histiocytes using chemotherapy and immunosuppressive drugs and, in FHL, if donor and resources are available, subsequent hematopoietic stem cell transplantation (HSCT). Biologic therapy is a promising therapeutic approach.
Keywords: Hemophagocytic lymphohistiocytosis, HLH, FLH