Neonatal infectious hemophagocytic lymphohistiocytosis
Keywords:
Hemophagocytic lymphohistiocytosis, neonatal period, life-threatening, diagnosis, therapyAbstract
Hemophagocytic lymphohistiocytosis (HLH) is a disorder of the mononuclear phagocytic system and a severe, life-threatening syndrome due to an excessive immune activation. This hyperinflammatory condition is often triggered by a variety of agents or events, mostly genetic or infectious; and its scarcity in the neonatal period often delay diagnosis and adequate management. We report a peculiar neonatal case with multivisceral involvement, managed promptly and aggressively. Discussing the most important relevant points in both diagnosis and therapy of this peculiar disorder is highlighted in purpose of increasing awareness and enhancing early determinant recognition.
References
McClain K. Clinical features and diagnosis of hemophagocytic lymphohistiocytosis. Up to Date: Topic. 87499, 2014.
Suzuki N, Morimoto A, Ohga S, Kudo K, Ishida Y, Ishii E, HLH/LCH Committee of the Japanese Society of Pediatric Hematology. Characteristics of hemophagocytic lymphohistiocytosis in neonates: a nationwide survey in Japan. J Pediatr 155:235-238, 2009.
Bousfiha A et al. The 2015 IUIS phenotypic classification for primary immunodeficiencies. J Clin Immunol 35:727-738, 2015.
Tanoshima R, Takahashi H, Hokosaki T, Yamaguchi K, Goto S, Kai S. Hemophagocytic lymphohistiocytosis in very young infants. Pediat Blood Cancer 52:137-139, 2009.
Janka GE. Hemophagocytic syndromes. Blood Rev 21:245-253, 2007.
Rouphael NG et al. Infections associated with haemophagocytic syndrome. Lancet Infect Dis 7:814-22, 2007.
Freeman HR, Ramanan AV. Review of haemophagocytic lymphohistiocytosis. Arch Dis Child 96:688-693, 2011.
Kapoor S. Distinguishing hemophagocytic lymphohistiocytosis from hemochromatosis in patients with hyperferritinemia. Pediatr Blood Cancer. 50:1287- 1288, 2008.
Allen CE, Yu X, Kozinetz CA, McClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 50:1227-1235, 2008.
Mahlaoui N, Ouachée-Chardin M, deSaint Basile G et al. Immunotherapy of familial hemophagocytic lymphohistiocytosis with antithymocyte globulins: a single-center retrospective report of 38 patients. Pediatrics 120:e622-628, 2007.
Ansuini V, Rigante D, Esposito S. Debate around infection-dependent hemophagocytic syndrome in paediatrics. BMC Infect Dis 13:15, 2013.
Pramanik S, Pal P, Das PK, Chakrabarty S, Bhattacharya A, Banerjee S. Reactive haemophagocytic lymphohistiocytosis. Indian J Pediatr. 76: 643-645, 2009.
Gonzalez F, Vincent F, Cohen Y. Syndrome d’activation macrophagique d’origine infectieuse: étiologies et prise en charge. (Article in French). Réanimation 18:284-290, 2009.
Downloads
Published
Issue
Section
License
Authors who publish with the American Journal of Experimental and Clinical Resaerch (AJECR) agree to the following terms:
- Authors retain copyright and grant the journal right of first publication with the work simultaneously licensed under a Creative Commons Attribution License (CC BY-NC-ND) that allows others to share the work with an acknowledgement of the work's authorship and initial publication in this journal.
- Authors are able to enter into separate, additional contractual arrangements for the non-exclusive distribution of the journal's published version of the work (e.g., post it to an institutional repository or publish it in a book), with an acknowledgement of its initial publication in this journal.
- Authors are permitted and encouraged to post their work online (e.g., in institutional repositories or on their website) prior to and during the submission process, as it can lead to productive exchanges, as well as earlier and greater citation of published work (See The Effect of Open Access).

