DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20194616

Double trouble: hemophagocytic lymphohistiocytosis in a child with Dengue fever

Minal Wade, Nusrat Inamdar, Baraturam Bhaisara, Anupama Mauskar

Abstract


The epidemic of Dengue is steadily increasing in more than 100 endemic countries. During critical phase, of this disease, shock with organ dysfunction and severe bleeding, can occur. Rarely, it can be further complicated by Hemophagocytic lymphohistiocytosis (HLH), which results from aberrant activation of immune mechanism. HLH is a rare, frequently fatal if untreated condition. It challenging to diagnose because initial symptoms mimic other conditions which are more common. Dengue induced Secondary Hemophagocytic lymphohistiocytosis (HLH), may be responsible for severe form of Dengue with adverse outcomes. We describe a case of an infant, with Dengue fever whose clinical situation deteriorated after being stable during initial phases of illness due to development of Secondary Hemophagocytic lymphohistiocytosis (HLH). The child had persistent fever, anemia, hepatomegaly with deranged coagulation which directed towards diagnosis of Secondary Hemophagocytic lymphohistiocytosis (HLH). We discuss the features of our case and its management to sensitize the clinicians to consider this condition in patients with severe Dengue fever.


Keywords


Bicytopenia, Dengue fever, Ferritin, Infection, Immunoglobulin, Secondary hemophagocytic lymphohistiocytosis

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References


Bhatt S, Gething PW, Brady OJ, Messina JP, Farlow AW, Moyes CL, et al. The global distribution and burden of dengue. Nature. 2013 Apr;496(7446):504-7.

La Rosée P, Horne A, Hines M, von Bahr Greenwood T, Machowicz R, Berliner N, et al. Recommendations for the management of hemophagocytic lymphohistiocytosis in adults. Blood. 2019 Jun 6;133(23):2465-77.

Fisman DN. Hemophagocytic syndromes and infection. Emerg Infect Dis. 2000 Nov-Dec;6(6):601-8.

Rosado FGN, Kim AS. Hemophagocytic Lymphohistiocytosis: An Update on Diagnosis and Pathogenesis. Am J Clin Pathol. 2013 June;139(6):713-27.

Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, et al. HLH‐2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatric blood and cancer. 2007 Feb;48(2):124-31.

Michael B. Jordan, Carl E. Allen, Sheila Weitzman, Alexandra H. Filipovich, Kenneth L. McClain. How I treat hemophagocytic lymphohistiocytosis. Blood. 2011;118:4041-52.

Ray U, Dutta S, Mondal S, Bandyopadhyay S. Severe dengue due to secondary hemophagocytic lymphohistiocytosis: A case study. ID Cases. 2017 Jan 1;8:50-3.

Ellis EM, Sharp TM, Perez-Padilla J, González L, Poole-Smith BK, Lebo E, et al. Incidence and risk factors for developing dengue-associated hemophagocytic lymphohistiocytosis in Puerto Rico, 2008-2013. PLoS neglected tropical diseases. 2016 Aug 24;10(8):e0004939.

Sharp TM, Gaul L, Muehlenbachs A, Hunsperger E, Bhatnagar J, Lueptow R, et al. Fatal hemophagocytic lymphohistiocytosis associated with locally acquired dengue virus infection-New Mexico and Texas, 2012. MMWR. Morbidity Mort Week Report. 2014 Jan 24;63(3):49-54.

Allen CE, Yu X, Kozinetz CA, McClain KL. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer. 2008 Jun;50(6):1227-35.