Hemophagocytic Lymphohistiocytosis Associated with Cytomegalovirus Infection in an Immunocompetent Infant: A Diagnostic and Therapeutic Challenge!
Tóm tắt
Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome that results from inappropriate activation of the immune system. Many viral agents are known to trigger HLH but cytomegalovirus (CMV) associated HLH is rarely described. We report a case of CMV related HLH in a 3½ month old immunocompetent male infant who presented with fever, respiratory distress and hepatosplenomegaly. He had fulminant sepsis like course in the hospital as he continued to have hectic fever spikes, progressive pneumonia, increasing hepatosplenomegaly and multiple episodes of generalized convulsions. Investigations revealed bicytopenia, biochemical hepatitis, hyperferritinemia and hypofibrinogenemia. CMV IgM serology was reactive in both infant and mother. Diagnosis of CMV-HLH was made as per HLH 2004 diagnostic protocol. Infant was successfully treated with intravenous ganciclovir along with dexamethasone and etoposide.
Tài liệu tham khảo
Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku et al (2007) HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 48(2):124–131
Jordan MB, Allen CE, Weitzman S, Filipovich AH, McClain KL (2011) How I treat hemophagocytic lymphohistiocytosis. Blood 118(15):4041–4052
Ansuini V, Rigante D, Esposito S (2013) Debate around infection-dependent hemophagocytic syndrome in paediatrics. BMC Infect Dis 13(1):15
Imashuku S, Ueda I, Ishii E, Teramura T, Mori K, Morimoto A, Sako M, Ishii E (2005) Occurrence of haemophagocytic lymphohistiocytosis at less than 1 year of age: analysis of 96 patients. Eur J Pediatr 164(5):315–319
Avila-Agüero ML, Paris MM, Alfaro W, Avila-Agüero CR, Faingezicht I (2003) Ganciclovir therapy in cytomegalovirus (CMV) infection in immunocompetent pediatric patients. Int J Infect Dis 7(4):278–281
Palazzi D, McClain K, Kaplan S (2003) Hemophagocytic syndrome in children: an important diagnostic consideration in fever of unknown origin. Clin Infect Dis 36(3):306–312
Gupta A, Sen R, Batra C, Banerjee D, Gupta A, Jain M (2011) Hemophagocytic syndrome secondary to cytomegalovirus infection in an infant. J Cytol 28(1):36
Oloomi Z, Heshmat M (2006) Cytomegalovirus infection-associated hemophagocytic syndrome. Arch Iran Med 9:284–287
Hisham M, Remadevi S, Shabina MB, Jayakrishnan MP (2013) Cytomegalovirus infection associated hemophagocytic syndrome. J Acad Clin Microbiol 15(1):22
Knorr B, Kessler U, Pöschl J, Fickenscher H, Linderkamp O (2007) A haemophagocytic lymphohistiocytosis (HLH)-like picture following breast milk transmitted cytomegalovirus infection in a preterm infant. Scand J Infect Dis 39(2):173–176