Hemophagocytic Lymphohistiocytosis Associated with Cytomegalovirus Infection in an Immunocompetent Infant: A Diagnostic and Therapeutic Challenge!

Abstract

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 3 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.

Severe complication during remission of Crohn’s disease: hemophagocytic lymphohistiocytosis due to acute cytomegalovirus infection

“…It can be divided into 2 forms. The primary or hereditary form is linked with certain gene mutations coding for pathways in cytotoxic T-cells and natural killer cells and affects children more commonly [16,17]. The secondary form is acquired and associated with infections, malignant tumors, autoimmune diseases, or immunosuppressive treatments [1,2].…”

Hemophagocytic lymphohistiocytosis in a patient with Sjögren’s syndrome: case report and review

“…Some examples of bacterial etiologies are mycobacteria, Brucella, Rickettsia, Hemophilus influenzae and Serratia sp. The most common parasites are Leishmania and Toxoplasma [22][23][24][25][26][27][28][29][30].…”

One Hemophagocytic Syndrome Relating to Adult Epstein-Barr Virus Infection and a Literature Review

“…HLH includes infection-associated hemophagocytic syndrome (IAHS) and malignant tumor hemophagocytic syndrome (MAHS). Most IAHS cases are caused by viruses such as EB, cytomegaloviruses, hepatitis E, and hepatitis B, and bacteria, such as Escherichia coli, Streptococcus pneumoniae, Salmonella typhi, and a serotype of S. enterica [7] [8]. Most MAHS cases are caused by malignant lymphoma.…”