Subject(s)
Chromosome Deletion , Chromosomes, Human, Pair 7 , Genetic Predisposition to Disease/genetics , Myelodysplastic Syndromes/complications , Myelodysplastic Syndromes/genetics , Bone Marrow , Child , Child, Preschool , Hemoglobins , Humans , Male , Myelodysplastic Syndromes/diagnosis , Myelodysplastic Syndromes/physiopathologyABSTRACT
A 5-year-old immunocompetent girl presented with fever, jaundice, hepatosplenomegaly and pancytopenia. The peripheral blood smear demonstrated mixed malaria infection (Plasmodium vivax and Plasmodium falciparum). Fever was persistent despite antimalarials in the absence of any coexisting bacterial or viral infection. Laboratory findings included cytopaenia, hyperbilirubinaemia, hyperferritinaemia, hypertriglyceridaemia, hyponatraemia, deranged partial thromboplastin time, decreasing ESR and megaloblastic changes on bone marrow aspiration. A final diagnosis of haemophagocytic lymphohistiocytosis (HLH) with megaloblastic anaemia associated with severe mixed malaria was made. There was a dramatic response to corticosteroid treatment with improvement in her clinical condition. This report endorses the use of corticosteroids in malaria-associated HLH whenever there is no clinical improvement with antimalarials alone.
Subject(s)
Anemia, Megaloblastic/complications , Coinfection/complications , Lymphohistiocytosis, Hemophagocytic/complications , Malaria, Falciparum/complications , Malaria, Vivax/complications , Adrenal Cortex Hormones/therapeutic use , Anemia, Megaloblastic/pathology , Anti-Inflammatory Agents/therapeutic use , Antimalarials/therapeutic use , Blood Chemical Analysis , Child, Preschool , Coinfection/drug therapy , Coinfection/pathology , Female , Humans , Lymphohistiocytosis, Hemophagocytic/drug therapy , Lymphohistiocytosis, Hemophagocytic/pathology , Malaria, Falciparum/drug therapy , Malaria, Falciparum/pathology , Malaria, Vivax/drug therapy , Malaria, Vivax/pathology , Pancytopenia/diagnosis , Pancytopenia/pathology , Treatment OutcomeABSTRACT
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.
