Enterovirus-associated hemophagocytic syndrome in children with malignancy: report of three cases and review of the literature
| Autor: | Athanassios Tsakris, Maria Theodoridou, Maria Moschovi, Nicholas Spanakis, P Kalabalikis, Fotini Tzortzatou-Stathopoulou, Katerina Katsibardi |
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| Rok vydání: | 2007 |
| Předmět: |
Male
Pediatrics medicine.medical_specialty Adolescent Lymphoma Multiple Organ Failure Fulminant Hepatosplenomegaly medicine.disease_cause Malignancy Lymphohistiocytosis Hemophagocytic chemistry.chemical_compound Fatal Outcome Biopsy Enterovirus Infections Humans Medicine Child medicine.diagnostic_test business.industry Pleconaril medicine.disease Pancytopenia Surgery chemistry Pediatrics Perinatology and Child Health Enterovirus Female medicine.symptom Differential diagnosis business |
| Zdroj: | European Journal of Pediatrics. 167:97-102 |
| ISSN: | 1432-1076 0340-6199 |
| Popis: | Enteroviruses can cause severe manifestations in children with malignancy. Infection-associated hemophagocytic syndrome (IAHS) due to enterovirus is a rare entity in children. Patients with malignancy and IAHS due to enterovirus were retrospectively evaluated at the University of Athens' Hematology-Oncology pediatric unit within a 6-year period (2000-2006). IAHS occurred in three cases among 56 patients with documented enteroviral infection. The diagnosis of IAHS was confirmed by bone marrow aspiration and biopsy. Nested reverse transcriptase-polymerase chain reaction (RT-PCR), sequencing of the amplified alleles, and immunohistochemistry were performed to document the presence of enterovirus. The type of enterovirus was specified by indirect immunofluorescence assay. At the early phase of the disease, patients presented mild, non-specific viral symptoms, persistent unexplained fever, and pancytopenia. At the late phase, patients had more severe manifestations, such as persistent high fever, diarrhea, weight loss, hepatosplenomegaly, and hepatic dysfunction. The therapeutic approach consisted of supportive care, administration of immunoglobulin (400 mg/kg or 2 g/kg), and pleconaril. All patients had fatal outcome; two patients succumbed to multiorgan failure (MOF), while one patient succumbed to ventricular fibrillation. IAHS usually has fulminant course and leads to severe and life-threatening complications, such as liver failure and MOF. IAHS should always be included in the differential diagnosis of viral syndrome or unexplained fever. The therapeutic approach for IAHS should be administered as early as possible, before the progression to irreversible tissue damage. Early therapeutic intervention involving high doses of immunoglobulin might be beneficial for the patient's outcome. |
| Databáze: | OpenAIRE |
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