Rivista di formazione e aggiornamento professionale del pediatra e del medico di base, fondata nel 1982. In collaborazione con l'Associazione Culturale Pediatri.
Login Abbonamenti Pubblicazioni Carrello Registrazione Perché registrarsi? Contatti

Focus

Una febbre alta e persistente con epatosplenomegalia e citopenia

High and persistent fever with epatosplenomegaly and cytopenia

L. de Seta, M.S. Sabatino, F. de Seta, P. Siani


Settembre 2015 - pagg. 431 -441

Abstract
The case described is that of an 11-year-old child presenting with high fever, pharyngotonsillar exudate, submandibular strangles and hepatosplenomegaly. The elevated number of VCA IgM antibodies led to a diagnosis of infectious mononucleosis. The persistence of the fever for more than two weeks associated with thrombocytopenia and anaemia and the serious general conditions of the patient have induced the provider to perform a fine-needle bone marrow aspiration that has revealed the presence of macrophages in active hemophagocytosis. This condition, associated with other symptoms, has resulted in the diagnosis of a secondary hemophagocytic lymphohistiocytosis, a rare and serious complication of infectious mononucleosis.
Contenuto riservato

Per leggere l'articolo completo è necessario effettuare il login.

Non sei ancora registrato? Registrati

Bibliografia

1. Henter JI, Horne A, Aricò M, et al. HLH- 2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007;48:124-31. 2. Sieni E, Cetica V, Aricò M. Linfoistiocitosi emofagocitica: una sfida diagnostica per il pediatra. Medico e Bambino 2012;31:21-9. 3. Huang SC, Chen JS, Cheng CN, et al. Hypoalbuminaemia is an independent predictor for hemophagocytic lymphohistiocytosis in childhood Epstein-Barr virus-associated infectious mononucleosis. Eur J Haematol 2012;89:417-22. 4. Henter JI, Samuelsson-Horne A, Aricò M, et al. Histocyte Society. Treatment of hemophagocytic lymphohistiocytosis with HLH- 94 immunochemotherapy and bone marrow transplantation. Blood 2002;100:2367-73. 5. Fisman DN. Hemophagocytic syndromes and infection. Emerg Infect Dis 2000;6:601-8. 6. Perez N, Virelizier JL, Arenzana-Seisdedos, et al. Impaired natural killer activity in lymphohistiocytosis syndrome. J Pediatr 1984; 104:569-73. 7. Jordan MB, Allen E, Weitzman S, et al. How I treat hemophagocytic lymphohistiocytosis. Blood 2011;118:4041-52. 8. Jaffe ES, Costa J, Fauci AS, et al. Malignant lymphoma and erythrophagocytosis simulating malignant histiocytosis. Blood 2002;100: 2367-73. 9. Ravelli A, Magni-Manzoni S, Pistorio A, et al. Preliminary diagnostic guideline for macrophage activation syndrome complicating sistemic juvenile idiopathic arthritis. J Pediatr 2005;146:598-604. 10. Henter JI, Ehrnst A, Andersson J, et al. Familial hemophagocytic lymphohistiocytosis and viral infections. Acta Paediatr 1993; 82:369-72. 11. Davì S, Consolaro A, Guseinova D, et al. An international consensus survey of diagnostic criteria for macrophage activation syndrome in systemic juvenile idiopathic arthritis. J Rheumatol 2011;38:764-8. 12. Hazen MM, Woodward AL, Hofmann I, et al. Mutations of the hemophagocytic lymphohistiocytosis- associated gene UNC13D in a patient with systemic juvenile idiopathic arthritis. Arthritis Rheum 2008;58:567-7. 13. Villanueva J, Lee S, Giannini EH, et al. Natura killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome. Arthritis Res Ther 2005;7:R30-7. 14. Ravelli A. Macrophage activation syndrome. Curr Opin Rheumatol 2002;14:548-52. 15. Lega S, Dell’Acqua F, Minute M, Vendemini F, Cattelani C. Cosa deve sapere il pediatra della linfoistiocitosi emofagocitica attraverso i casi degli specializzandi (Focus). Medico e Bambino 2015;34:437-41.

Corrispondenza: ludeseta@tin.it