Settembre 2015 - Volume XXXIV - numero 7

Medico e Bambino



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. LA LINFOISTIOCITOSI EMOFAGOCITICA. Medico e Bambino 2015;34(7):431-441


Una febbre alta e persistente con epatosplenomegalia e citopenia

Luciano de Seta1, M. Simona Sabatino1, Federica de Seta2, Paolo Siani3

1UOC di Pediatria e Patologia Neonatale, Ospedale S. Paolo, Napoli
2UOC di Pediatria, AORN Santobono-Pausilipon, Napoli
3Dipartimento di Scienze Mediche Traslazionali, Sezione di Pediatria, Università “Federico II” di Napoli

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High and persistent fever with epatosplenomegaly and cytopenia

Key words: Infectious mononucleosis, Hemophagocytic lymphohistiocytosis, Hemophagocytic syndrome, Epstein-Barr virus

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.

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

. Una febbre alta e persistente con epatosplenomegalia e citopenia. Medico e Bambino 2015;34(7):431-441


Cosa deve sapere il pediatra della linfoistiocitosi emofagocitica attraverso i casi degli specializzandi

Sara Lega1, Fabiola Dell’Acqua2, Marta Minute1, Francesca Vendemini3, Chiara Cattelani4

1Università di Trieste; 2Università degli Studi di Milano-Bicocca; 3Università di Bologna; 4Università di Modena e Reggio Emilia

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What tha paediatrician should know on hemophagocytic lymphohistiocytosis through the cases managed by residents

Key words: Hemophagocytic lymphohistiocytosis, Macrophage activation syndrome, Leishmaniasis

Hemophagocytic lymphohistiocytosis (HLH) is a potentially life-threatening syndrome caused by severe hypercytokinemia due to a highly stimulated but ineffective immune response. HLH is not a disease by its own but is rather a common final consequence of an inherited or acquired inability of the immune system to cope with a trigger, which in most cases is an infection. HLH in autoinflammatory and autoimmune conditions is usually termed macrophage activation syndrome (MAS) and can be as severe as other forms of HLH. In absence of treatment HLH has a high mortality rate due to the overwhelming immune activation, and early recognition with prompt immunosuppressive treatment may be life saving. The paper presents four cases of HLH/MAS: each one has a different underlying cause and shows a possible presenting picture of the disease, helping paediatrician to recognize it.

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S. Lega, F. Dell’Acqua, M. Minute, F. Vendemini, C. Cattelani

. Cosa deve sapere il pediatra della linfoistiocitosi emofagocitica attraverso i casi degli specializzandi. Medico e Bambino 2015;34(7):431-441

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