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Maria Stella Graziani

Deputy Director
Martina Zaninotto

Associate Editors
Ferruccio Ceriotti
Davide Giavarina
Bruna Lo Sasso
Giampaolo Merlini
Martina Montagnana
Andrea Mosca
Paola Pezzati
Rossella Tomaiuolo
Matteo Vidali

EIC Assistant
Francesco Busardò

International Advisory Board Khosrow Adeli Canada
Sergio Bernardini Italy
Marcello Ciaccio Italy
Eleftherios Diamandis Canada
Philippe Gillery France
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Hans Jacobs The Netherlands
Eric Kilpatrick UK
Magdalena Krintus Poland
Giuseppe Lippi Italy
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Ana-Maria Simundic Croatia
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Via L. Temolo 4, 20126 Milano

Responsible Editor
Giuseppe Agosta

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Chiara Riva
Biomedia srl
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ISSN print: 0393 – 0564
ISSN digital: 0392- 7091

BC: Articoli scritti da C. Califano

Un soggetto con pancitopenia e febbre persistente
A subject with pancytopenia and persistent fever
<p>We report a case of a 75-years-old female with pancytopenia, fever unresponsive to antibiotic therapy and persistent cough, hepatosplenomegaly and lymphadenopathy. Bone marrow shows pathological cells; some of them showed &ldquo;hand-mirror&rdquo; morphology and numerous hemophagocytosis. Atypical T lymphocytes with CD3+CD5-CD7-CD2++CD8+CD56- immunophenotype are detected by flow-cytometric analysis. Based on this evidence, Hemophagocytic lymphohistiocytosis (HLH) in lymphoproliferative disease is suspected and confirmed by biopsy. HLH is a rare and life-threatening hematologic disease caused by excessive activation of immune system resulting in a systemic hyperinflammation with tissue destruction and multiorgan failure. According to HLH-2004 diagnostic criteria, HLH can be diagnosed in a patient with mutations in HLH-related genes or with at least 5 out of 8 diagnostic criteria (fever, hemophagocytosis, splenomegaly, high ferritin, elevated soluble-CD25, cytopenia, low natural killer cell activity, and hypertryglyceridemia or hypofibrinogenemia). HLH diagnosis is very challenging and integration&nbsp;of clinical-anamnestic, instrumental and laboratory information are essential for diagnosis and therapeutic strategy.</p>
Biochimica Clinica ; 17(1)
Casi clinici - Case report