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Editor-in-chief
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
Kjell Grankvist Sweden
Hans Jacobs The Netherlands
Eric Kilpatrick UK
Magdalena Krintus Poland
Giuseppe Lippi Italy
Mario Plebani Italy
Sverre Sandberg Norway
Ana-Maria Simundic Croatia
Tommaso Trenti Italy
Cas Weykamp The Netherlands
Maria Willrich USA
Paul Yip Canada
Publisher
Biomedia srl
Via L. Temolo 4, 20126 Milano
Responsible Editor
Giuseppe Agosta
Editorial Secretary
Chiara Riva
Biomedia srl
Via L. Temolo 4, 20126 Milano
Tel. 0245498282
email: biochimica.clinica@sibioc.it
<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 “hand-mirror” 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 of clinical-anamnestic, instrumental and laboratory information are essential for diagnosis and therapeutic strategy.</p>
Biochimica Clinica ; 45(4) e30 Casi clinici - Case report
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