English 
Italian Un soggetto con pancitopenia e febbre persistente
A subject with pancytopenia and persistent fever
AUTORI
UOC Ematologia, Presidio Ospedaliero “A. Tortora” Pagani, Salerno
Questo lavoro è stato in parte presentato al 52° Congresso Nazionale SIBioC, 6-8 Ottobre 2020, Virtual Edition, nella Sessione Casi Clinici
ABSTRACT
A subject with pancytopenia and persistent fever
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.