Hepatic sinusoidal hemophagocytosis with and without hemophagocytic lymphohistiocytosis.

De Gottardi, Jacqueline; Montani, Matteo; Angelillo-Scherrer, Anne; Rovó, Alicia; Berzigotti, Annalisa (2019). Hepatic sinusoidal hemophagocytosis with and without hemophagocytic lymphohistiocytosis. PLoS ONE, 14(12), e0226899. Public Library of Science 10.1371/journal.pone.0226899

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BACKGROUND/PURPOSE Hemophagocytic lymphohistiocytosis (HLH) is a rare, life threatening hyperinflammatory syndrome. Sinusoidal hemophagocytosis is occasionally observed on liver biopsy in patients who do not have clinical suspicion of HLH. We aimed at comparing the clinical characteristics and outcomes of patients with signs of hemophagocytosis on liver biopsy meeting and not meeting the HLH diagnostic criteria. METHODS We reviewed the clinical, laboratory features and outcomes of all adult patients consecutively admitted in our center between 08/2011 and 08/2017 presenting with liver histology showing sinusoidal hemophagocytosis and of critically ill patients presenting with severe liver disease in whom hemophagocytosis was histologically confirmed. The characteristics of patients fulfilling and not fulfilling the diagnostic criteria of HLH were compared. RESULTS We identified 12 cases (58% male, median age 61, 75% with a chronic underlying disease) with liver histology showing sinusoidal hemophagocytosis. All had at least some of the clinical features typically associated with HLH. Six were critical ill patients. In 4 cases with insufficient laboratory and clinical criteria, liver biopsy allowed to confirm the HLH diagnosis. Six patients died, of which four met the diagnostic criteria for HLH. Two patients with chronic liver disease died despite not fulfilling the diagnostic criteria of HLH. CONCLUSION Hemophagocytosis on liver biopsy may contribute to confirming a diagnosis of HLH in suspected cases with indeterminate clinical and laboratory findings. Sinusoidal hemophagocytosis in patients with cirrhosis was associated with bad outcome.

Item Type:

Journal Article (Original Article)


04 Faculty of Medicine > Department of Haematology, Oncology, Infectious Diseases, Laboratory Medicine and Hospital Pharmacy (DOLS) > Clinic of Haematology and Central Haematological Laboratory
04 Faculty of Medicine > Service Sector > Institute of Pathology
04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine > Hepatology

UniBE Contributor:

Montani, Matteo; Angelillo, Anne; Rovó, Alicia and Berzigotti, Annalisa


600 Technology > 610 Medicine & health
500 Science > 570 Life sciences; biology




Public Library of Science




Thi Thao Anh Pham

Date Deposited:

08 Jan 2020 14:18

Last Modified:

12 Jan 2020 02:43

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