ObjectiveTo analyze the epidemic characters, pathogenesis, clinical manifestations, laboratory data, therapeutic response, prognosis and the risk factors in patients with gram-negative bacilli septicemia-associated hemophagocytic lymphohistiocytosis(HLH).

Methods A retrospective study was performed on 32 patients with gram-negative bacilli septicemia-associated HLH who were admitted to The First Affiliated Hospital of Nanjing Medical University from January 2010 to December 2015.

Results 32 out of 1430 patients (2.39%) with gram-negative bacilli septicemia were confirmed to have HLH. The incidence of HLH in gram-negative bacilli subspecies were: Pseudomonas aeruginosa, 1.55%; Klebsiella pneumonia, 2.25%; Escherichia coli, 2.26%; Acinetobacter baumannii, 3.79%; Enterobacter cloacae 4.35%; Brucella abortus, 14.29%; Salmonella choleraesuis, 50%, respectively. There were no statistical differences among the incidence of the pathogens. And the incidence of gram-negative bacilli septicemia-associated HLH was not related to age or gender. The main clinical manifestations included persistent fever (100%) and shock (37.50%). The most prominent laboratory abnormalities were hemocytopenia, abnormal hepatic function, and elevated ferritin. 10 patients have been performed bone marrow cytology and all of them had hemophagocytosis in bone marrow samples. Among 32 patients, 18 patients survived (56.25%) and 14 patients died (43.75%). Treatment with antibiotic or antibiotic plus corticosteroid (including supportive treatments) was effective in our study. Univariate logistic regression analysis: hypofibrinogenemia was a risk factor for mortality prediction (OR 8.750,95%CI 1.756-43.600).

Conclusion The incidence of gram-negative bacilli septicemia-associated HLH was low (2.39%), but it had a high mortality (43.75%). Treatment with antibiotic and antibiotic plus corticosteroid (including supportive treatments) were recommended for this disease. Hypofibrinogenemia was a adverse prognostic factor for therapy.

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Disclosures

No relevant conflicts of interest to declare.

Topics:
hemophagocytic lymphohistiocytosis, septicemia, antibiotics, adrenal corticosteroids, glucocorticoids, hypofibrinogenemia, mineralocorticoids, signs and symptoms, supportive care, bone marrow specimen

Author notes

*

Asterisk with author names denotes non-ASH members.

© 2017 by The American Society of Hematology
2017
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