Clinical features of hemophagocytic lymphohistiocytosis with abnormal liver function as the initial manifestation

Objective To investigate the clinical and laboratory features of hemophagocytic lymphohistiocytosis ( HLH) with abnormal liver function as the initial manifestation, and to increase the awareness of this disease. Methods A retrospective analysis was performed for the clinical data of 11 patients who attended the hospital due to abnormal liver function from November 2011 to October 2017 and were diagnosed with HLH, including their medical history, etiology, clinical manifestation, laboratory examination results, and prognosis. Results Of all11 patients, 9 ( 81. 8%) had infection as the etiology of HLH, and as for clinical manifestation, 11 ( 100%) had pyrexia and 9 ( 81. 8%) had splenomegaly. Laboratory examination showed varying degrees of abnormal liver function in all patients, among whom 10 ( 90. 9%) had elevated alanine aminotransferase, 11 ( 100%) had elevated aspartate aminotransferase, 10 ( 90. 9%) had an increase in total bilirubin, 9 ( 81. 8%) had a reduction in albumin, 10 ( 90. 9%) had a reduction in cholinesterase, 9 ( 81. 8%) had an increase in gamma-glutamyl transpeptidase, 10 ( 90. 9%) had an increase in alkaline phosphatase, and 5 ( 83. 3%) had an increase in lactate dehydrogenase. Elevated ferritin in the early course of the disease had the highest sensitivity ( 11/11, 100%) in the diagnosis of this disease, followed by triglyceride >3 mmol/L ( 6/7, 85. 7%) . In the early course of the disease, the positive rates of peripheral cytopenia of two or more lineages and reduced fibrinogen were 54. 5% and 45. 4%, respectively. Dynamic monitoring showed that all patients experienced peripheral cytopenia of two or more lineages. Hemophagocytosis was observed in all patients. NK cell activity and soluble CD25 ( s CD25) level were measured for 7 patients, and they all had a reduction in NK cell activity and an increase in s CD25 level. Conclusion Some diagnostic indices of HLH have a low positive rate in the early course of the disease. For patients with unexplained abnormal liver function and persistent fever, HLH cannot be excluded even if no significant peripheral cytopenia is observed in the early stage. The change in blood cells should be monitored, and laboratory examination including ferritin and blood lipids should be performed as early as possible to assist early diagnosis and treatment.