Clinical characteristics of hemophagocytic lymphohistiocytosis

Abstract

BACKGROUND:

There is no specificity in the clinical presentation of hemophagocytic lymphohistiocytosis (HLH).

OBJECTIVE:

To study some clinical, etiological, and prognostic features of HLH to improve the clinical understanding of the disease.

METHODS:

Retrospective analysis of the clinical data of 125 patients with HLH admitted to our hospital from June 2015 to August 2021, including clinical characteristics, laboratory indicators, and survival period. Statistical analysis was performed from the overall group of study indicators, which included population, children, and adults.

RESULTS:

In the whole population, sex, age, blood myoglobin, and NK cell ratio of M-HLH and non-M-HLH patients ( $P<$ 0.05), serum albumin, and direct bilirubin were independent correlates of M-HLH. In the pediatric group, age and the proportion of NK cells were significantly different between M-HLH and non-M-HLH patients ( $P<$ 0.05). Multivariate Logistic regression analysis showed that all factors were not significantly associated with M-HLH. The associated regression analysis showed that all factors were not significantly associated with M-HLH. ROC curve analysis showed that the best predictive value of NK cell percentage for M-HLH diagnosis in the overall population was 4.96% in the pediatric group and 4.96% in the adult group. The best predictive value for M-HLH diagnosis was 2.08%. The univariate analysis showed that platelet count, alanine aminotransferase, aspartate aminotransferase, serum albumin, direct bilirubin and indirect bilirubin affected prognosis; COX regression showed that none of these factors had a significant relationship. The overall median survival time was 20.7 months in the adult group, 44.3 months in non-M-HLH patients, and 7.73 months in M-HLH patients ( $p=$ 0.011); univariate analysis showed that platelet count and serum albumin level affected prognosis; COX regression results in serum albumin level was an independent risk factor for prognosis.

CONCLUSION:

The survival rate of non-M-HLH was significantly better than that of M-HLH; the proportion of NK cells had predictive value for the diagnosis of M-HLH; in the general population, non-M-HLH was more likely to have abnormal liver function than M-HLH: lower platelet count and serum albumin level were associated with poor prognosis, and the lower the platelet count and serum albumin level, the worse the prognosis: in addition, adults with lower serum albumin levels are also associated with poor prognosis.