Clinical characteristics and prognostic factors of adult hemophagocytic syndrome patients: a retrospective study of increasing awareness of a disease from a single-center in China

Abstract

Background: Hemophagocytic lymphohistiocytosis (HLH) is a relatively rare but life-threatening disease with confusing clinical manifestations, rapidly deteriorating health, high morbidity and mortality.

Methods: To improve the recognition as well as understanding of this disorder, we analyzed clinical characteristics and prognostic factors from 85 adult patients diagnosed with HLH in our hospital from April 2005 to June 2014.

Results: Patients with HLH displayed variable clinical markers across a wide spectrum. These included fever and hyperferritinemia (100%), elevated lactate dehydrogenase (LDH) (98.8%), two or three cytopenia (92.2%), splenomegaly (72.9%), hypofibrinogenemia (69.4%), hypertriglyceridemia (64.7%), hemophagocytosis (51.7%), and hepatomegaly (24.7%). Patients with active Epstien-Barr Virus (EBV) infection had a median overall survival (OS) of 65 days. Those displaying malignancy had very poor survival (median OS: 40 days). However, patients in rheumatic and non-EBV infection groups had relatively superior prognosis (not reached). Univariate analysis showed that Fibrinogen (Fbg) <1.5 g/L, platelet number (PLT) <40 × 10(9)/L and LDH ≥1000 U/L were factors that negatively affected survival (P = 0.004, 0.000, 0.002). Multivariate analysis showed that PLT <40 × 10(9)/L was the independent adverse factor (HR = 0.350, 95% CI: 0.145-0.844, P = 0.019).

Conclusions: HLH had very complex clinical manifestations and high death rate. Patients with active EBV infection, malignancy, Fbg <1.5 g/L, PLT <40 × 10(9)/L and LDH ≥1000 U/L had high risk of death as well as inferior survival, and these patients require systemic targeted treatments as early as possible.

Figure 1

The characteristics of 85 adult HLH patients. A. The percentages of clinical symptoms, signs and laboratory examinations in 85 HLH patients. A: fever, B: ferritin ≥500 g/L, C: LDH ≥220 U/L, D: cytopenia in two or more lineages, E: GPT >40 U/L, F: splenomegaly, G: hypofibrinogenaemia, H: hypertriglyceridemia, I: KPTT prolonged 10 S, J: Hemophagocytosis in bone marrow, K: PT prolonged 3S, L: jaundice, M: Lymphadenopathy, N: hepatomegaly, O: renal impairment, P: skin rash, Q: epilepsy. B. The distribution of diagnostic time in 85 HLH patients. C. The initially hospitalized departments in 85 HLH patients.

Figure 2

The number of survive patients in different subgroups.

Figure 3

Survival analysis in 85 cases and different subgroups. A. Kaplan-Meier survival in 85 HLH patients. B. The survival curves of patients in the fibrinogen (Fbg) subgroup. C. The survival curves of patients in the platelet (PLT) subgroup. D. The survival curves of patients in the lactate dehydrogenase(LDH) subgroup.