Clinical characteristics and prognostic analysis of secondary hemophagocytic syndrome in adults / 白血病·淋巴瘤

ABSTRACT

Objective:To investigate the clinical characteristics and prognosis of secondary hemophagocytic syndrome (HPS) in adults.Methods:The clinical data of 43 adult patients with primary secondary HPS in Mianyang Central Hospital of Sichuan Province from January 2014 to June 2020 were retrospectively analyzed. The patient's clinical manifestations and biochemical indicators were summarized. The clinical characteristics of patients between high-level ferritin (≥5 000 μg/L) group and low-level ferritin (<5 000 μg/L) group, lymphoma-related HPS group and infection-related HPS group were compared. Logistic regression model was used for multivariate survival analysis.Results:All patients had fever as the main complaint, and infections were manifested at the onset or during treatment. In all patients, 88.4% (38/43) had hemophagocytosis in the bone marrow; 88.4% (38/43) had varying degrees of cytopenia; 36.6% (15/41) had reduced fibrinogen [median value 0.93 g/L (0.50-1.35 g/L)]; 76.7% (33/43) had elevated aminotransferase. Compared with the low-level ferritin group (19 cases), the high-level group (21 cases) had a lower proportion of monocytes (0.081±0.040 vs. 0.163±0.076, P = 0.016), the incidence of fibrinogen reduction and the incidence of aminotransferase elevation were high [60.0% (12/20) vs. 11.1% (2/18), P = 0.002; 95.2% (20/21) vs. 52.6% (10/19), P = 0.006]. Compared with infection-related HPS patients, lymphoma-related HPS patients had a higher incidence of lymphadenopathy [72.7% (16/22) vs. 27.8% (5/18), χ2 = 8.021, P = 0.005] and a lower incidence of aminotransferase elevation [59.1% (13/22) vs. 94.4% (17/18), χ2 = 4.848, P = 0.028]. Multivariate analysis showed that the high proportion of neutrophils ( OR = 0.886, 95% CI 0.786-0.998, P = 0.046) and high-level albumin ( OR = 0.376, 95% CI 0.156-0.907, P = 0.030) after treatment were protective factors for survival of patients. Conclusions:Among adult patients with primary secondary HPS, the proportion of monocytes in patients with ferritin ≥5 000 μg/L is low, and liver damage and fibrinogen reduction are more likely to occur. Lymphoma-related HPS patients are more likely to have lymphadenopathy, and infection-related HPS patients are more likely to have liver damage. After treatment, a low proportion of neutrophils and hypoproteinemia indicate a poor prognosis in adult patients with secondary HPS.