Characteristic and prognosis of patients with non-EBV infection-associated hemophagocytic lymphohistiocytosis.
10.3760/cma.j.issn.0253-2727.2022.02.007
- VernacularTitle:非EB病毒病原体所致感染相关噬血细胞综合征的临床特征及预后
- Author:
Ya Hong YOU
1
;
Jing Shi WANG
1
;
Zhao WANG
1
Author Information
1. Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China.
- Publication Type:Journal Article
- Keywords:
Clinical characteristics;
Hemophagocytic lymphohistiocytosis;
Infection;
Outcome
- MeSH:
Adult;
Aged;
Epstein-Barr Virus Infections/complications*;
Female;
Herpesvirus 4, Human;
Humans;
Lymphohistiocytosis, Hemophagocytic/diagnosis*;
Male;
Middle Aged;
Prognosis;
Retrospective Studies
- From:
Chinese Journal of Hematology
2022;43(2):128-133
- CountryChina
- Language:Chinese
-
Abstract:
Objective: To explore the clinical characteristics and outcomes of patients with non-Epstein-Barr virus (EBV) infection-associated hemophagocytic lymphohistiocytosis (IAHLH) . Methods: Clinical data of 48 patients diagnosed with non-EBV IAHLH in Beijing Friendship Hospital from January 2015 to March 2021 were collected, and the clinical characteristics, treatment, curative effect and prognosis of the patients were analyzed retrospectively. Results: This study included 48 patients, 28 males and 20 females, with a median (range) age of 34.5 (2-74) years. Pathogens that cause IAHLH were as follows: virus (16 cases, 33.3%) , bacteria (17 cases, 35.4%) , parasitic agents (13 cases, 27.1%) , and fungi (2 cases, 4.2%) . The median time from onset to diagnosis of hemophagocytic syndrome (HLH) was 40 (10-160) days. The median (range) time duration from prodrome to the definite diagnosis of IAHLH was 67 (23-270) days. The clinical characteristics were fever (48 cases, 100%) , splenomegaly (34 cases, 70.8%) , cytopenia (38 cases, 79.1%) , elevated ferritin (45 cases, 93.8%) , elevated fasting triglyceride levels (7 cases, 14.6%) , hypofibrinogenemia (17 cases, 35.4%) , decrease natural killer cell activity (26 in 44 cases, 59.1%) , and elevated sCD25 (35 cases, 74.5%) . Twenty-five patients (52.1%) had adenopathy. Once a certain pathogen was identified as the causative factor of hemophagocytic lymphohistiocytosis (HLH) , cytotoxic agents and glucocorticoids were withdrawn, and specific pathogen-directed treatment was initiated. After treatment, 36 cases (75.0%) achieved complete response, and 14 of 15 patients (93.3%) with parasitic and fungal HLH got a response; however, the response rate of patient with bacterial and viral HLH was only 66.7% (22 of 33 patients) . The estimated 5-year overall survival rate was 72.3% (95%CI 50.3%-69.8%) . The adverse prognostic factors were total bilirubin over the upper limit of normal (OR=20.0, 95%CI 1.1-378.3, P=0.046) and pathogenic infection not fully controlled (OR=19.9, 95%CI 2.9-134.5, P=0.002) . Conclusion: Non-EBV IAHLH has a good prognosis. When diagnosed, cytotoxic agents and glucocorticoids should be tapered off, and pathogen-targeted therapy should be critically administered to clear the triggering infection.
