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Characteristic and prognosis of patients with non-EBV infection-associated hemophagocytic lymphohistiocytosis / 中华血液学杂志
Chinese Journal of Hematology ; (12): 128-133, 2022.
Article in Chinese | WPRIM | ID: wpr-929544
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as the causative factor of hemophagocytic lymphohistiocytosis (HLH) , cytotoxic agents and glucocorticoids were withdrawn, and specific pathogen-directed treatment was initiated.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.
Subject(s)

Full text: Available Index: WPRIM (Western Pacific) Main subject: Prognosis / Retrospective Studies / Herpesvirus 4, Human / Epstein-Barr Virus Infections / Lymphohistiocytosis, Hemophagocytic Type of study: Observational study / Prognostic study Limits: Adult / Aged / Female / Humans / Male Language: Chinese Journal: Chinese Journal of Hematology Year: 2022 Type: Article

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Full text: Available Index: WPRIM (Western Pacific) Main subject: Prognosis / Retrospective Studies / Herpesvirus 4, Human / Epstein-Barr Virus Infections / Lymphohistiocytosis, Hemophagocytic Type of study: Observational study / Prognostic study Limits: Adult / Aged / Female / Humans / Male Language: Chinese Journal: Chinese Journal of Hematology Year: 2022 Type: Article