A Decade of Lymphoma-Associated Hemophagocytic Lymphohistiocytosis: Does the Outcome Improve?

Abstract

To investigate the potential treatment evolution and outcome improvement, we retrospectively compared clinical characteristics, therapeutic strategies, treatment responses, and overall survival (OS) in patients diagnosed and treated with lymphoma-associated HLH between 2004–2012 (n = 30) and 2013–2021 (n = 26). Our study showed that the clinical characteristics of lymphoma-associated HLH did not substantially change over the past two decades. However, more patients diagnosed in 2013–2021 were tested for Epstein–Barr virus than those diagnosed in 2004–2012 (69.3% vs. 33.3%; p = 0.021). In addition, Eastern Cooperative Oncology Group performance status 3–4 (hazard ratio (HR): 5.38; 95% confidence intervals (CI): 2.49–11.61; p < 0.001) and jaundice (HR: 2.91; 95% CI: 1.37–6.18; p = 0.006) were poor prognostic factors for lymphoma-associated HLH. With a comparable response rate of lymphoma treatment, patients treated in 2013–2021 had a numerically greater median OS than those treated in 2004–2012 (23.6 ± 19.8 vs. 9.7 ± 4.5 months). However, the difference was not statistically significant (p = 0.334). In conclusion, early diagnosis and tailored treatments that balance efficacy and adverse events remain the key to obtaining a better outcome in lymphoma-associated HLH.

Epstein-Barr Virus-Positive Lymphoma-Associated Hemophagocytic Syndrome: A Retrospective, Single-Center Study of 51 Patients

“…Studies investigating prognosis predictors of LAHS patients are rare. Higher ECOG score, skin involvement, lower fibrinogen level, jaundice have been reported as risk factors of LAHS prognosis ( 24 , 35 , 36 ). Studies exploring risk factors of EBV-positive LAHS are lacking.…”

Clinical features and prognostic analysis of lymphoma‑associated hemophagocytic syndrome: A report of 139 cases

“…In addition, a study of 567 patients with HPS in Japan between 2001 and 2005 showed that patients with NK/T-LAHS had the worst prognosis, with a 5-year survival rate of <15%, followed by familial HPS and B-LAHS (3). Numerous studies have confirmed that the prognosis of patients with LAHS is relatively poor (3)(4)(5)16,17). Due to its rarity and the heterogeneity of inducing factors and clinical outcomes, the management of LAHS remains challenging.…”

“…Secondary HPS may develop as a consequence of strong immune activation due to severe infections, malignancies or autoimmune diseases. Currently, the most common secondary HPS is lymphoma-associated HPS (LAHS), which has been reported in 20-67% of patients (3)(4)(5).…”