Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study

Authors

Huting Hou^1, , Xudong Zhang¹, Siyu Qian¹, Zeyuan Wang¹, Meng Dong¹, Xiaojuan Zhang², Xiaoguang Duan², Yue Zhang¹, Qing Wen¹, Jingjing Ge¹, Yaxin Lei¹, Mingzhi Zhang¹, Qingjiang Chen^{1, *}

¹Department of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan Province, No. 1 Jianshe East Road, Zhengzhou 450052, China

²General ICU, The First Affiliated Hospital of Zhengzhou University, No. 1 Jianshe East Road, Zhengzhou 450052, China

^*Corresponding author. E-mail: qingjiang_c@126.com

DOI

10.2991/icres.k.211129.001How to use a DOI?

Keywords

B-cell lymphoma; hemophagocytic syndrome; clinical features; prognosis

Abstract

Purpose: Current knowledge of Lymphoma Associated Hemophagocytic Syndrome (LAHS) mainly relies on T/NK-cell LAHS cases, while B-cell lymphoma associated hemophagocytic syndrome (B-LAHS) variant are rare. We aimed at identifying a single-center cohort of patients with B-LAHS to shed light on relevant clinical and laboratory features, diagnosis, treatment and prognosis.

Methods: The clinical data of 36 patients with B-LAHS, admitted to the First Affiliated Hospital of Zhengzhou University in the time period ranging from January 2011 to May 2021 were analyzed retrospectively. Survival analysis was performed using the Kaplan–Meier method and groups were compared using the log-rank test.

Results: The 36 patients included 17 males and 19 females with a median age of 53.5 years (range: 28–74 years). Among these, the most common histopathological type diagnosed was diffuse large B-cell lymphoma (69%). The initial symptoms included fever (100%), splenomegaly (97%), multicavity effusion (83%), abnormal liver function (58%), and jaundice (44%). Patients treated with dexamethasone only seemed to have an inferior prognosis compared to those treated with etoposide-involving regimens (χ² = 20.037, p < 0.001). Patients received based on the Hemophagocytic Lymphohistocytosis (HLH)-2004 protocol combined with multidrug chemotherapy had a significantly improved Overall Survival (OS) compared to patients who only underwent treatment according to the HLH-2004 regimen (χ² = 30.744, p < 0.001). The 2-week mortality rate was 13.9% and the 1-year OS rate was 19.4%. Compared with the median survival time of 34 patients without Epstein–Barr virus (EBV) infection, 97 days (range: 24–322 days), two patients with EBV infection died within 8 days.

Conclusion: B-LAHS is relatively rare and has a high early mortality rate, mostly in middle-aged and elderly patients. The HLH-2004 regimen combined with multidrug chemotherapy is a reasonable choice for the treatment of B-LAHS, and EBV infection may be used as a reference indicator of a poor prognosis.

Copyright

© 2021 First Affiliated Hospital of Zhengzhou University. Publishing services by Atlantis Press International B.V.

Open Access

This is an open access article distributed under the CC BY-NC 4.0 license (http://creativecommons.org/licenses/by-nc/4.0/).

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TY  - JOUR
AU  - Huting Hou
AU  - Xudong Zhang
AU  - Siyu Qian
AU  - Zeyuan Wang
AU  - Meng Dong
AU  - Xiaojuan Zhang
AU  - Xiaoguang Duan
AU  - Yue Zhang
AU  - Qing Wen
AU  - Jingjing Ge
AU  - Yaxin Lei
AU  - Mingzhi Zhang
AU  - Qingjiang Chen
PY  - 2021
DA  - 2021/12/04
TI  - Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study
JO  - Intensive Care Research
SP  - 45
EP  - 50
VL  - 1
IS  - 3-4
SN  - 2666-9862
UR  - https://doi.org/10.2991/icres.k.211129.001
DO  - 10.2991/icres.k.211129.001
ID  - Hou2021
ER  -