Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study
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...
| Main Authors: | , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Springer
2021-12-01
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| Series: | Intensive Care Research |
| Subjects: | |
| Online Access: | https://doi.org/10.2991/icres.k.211129.001 |
| _version_ | 1797274460322004992 |
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| author | Huting Hou Xudong Zhang Siyu Qian Zeyuan Wang Meng Dong Xiaojuan Zhang Xiaoguang Duan Yue Zhang Qing Wen Jingjing Ge Yaxin Lei Mingzhi Zhang Qingjiang Chen |
| author_facet | Huting Hou Xudong Zhang Siyu Qian Zeyuan Wang Meng Dong Xiaojuan Zhang Xiaoguang Duan Yue Zhang Qing Wen Jingjing Ge Yaxin Lei Mingzhi Zhang Qingjiang Chen |
| author_sort | Huting Hou |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-07T14:59:41Z |
| format | Article |
| id | doaj.art-a94d8b861ac8445892208257277bf151 |
| institution | Directory Open Access Journal |
| issn | 2666-9862 |
| language | English |
| last_indexed | 2024-03-07T14:59:41Z |
| publishDate | 2021-12-01 |
| publisher | Springer |
| record_format | Article |
| series | Intensive Care Research |
| spelling | doaj.art-a94d8b861ac8445892208257277bf1512024-03-05T19:15:10ZengSpringerIntensive Care Research2666-98622021-12-0113-4455010.2991/icres.k.211129.001Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center StudyHuting Hou0Xudong Zhang1Siyu Qian2Zeyuan Wang3Meng Dong4Xiaojuan Zhang5Xiaoguang Duan6Yue Zhang7Qing Wen8Jingjing Ge9Yaxin Lei10Mingzhi Zhang11Qingjiang Chen12Department of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceGeneral ICU, The First Affiliated Hospital of Zhengzhou UniversityGeneral ICU, The First Affiliated Hospital of Zhengzhou UniversityDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceDepartment of Oncology, The First Affiliated Hospital of Zhengzhou University; Lymphoma Treatment Center of Henan ProvinceAbstract 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.https://doi.org/10.2991/icres.k.211129.001B-cell lymphomahemophagocytic syndromeclinical featuresprognosis |
| spellingShingle | Huting Hou Xudong Zhang Siyu Qian Zeyuan Wang Meng Dong Xiaojuan Zhang Xiaoguang Duan Yue Zhang Qing Wen Jingjing Ge Yaxin Lei Mingzhi Zhang Qingjiang Chen Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study Intensive Care Research B-cell lymphoma hemophagocytic syndrome clinical features prognosis |
| title | Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study |
| title_full | Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study |
| title_fullStr | Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study |
| title_full_unstemmed | Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study |
| title_short | Clinical Features and Prognosis of B-cell Lymphoma-associated Hemophagocytic Syndrome: A Retrospective Single Center Study |
| title_sort | clinical features and prognosis of b cell lymphoma associated hemophagocytic syndrome a retrospective single center study |
| topic | B-cell lymphoma hemophagocytic syndrome clinical features prognosis |
| url | https://doi.org/10.2991/icres.k.211129.001 |
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