Background/aim: This research study was conducted to examine the clinical characteristics and post-splenectomy survival outcomes of patients diagnosed with hepatosplenic T-cell lymphoma (HSTCL).
Materials And Methods: A total of 10 cases of HSTCL patients admitted to the Hematology Department of Fudan University Affiliated Huadong Hospital between January 2012 and December 2021 were included. In addition, we also included 30 other cases reported from domestic and international sources. All pathological specimens were stained with hematoxylin and eosin (H&E) and immunohistochemistry, with color development using DAB staining. Survival analysis was conducted using Kaplan-Meier curves and log-rank tests.
Results: In the 10 HSTCL patients, Epstein-Barr virus (EBV) infection was confirmed. Six patients had died, with 5 of them within 1 year of disease onset. Survival analysis showed poorer prognosis in patients with hemophagocytic syndrome and thrombocytopenia. Patients who underwent splenectomy followed by chemotherapy had a higher median and average survival time compared to those who only received chemotherapy. The study included a total of 40 HSTCL patients, with 29 males and 11 females, and an average age of onset at 42.3 years. All patients presented with fever, with some exhibiting emaciation and/or hemophagocytic syndrome. Splenomegaly, hepatomegaly, lymphadenopathy, and bone marrow involvement were found in the patients. Common laboratory findings included leukopenia, anemia, and thrombocytopenia. All patients exhibited elevated ferritin levels and decreased blood calcium levels.
Conclusion: Those patients suffering from hemophagocytic syndrome at the onset of this disease face greater treatment-related difficulties and a higher risk of mortality. Combined chemotherapy after splenectomy may improve HSTCL patient survival.
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http://dx.doi.org/10.1186/s12957-024-03613-0 | DOI Listing |
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC11656711 | PMC |
World J Surg Oncol
December 2024
Department of Hematology, Huadong Hospital, Fudan University, No.221, Yan 'an West Road, Jing' an District, Shanghai, 200040, China.
Background/aim: This research study was conducted to examine the clinical characteristics and post-splenectomy survival outcomes of patients diagnosed with hepatosplenic T-cell lymphoma (HSTCL).
Materials And Methods: A total of 10 cases of HSTCL patients admitted to the Hematology Department of Fudan University Affiliated Huadong Hospital between January 2012 and December 2021 were included. In addition, we also included 30 other cases reported from domestic and international sources.
World J Clin Oncol
June 2024
Stem Cell Transplantation and Cellular Therapy, John Theurer Cancer Center, Hackensack, NJ 07601, United States.
Background: Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive peripheral T-cell lymphoma with historically dismal outcomes, representing less than one percent of non-Hodgkin lymphomas. Given its rarity, the true incidence of HSTCL is unknown and most data have been extrapolated through case reports. To the best of our knowledge, the largest and most up to date study addressing the epidemiology and outcomes of patients with HSTCL in the United States covered a period from 1996 to 2014, with a sample size of 122 patients.
View Article and Find Full Text PDFBiomedicines
April 2024
Laboratory of Clinical Research and Advanced Diagnostics, Centro di Riferimento Oncologico della Basilicata (IRCCS-CROB), 85028 Rionero In Vulture, Italy.
Lymphoproliferative diseases are a heterogeneous set of malignant clonal proliferations of lymphocytes. Despite well-established diagnostic criteria, the diagnosis remains difficult due to their variety in clinical presentation and immunophenotypic profile. Lymphoid T-cell disorders are less common than B-cell entities, and the lack of a clear immunophenotypic characteristic makes their identification hard.
View Article and Find Full Text PDFTransplant Cell Ther
May 2024
Division of Hematology and Medical Oncology, Mayo Clinic, Jacksonville, Florida. Electronic address:
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive type of peripheral T-cell lymphoma with median overall survival (OS) of approximately 1 year. Data on the effectiveness of hematopoietic cell transplantation (HCT) is limited, as is the choice between autologous HCT (auto-HCT) and allogeneic HCT (allo-HCT) in the treatment of this disease. To evaluate the outcome of patients with HSTCL who underwent either auto-HCT or allo-HCT, we performed a multi-institutional retrospective cohort study to assess outcomes of HCT in HSTCL patients.
View Article and Find Full Text PDFBr J Haematol
June 2024
University of Nottingham, Nottingham, UK.
To address the lack of contemporary population-based epidemiological studies of hepatosplenic T-cell lymphoma (HSTCL), we undertook a population-based study of ICD-O-3-coded HSTCL in England. We used the National Cancer Registration Dataset and linked datasets on hospital admissions, Systemic Anti-Cancer Therapy, socio-demographics, comorbidities and death, identifying cases from 1 January 2013 to 31 December 2019 with survival data up to 5 January 2021. Crude and directly age-standardised incidence rates per million persons per year were calculated.
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