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Objective:To investigate the effects of hemophagocytic syndrome also known as hemophagocytic lymphohistiocytosis (HLH) on the clinical features and therapeutic efficacy of patients with Epstein-Barr virus-positive T-cell lymphoma (EBV-TCL).Methods:The clinical data of patients with EBV-TCL diagnosed by pathological examination in the First Affiliated Hospital of Guangzhou Medical University from November 2015 to August 2020 were retrospectively analyzed. According to whether they were accompanied with HLH at the time of onset, patients were divided into HLH group (10 cases) and non-HLH group (13 cases), and the clinical features and prognosis of the two groups were compared. The curative effects of different treatment methods and patients with different plasma EBV-DNA titers were compared.Results:Among 23 patients, 3 cases (13.0%) were in Ann Arbor stage Ⅰ-Ⅱ, 20 cases (87.0%) were in stage Ⅲ-Ⅳ; the International Prognostic Index (IPI) score was 1 point in 3 cases (13.0%), 2 points in 4 cases (17.4%), 3 points in 8 cases (34.8%), 4 points in 8 cases (34.8%). In the HLH group, there were 2 cases of aggressive NK-cell leukemia and 3 cases of childhood systemic EBV-TCL. There were no cases of above two pathological types in the non-HLH group. In the HLH group, the proportions of patients with fever, bone marrow invasion, IPI score > 2 points, and EBV-DNA > 10 4 copies/ml were higher than those in the non-HLH group (all P < 0.05). The objective response rate (complete remission plus partial remission) of all patients after chemotherapy was 47.8% (11/23); there were 3 cases undergoing hematopoietic stem cell transplantation in both the HLH group and the non-HLH group, and all achieved objective remission. The objective remission of 7 patients and 10 patients who did not undergo hematopoietic stem cell transplantation in the HLH group and non-HLH group after lymphoma chemotherapy had 0 case and 5 cases, respectively, and the difference was statistically significant ( P = 0.044). In the chemotherapy alone group, 5 of 17 patients had objective remission, 6 patients in the chemotherapy plus transplantation group had objective remission, and the difference was statistically significant ( P = 0.039). Among 16 patients whose plasma EBV-DNA titers turned negative, 11 patients had objective remission, and 7 patients whose plasma EBV-DNA titers were continuously positive had no objective remission, and the difference was statistically significant ( P = 0.001). The 1-year overall survival rate of all patients was 69.3%, and the 2-year overall survival rate was 52.0%. In the HLH group, the 1-year and 2-year overall survival rates of 7 patients receiving chemotherapy alone and 3 patients receiving chemotherapy plus transplantation were 42.9% and 66.7%, respectively. In the non-HLH group, the 1-year overall survival rates of 10 patients receiving chemotherapy alone and 3 patients receiving chemotherapy plus transplantation were 80.0% and 100.0%, respectively; the 2-year overall survival rates were 26.7% and 100.0%,respectively. The overall survival of patients receiving chemotherapy plus transplantation was better than that of those receiving chemotherapy alone in both the HLH group and the non-HLH group, and differences were statistically significant (all P < 0.05). Conclusions:The general clinical stage of patients with EBV-TCL is later, and the prognosis of EBV-TCL patients with HLH is worse. The therapeutic efficacy may be related to plasma EBV-DNA titers. Hematopoietic stem cell transplantation can improve the remission rate.
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Objective:This study aims to examine the clinicopathological features, diagnosis, and treatment of pulmonary margin-al zone B-cell lymphoma of mucosa-associated lymphoid tissue (PMZL-MALT). Methods:The clinicopathological features and immu-nohistochemical staining of CD20, CD79a, CD5, CD10, CD23, CyclinD1, and Ki-67 in seven patients with PMZL-MALT were ana-lyzed. Results:These patients, with a median age of 58 years, included three males and four females. Most of the patients suffered from cough, anhelation, and irregular fever. No specific imaging manifestation was observed. Tumor cells were positive for CD19 and CD20 but negative for CD5, CD10, and CyclinD1. The positive rate of Ki-67 was low. Conclusion:PMZL-MALT cases are easily misdiag-nosed because of the absence of specific clinical characteristics and X-ray features. Final diagnosis depends on pathological examina-tions.
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Objective To investigate the distribution of TCR Vβ genealogy and clonal expansion in peripheral blood after infusing mesenchymal stem cells (MSC) in patients with chronic GVHD. Methods The complementarity determining region 3 (CDR3) of 24 TCR Vβ subfamily genes in peripheral blood mononuclear cell from 1 case with cGVHD after allogeneic hematopoietic stem cell transplantation (Allo-HSCT),who were treated with infusing MSC,were amplified using RT-PCR. The blood samples were taken at the first and the fifth day after 1st infusion; and the first day,the 10 th day and the 20 th day after the second infusion of MSC,as well as the MSC infused as control . The products were labelled by fluorescein and then analyzed the CDR3 size with gene scan technique to determine the clonality of T cells. Results There were no expression of TCR Vβ subfamily with the MSC infused and after the 1st day of the first infusion of MSC. Then 3,10,14,10 Vβ subfamilies clones are appeared at the other time points,of which were polyclone and oligoclone predominately. In the same time,the manifestations of cCVHD have been abated. Conclusion MSC played a certain role in reviving the immune function of the patients after Allo-HSCT and mitigating the disease of chronic GVHD. Lineage analysis of TCR Vβ subfamily showed some predominant expression.
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Objective To investigate the impact of auto and allogenic mesenchymal stem cells (MSC) transplantation on hematopoietic reconstitution. Methods MSC from auto, donor bone marrow or embryonic tissue were cultured and expanded in vitro in the serum culture system. Five patients received hematopoietic stem cell transplantation (HSCT) were investigated. Case 1 of systemic lupus erythematosus and Case 2 of non-hodgkin' s lymphoma (NHL) received auto MSC transplant before auto-HSCT. Case 3 of paroxysmal nocturnal hemoglobinuria received HLA-identical allogenic MSC transplant before HLA-identical allo-HSCT.Case 4 of chronic myelocytic leukemia and Case 5 of NHL had delayed hematopoietic reconstitution (129th and 78th day, respectively) after allo- and auto-HSCT, respectively, and received MSC from embryonic tissue.Results Case 1, 2 and 3 had no manifested side effects after MSC transplantation combined with HSCT.Neutrophil count of case 1, 2, and 3 were over 0.5 ×109/L at 1st, 10th and 10th day, respectively, platelet count were over 20 ×109/L at 1st, 8th and 33th day, respectively, and agranulocytosis at Ost, 7th and 12th day, respectively. The treatment of embryonic tissue MSC transplant was confirmed to fail for Case 4 and 5.Conclusion The time of MSC transplant has a great impact on hematopoietic reconstitution. MSC transplantation and HSCT performed simultaneously can improve hematopoietic reconstitution. However, the impact of MSC on patients with delayed hematopoietic reconstitution after HSCT needs further study.