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Objective:To investigate the clinical manifestations, laboratory tests, diagnosis and treatment of discordant lymphoma (DL).Methods:The clinical data of a patient with EB virus-positive DL admitted to Taizhou People's Hospital in November 2019 were retrospectively analyzed and the related literature was reviewed.Results:The patient underwent a cervical lymph node biopsy pathology examination at onset, and then results suggested angioimmunoblastic T-cell lymphoma (AITL). The patient subsequently developed gastrointestinal bleeding and underwent resection of small bowel lesions, and postoperative pathology suggested diffuse large B-cell lymphoma (DLBCL). The patient was finally diagnosed as DL. The R2-CHOP chemotherapy regimen was given to the patient, but the patient still had recurrent gastrointestinal bleeding and poor general condition. The patient refused chemotherapy and was changed to lenalidomide monotherapy. Finally, the patient died due to multiorgan failure, with an overall survival of 13 months.Conclusions:DL is rarely seen in lymphoma, whereas the combination of AITL and DLBCL is extremely rare. The clinicians need to improve the understanding of this disease to avoid misdiagnosis and missed diagnosis.
ABSTRACT
Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of peripheral T-cell lymphoma (PTCL) with aggressive behaviour and poor prognosis.Due to the rarity of the disease,few prospective controlled randomized clinical trials were conducted,and the standard therapeutic option for AITL has not been established.Although conventional anthracycline-based chemotherapy achieves response rates of up to 50 ％,most of the patients relapse,the median survival time and overall survival rate are unsatisfactory.Recent studies have demonstrated that hematopietic stem cell transplantation and cyclosporine show promising efficacy in the treatment of AITL.This current review mainly focused on the advance of treatment for AITL.
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Angioimmunoblastic T-cell lymphoma (AITL) is a distinct peripheral T-cell lymphoma entity originating from follicular helper T (TFH) cell with peculiar clinical and pathological features.Today,it is still difficult to realize its etiology and pathogenesis clearly. This review presents the latest updated progresses on etiology and pathogenesis of AITL.
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Objective To improve the diagnosis efficiency of patients with angioimmunoblastic T-cell lymphoma (AILT)by analyzing the clinical characteristics and curative effect of AILT. Methods Retrospective studies were used on clinicopathological features,immunophenotypes,treatment and survival of 20 angioimmunoblastic T-cell lymphoma patients,who were collected between January 2005 and January 2010 of Beijing Military General Hospital. Results In the 20 patients receiving chemotherapy,the median age was 55.9 years old.All of the 20 had lymph nodes and 11 of whom were accompanied with B group of symptoms,which were confirmed by lymph node biopsy and T cell antigen CD3, CD45Ro positive expression in all the patients. ALL of the patients received the CHOP regimen and combining with other treatment such as DICE,ESHAP, Hyper-CVAD or autologous hematopoietic stem cell transplantation. A follow-up by 5 to 69 months showed that median survival was 20.2 (5-69) months and 1,2,and 3-year disease free survival (DFS) were 67 ％,33 ％,and 11％ respectively.8 patients of the group (40 ％) with a sustained remission (CCS) respectively survived 63,47,27,24,24,12,5,1 months so far.Conclusion Most patients were older with many complications during chemotherapy, which resulted in easy relapse, and even the symptoms were alleviated at the early stage of the chemotherapy. IPI prognostic index is more important with long-term survival in AITL.Therefore further studies are required to improve the outcome.
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Angioimmunoblastic T cell lymphoma (AITL), which accounts for only 1~2% of non-Hodgkin's lymphomas, is commonly accompanied by skin lesions. Those associated with AITL include erythematous plaques, nodules or rashes. Histological examination of most lesions shows infiltration by malignant lymphocytes. Ichthyosis is a generalized skin disease characterized by hyperkeratosis, in which the skin acquires an appearance resembling fish scales. Some cases may be acquired, but most have a genetic basis. We report a case of AITL with associated ichthyosis that is considered to be inherited.
Subject(s)
Humans , Exanthema , Ichthyosis , Immunoblastic Lymphadenopathy , Lymphocytes , Lymphoma, Non-Hodgkin , Lymphoma, T-Cell , Skin , Skin Diseases , Weights and MeasuresABSTRACT
The latest study and development of etiology,pathogenesis,diagnosis,differential diagnosis and cytogenetics are reviewed in this paper.
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Objective:To evaluate angioimmunoblastic T-cell lymphoma(AITL) completely, we gave in-depth investigation of histopathological features, specific immunochemical markers, antigen receptor gene rearrangements and in situ hybridization for Epstein-Barr virus (EBV). Methods: 15 cases of typical AITL displayed effacement of the normal lymph node architecture partially or completely, abundance of arborizing high endothelial vessels, infiltration of polymorphic cells and hyperplastic atypical T lymphocytes with or without clear cytoplasm. Clinical characteristics, histological manifestations, and immunohistochemical staining for CD3, CD20, CD4,CD21, CXCL13, CD10, and BCL6 were analyzed. Polymerase chain reaction for immunoglobulin heavy chain (IgH) and T cell receptor ? (TCR?) rearrangements and in situ hybridization for Epstein-Barr virus encoded RNA (EBER-1) were performed.Results: Histologically, we found eight cases with regressed lymphoid follicles, six with absence of follicles and one with hyperplastic follicles with interfollicular lesions. We also found eight cases displaying aggregation of clear cells, four infiltration of large lymphoid cells, five abundant epithelioid histiocytes. CD20 staining showed hyperplasia of large B cells in four cases. CD21 expression exihibited extrafollicular expansion of follicular dendritic cell meshworks in 11 cases (73.3%), partially with a tendency of perivascular distribution. Positive rate for CXCL13 and CD10 are 73.3% and 6.7% respectively. Monoclonal rearrangements of TCR? were detected in 6/15 (40%) of cases, IgH rearrangements in 7/15 (46.7%), of which five were monoclonal, while two oligoclonal. 8 out of 15 cases (53.3%) contained EBV-positive cells. Among the four cases with large B cell proliferation, three were EBV-positive. Conclusion: AITL display great complexity and diversity clinicopathologically. Only when we recognize such diversity, can we reasonably apply and properly evaluate immunochemical markers and molecular techniques, and thus give a correct diagnosis.