EB virus-positive discordant lymphoma: report of 1 case and review of literature / 白血病·淋巴瘤

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.

Linfoma de células t angioimunoblástico em paciente idoso e com diagnóstico prévio de mononucleose infecciosa: um desafio para o tratamento oncológico ? relato de caso / Angioimmunoblastic T-cell lymphoma in elderly patient with a previous diagnosis of infectious mononucleosis: a challenge for cancer treatment ? case report

O linfoma de células T angioimunoblástico (AITL) é subtipo raro de linfoma de células T periférico que representa cerca de 2% dos linfomas não Hodgkin (LNH). Acomete predominantemente homens, na sexta e sétima décadas de vida e se apresenta como doença sistêmica. É agressivo no comportamento e responde mal à quimioterapia, com tratamento padrão ainda não definido. Este relato objetiva apresentar o prognósticodesfavorável nos pacientes com AITL e a falta de consolidação de seu tratamento. Os fatores que podem estar associados à má-resposta são: idoso, estágios avançados e sintomatologia constitucional.

Angioimmunoblastic T-cell lymphoma (AITL) is a rare subtype of peripheral T-cell lymphoma that represents about 2% of non-Hodgkin?s lymphomas (LNH). It affects mainly men, in the sixth and seventh decades of life, and appears as a systemic disease. It has an aggressive behavior and responds poorly to chemotherapy, still with undefined standard treatment. This report aims to provide the poor prognosis in patients with AITLand the lack of treatment consolidation. The factors that may be associated with the poor response are being elderly, advanced stages, and constitutional symptoms.

Comparación entre CHOP-like y R-CHOP en pacientes con linfoma no Hodgkin difuso de grandes células B y folicular: 10 años de experiencia del Hospital de Clínicas de Montevideo, Uruguay / Comparison between CHOP-like and R-CHOP in diffuse large B cell and follicular lymphoma

Background: The most common types of non-Hodgkin lymphoma (NHL) are diffuse large B cell (DLBCL) and follicular (FL). Aim: To analyze the benefit ofRituxi-mab in overall survival (OS) of patients with NHL. Material and Methods: Review of medical record of 230 adult patients with afirst episode of NHL admitted between 2002 and 2011. We included 67 patients with DLBCL and 36 patients with FL. Results: The overall response (OR) was 64% with 39% complete remissions (CR) in DLBCL treated with CHOP-like and 100% with 89% CR with R-CHOP. The median OS with CHOP-like was 21 months versus not attained R-CHOP (p = 0.016). There was a statistically significant difference in median event-free survival (EvFS) in favor of R-CHOP: not attained versus 8.3 months for CHOP-like (log rank (p = 0.002)). In FL, the OR in patients treated with R-CHOP or R-CHOP-like was 85%) with 54% CR. With CHOP-like the OR was 59%> with 18% CR. The OS at 24 and 36 months in patients treated with R-CHOP or R-CHOP-like was 83 and 65%. The figures for patients treated with CHOP-like were 80 and 66%> respectively. The progression free survival (PFS) was 21 months with CHOP-like versus not attained with R-QT (p = 0,043). Conclusions: When Rituximab was added to CHOP, there was a higher CR, EvFS and OS in DLBCL and higher CR and PFS in FL.

Immunoblastic morphology as a possible prognostic indicator for the outcome of the patients with diffuse large B cell lymphoma in era of the rituximab based treatment: single centre experience.

Recently the results from one large prospective study indicated that immunoblastic morphology and not immunohistohemical features predict the outcome of the Diffuse large B lymphoma (DLBL). In order to investigate the prediction value of the immunoblastic morphology (IB) as a possible prognostic indicator for the outcome of our DLBL patient treated with the Rituximab (R)-CHOP regimen we conducted a retrospective study. Our study enrolled 192 DLBL patients diagnosed and treated at the University Clinic of Hematology in the period between February 2002 and December 2007. They were all treated with R-CHOP regimen and the median follow-up of the patient was 36 months. We analyzed the biopsy samples immunohistochemically for markers of germinal center (BCL6), post-germinal center (MUM1) and apoptosis (BCL2).The patients were categorized as DLBL(132; 68.7%), IB(60; 31.2). The median overall survival time (OS) were 59.3 months in DLBL group and 42.2 months in IB group, and time to treatment (TT) were 56.8 and 30.6 months respectively for the IB group. The DLBL and IB groups were comparable regarding the age, gender distributions and all others already established prognostic parameters as performance status, advanced IPI, albumin level except for the low IPI 0-2 which was statistically associated with the DLBL group (p=.024). Our results did not show any statistical survival advantage and better outcome for the patient classified as DLBL when treated with R-CHOP and indicate that immunohistohemical markers do not really reflect the molecular diversity of the tumor. Our work shows that IB morphology is a major risk factor in DLBL patients treated with R-CHOP. Therefore this morphology appears to capture some adverse molecular events that a currently hard to detect with routine diagnostic procedures.

Advances of treatment for angioimmunoblastic T-cell lymphoma / 白血病·淋巴瘤

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.

Advances on etiology and pathogenesis of angioimmunoblastic T-cell lymphoma / 白血病·淋巴瘤

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.

Clinical analysis of 20 patients with angioimmunoblastic T-cell lymphoma / 白血病·淋巴瘤

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.

A case of angioimmunoblastic T cell lymphoma in a patient with ichthyosis / 대한내과학회지

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.

Development of angioimmunoblastic T-cell lymphoma / 白血病·淋巴瘤

The latest study and development of etiology,pathogenesis,diagnosis,differential diagnosis and cytogenetics are reviewed in this paper.

Clinicopathologic,immunohistochemical and molecular analysis in 15 cases of angioimmunoblastic T-cell lymphomas / 北京大学学报(医学版)

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.

Subclassification of diffuse large B-cell lymphomas according to the REAL classification: Distinction of immunoblastic and non-immunoblastic subtypes / 대한내과학회지

BACKGROUND: Diffuse large B-cell lymphoma (DLBL) category in the REAL classification includes histologically heterogeneous subtypes in Working Formulation or Kiel classification. Some investigators insist that the prognosis of B-cell immunoblastic lymphoma (IBL) is worse than other types of DLBL. This study was performed to determine the clinical significance of histological subclassification of DLBL. METHODS: All non-Hodgkin's lymphomas diagnosed at 3 hospitals in Korea between 1989 and 1995 were reclassified according to the REAL classification. Medical records of 404 patients with DLBL were reviewed. Their pathologies were categorized into IBL or non-IBL according to Working Formulation. We compared clinical characteristics and treatment outcomes of IBL with those of non-IBL. RESULTS: Of 404 DLBL patients, 341 cases (84%) were classified as non-IBL and 63 cases (16%) as IBL. Male patients were more common in IBL than in non-IBL (76% vs. 62%). IBL presented more often with advanced stage (III or IV) and B-symptoms than non-IBL (57% vs. 42%, 40% vs. 27%, respectively). In other clinical characteristics, no significant differences were found between the two groups. Complete response rates were 59% in IBL and 68% in non-IBL (p=0.137). With a median follow-up of 52 months (range 1-108 months), the median progression-free survival was 11 (95% confidence interval [95% CI] 8-14) months for IBL and 41 (95% CI 18-64) months for non-IBL (p=0.004). The median overall survival was 21 (95% CI 13-29) months for IBL and 72 months for non-IBL (p=0.002). A multivariate analysis for progression-free survival and overall survival showed that histological subtype (non-IBL vs. IBL) was a significant prognostic factor independent of International Prognostic Index (p=0.013 for progression-free survival, p=0.003 for overall survival). CONCLSUION: DLBL includes heterogeneous subtypes with different prognosis. Subclassification of DLBL into IBL and non-IBL has prognostic significance. IBL needs to be separated from other types of DLBL.

The radiographic findings of lymphoproliferative disorders of the lung / 中华放射学杂志

Objective To study the radiographic findings of lymphoproliferative disorders of the lung. Methods Twenty five patients with lymphoproliferative disorders of the lung were examined by X ray film, tomography, and CT. Results Multiple and mediastinal lymphadenopathy were observed in 2 patients with pulmonary pseudolymphoma. Multiple nodules or masses were observed in 4 patients with pulmonary lymphomatoid granulomatosis. Hilar and mediastinal lymphadenopathy was observed in each patient with angioimmunoblastic lymphadenophathy, 2 patients had multiple nodules or masses, 8 patients had single or multiple patchy infiltrations, 10 had diffuse interstitial infiltrations. 3 patients with Castlemen′ disease had a mass in the mediastinum, and another patient had mediastinal lymphadenopathy. Conclusion Radiographic findings of lymphoproliferative disorders of the lung are varied, and the final diagnosis relies on pathology.

Correlation between Clinical Outcome and Proliferation Index in Diffuse Large B-Cell Lymphoma

The diffuse large B-cell lymphoma category of the Revised European American Classification of Lymphoid Neoplasms (REAL) encompasses different morphologic lymphoma subtypes in a single entity, especially the diffuse large cell (DLC) and the immunoblastic (IBL) subtypes by Working Formulation (WF). The aim of this study is to determine the influence of the morphologic subdivision within this category with respect to clinical outcome and proliferative index using Ki-67 immunostainig combined with image analysis. We retrospectively reviewed 74 patients from 1990 to 1996, who were diagnosed with diffuse large B-cell lymphoma. All cases were reclassified according to REAL and Working Formulation (WF), and Ki-67 immunostaining was performed in all the cases. Fifty-eight cases (78.4%) were classified as DLC and 16 cases (21.6%) as IBL, according to WF. Twenty one cases (28.4%) showed nodal involvement and 53 cases (71.6%), extranodal involvement. All cases were found to display a variable degree of nuclear Ki-67 staining. A proliferative index of 50% or higher identified a group of patients (77%) who had poor clinical results. Overall survival was significantly reduced in these patients displaying high Ki-67 associated proliferative index compared to those with a low proliferative index (p=0.007). 5-year survival estimates were 93% in the low proliferative index group and 55% in the high proliferative index group. A multivariate regression analysis incorporating commonly used clinical prognostic factors confirmed the independent effect of proliferation index on survival. Moreover, all of the 16 IBL cases showed Ki-67 positivity of 50% or higher, which correlates with the poor clinical outcome compared to 70.7% of DLC (p=0.014). We conclude that subdivision of the diffuse large B-cell lymphoma category of the REAL classification is necessary in terms of prognostic significance in correlation with Ki-67 proliferative index.