Clinicopathological characteristics and prognosis of patients with diffuse large B-cell lymphoma / 白血病·淋巴瘤

Objective:To investigate the clinicopathological and molecular genetic characteristics and prognosis of patients with diffuse large B-cell lymphoma (DLBCL).Methods:The clinicopathological data of 152 DLBCL patients receiving consultation and routine physical examination in Peking University Third Hospital and Peking University School of Basic Medicine from January 2008 to December 2015 were retrospectively analyzed. Immunohistochemistry was used to detect the expressions of CD10, bcl-6, MUM1, GCET1, FOXP1. EB virus encoded small RNA (EBV-EBER) was detected by using in situ hybridization. The aberrations of bcl-2, bcl-6 and c-myc genes were detected by using fluorescence in situ hybridization (FISH) to screen double-hit lymphoma (DHL). Kaplan-Meier method was used to make survival analysis.Results:Among 152 cases of DLBCL, the ratio of male to female was 1.49:1, the median age of onset was 59 years (7-90 years), and 79 cases (52.0%) were primary lymph nodes. The median overall survival (OS) time of all cases was 16 months (1-101 months). The 1-year, 3-year and 5-year OS rates were 70.2%, 44.7%, 30.3%, respectively. The OS of R-CHOP treatment group was better than that of CHOP treatment group and untreated group ( P = 0.001). Among all 137 patients receiving double-hit histochemistry score (DHS), there were 56 cases with 0 score, 57 cases with 1 score, 24 cases with 2 scores; and the difference in the OS of different DHS score groups ( P = 0.311). FISH detection showed that among 29 cases achieving results of c-myc gene detection, there were 2 cases of splitting gene and 3 cases of gene amplification; among 26 cases achieving results of bcl-2 gene detection, 2 cases had bcl-2 gene amplification; among 26 cases achieving results of bcl-6 gene detection, 2 cases had bcl-6 gene amplification and 3 cases had splitting gene. It was found that myc and bcl-2 genes were amplified simultaneously in 1 case, accompanied with bcl-6 gene splitting, which was called triple-hit lymphoma. In DHS 0-score group, 1 case of double gene abnormality was found, and 1 case of single gene abnormality was found in group 1-score; in group 2-score, 5 cases were single gene abnormality and 1 case was three gene abnormality, so the gene abnormality was inconsistent with the protein expression. Conclusions:The incidence of DHL in DLBCL patients in China is low. The major gene abnormalities are c-myc or bcl-2, bcl-6 single gene abnormalities.

Progress of leukemia with NUTM1 gene rearrangement / 白血病·淋巴瘤

Molecular typing of leukemia is the basis of risk assessment and treatment options. NUTM1 gene (15q14) rearrangement is a novel molecular type of acute B lymphoblastic leukemia (B-ALL), which is mainly found in children (≥1 year old) and infants (< 1 year old). The number of patients is slightly more in children than infants. However, in infantile ALL, NUTM1 rearrangement is the second most common molecular abnormality. These children respond well to conventional chemotherapy regimens and with a good prognosis. The number of leukemia patients with NUTM1 gene rearrangement is still small, and there is no relevant study or case report in China. NUT protein encoded by NUTM1 gene is a chromatin regulator, which is related to histone acetylation regulation and chromatin remodeling. This article aims to introduce the clinicopathological features, detection methods, possible tumorigenic mechanisms and therapeutic prospects of leukemia with NUTM1 gene rearrangement, to increase the understanding of this type of leukemia and provide reference for the precise molecular subtyping and treatment.

Clinical application of NanoString fluorescent barcode technology in the molecular subtyping of patients with diffuse large B-cell lymphoma / 白血病·淋巴瘤

Objective:To explore the clinical application of NanoString fluorescent barcode technology in the molecular subtyping of diffuse large B-cell lymphoma (DLBCL), and to analyze the correlation between the cell-of-origin subtype and prognosis of patients.Methods:The tumor tissue samples of 12 patients with DLBCL at the Third People's Hospital of Datong of Shanxi Province and 8 patients with DLBCL at Peking University, Health Science Center between January 2014 and December 2019 were collected. According to Hans algorithm, all patients were divided into 1 case of germinal center-derived B-cell (GCB) type and 19 cases of non-GCB type. NanoString platform was used to analyze the expression level differences of 15 genes-related to Lymph2Cx molecular subtyping of all samples at mRNA level. Hierarchical clustering was used to subgroup 20 DLBCL cases and to contrast the prognosis in different subgroups according to the subtyping.Results:NanoString fluorescent barcode technology was used to detect samples of 20 DLBCL cases and hierarchical clustering analysis was performed, and then subtyping results showed that 11 cases were GCB-like type and 9 cases were activated B cell (ABC)-like type. Based on Hans algorithm, 10 GCB-like cases were non-GCB type. According to the survival analysis, GCB-like group had a better overall survival compared with that in ABC-like group ( P=0.019). Conclusion:NanoString fluorescent barcode technology can be successfully applied to the cell-of-origin subtyping of DLBCL, and the molecular subtyping strategy can effectively predict the prognosis of patients.

Clinicopathological features of liposarcoma of the extremities: analysis of 14 cases / 肿瘤研究与临床

Objective:To investigate the clinicopathological characteristics, diagnosis and differential diagnosis of liposarcoma of the extremities.Methods:Fourteen patients with atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL), dedifferentiated liposarcoma (DDL) and myxoid liposarcoma (ML) in Beijing Tiantan Hospital of Capital Medical University from March 2002 to December 2019 were collected. Fluorescence in situ hybridization (FISH) and immunohistochemical staining were used to detect the MDM2 gene amplification and expressions of S-100, CDK4 and MDM2 proteins in tumor tissues. The characteristics of different types of liposarcoma were comprehensively analyzed combined with clinical data and histopathological characteristics.Results:The age of 14 patients was (55.6±16.7) years old, with 10 males and 4 females. There were 2 cases of ALT/WDL, 6 cases of DDL, and 6 cases of ML. Immunohistochemistry showed that S-100 was positive in all patients and the patterns were scattered, partial and extensive. CDK4 and MDM2 were positive in both ALT/WDL and DDL, and weakly expressed in ML (4 cases). Vimentin was positive in all patients. MDM2 gene amplification was detected in 2 cases of ALT/WDL and 6 cases of DDL.Conclusion:Liposarcoma has a high incidence and obvious morphological diversity, accurate diagnosis is critical to the prognosis and treatment of the tumor.

Misdiagnosis as diffuse large B-cell lymphoma: clinicopathological analyses of 431 patients / 肿瘤研究与临床

Objective To analyze the cases that are easily misdiagnosed as diffuse large B-cell lymphoma (DLBCL),and to explore the diagnosis,differential diagnosis and clinicopathological features of DLBCL.Methods The clinicopathological data of 431 cases preliminarily diagnosed as DLBCL in the Department of Pathology,School of Basic Medical Science,Peking University from October 2016 to December 2017 were retrospectively analyzed.Further immunohistochemical staining and molecular biological tests were performed if necessary.Results The diagnostic accuracy rate of DLBCL was 88.86％ (383/431).Misdiagnosis mainly occurred in follicular lymphoma (36 cases,8.35％),high-grade B-cell lymphoma (HGBL) with gene rearrangement of c-myc and bcl-2 or bcl-6 (4 cases,0.93％),atypical lymphoid tissue hyperplasia (3 cases,0.70％),mantle cell lymphoma (3 cases,0.70％),classic Hodgkin lymphoma (CHL) (1 case,0.23％) and composite lymphoma (DLBCL and CHL) (1 case,0.23％).Except the poor quality of sections,the lack of immunohistochemical markers,the poor knowledge of the diagnosis of DLBCL and high-grade FL as well as HGBL were the main reasons of misdiagnosis.Conclusions The diagnosis and differential diagnosis of DLBCL is based on morphology,and it needs to combine with the corresponding immunohistological markers and molecular biological detection when necessary.The recognition of clinical and pathological features of various types of misdiagnosed lymphomas should be strengthened.

Posttransplant lymphoproliferative disorders: a clinicopathological analysis of 11 cases / 肿瘤研究与临床

Objective@#To analyze the clinicopathological features of the posttransplant lymphoproliferative disorders (PTLD) and to improve the diagnostic levels.@*Methods@#The clinical data of 11 patients diagnosed with PTLD between January 2008 and January 2018 from Henan Provincial People's Hospital, Peking University Science Center and the Affiliated Third Hospital of Peking University were collected. The clinicopathological features and the potential prognostic predictors were retrospectively analyzed by using immunohistochemical staining, EB virus in situ hybridization, fluorescence in situ hybridization and gene sequencing.@*Results@#There were 9 males and 2 females in 11 PTLD patients, and the median age of the total patients was 18 years old (3-34 years old). The median time of 9 cases who underwent hematopoietic stem cell transplantation developing PTLD was 4 months (2-24 months) after the transplantation. The other 2 cases undergoing solid organ transplantation (SOT) occurred PTLD after 6 months and 13 months, respectively. The lymph node was the most common site to be involved (9 cases), 1 case occurred in liver and 1 case occurred in nasopharynx site. Among 11 patients, 3 cases were classified as polymorphic PTLD (P-PTLD) and the other 8 cases were monomorphic PTLD (M-PTLD). EB virus of all cases was positive, and 8 cases of M-PTLD were classified as diffuse large B-cell lymphoma (DLBCL). Fluorescence in situ hybridization was used to detect bcl-2, myc, IGH and A20 gene, and only one case had the gene break of IGH, while other cases didn't find any other abnormalities. Ig gene clone analysis was made in 5 patients with PTLD, including 4 cases of M-PTLD with gene rearrangement and 1 case of P-PTLD without gene rearrangement. Univariate analysis showed that age (≤18 years old) was associated with poor prognosis (P = 0.040).@*Conclusions@#The clinicopathologic features of PTLD are various and infected by EB virus. Gene rearrangement can help the diagnosis.

Association between radiographic and clinicopathological features and prognosis in patients with extranodal NK/T cell lymphoma / 肿瘤研究与临床

Objective To explore the CT and MRI imaging and clinicopathological features of extranodal NK/T cell lymphoma (NK/TCL). Methods Sixty-six patients with NK/TCL diagnosed from 2002 June to 2016 April in Beijing Tongren Hospital with intact CT and/or MRI imaging results were enrolled in this study. All the patients had tailed clinical information and follow-up. The imaging and clinicopathological features were analyzed retrospectively and their prognostic value on overall survival was analyzed. Results There were 49 males and 17 females with median age of 42 years. The median follow-up time was 18 months. The cases showed surrounding invasions including 10 cases (15.2 %) in soft palate, 5 cases (7.6 %) in hard palate, 2 cases(3.0 %) in tonsil, 8 cases(12.1 %) in upper lip, 13 cases(19.7 %) in maxillofacial soft tissue, 9 cases (13.6 %) in eyelid, 10 cases (15.2 %) in orbital, 3 cases (4.5 %) in maxilla, 6 cases (9.1 %) in pterygopalatine fossa,6 cases(9.1 %)in infratemporal fossa,3 cases(4.5 %)in skull base, 3 cases(4.5 %) in eyeball and 2 cases (3.0 %) in brain tissue. Kaplan-Meier survival analysis found that the 2-year overall survival rates of the patients with the involvement of hard palate, upper lip, maxillofacial soft tissue, eyelid, orbital, maxillary, eyeball and brain organizer were lower than those of the patients without the involvement of these sites(χ2values were 4.470,4.041,4.456,13.933,8.986,4.000,44.121,6.527,16.822,respectively, all P< 0.05). Further multivariate Cox regression analysis showed that maxilla and brain involvement were independent adverse factors (RR=34.717, 95 % CI 3.404-354.035, P=0.003; RR=37.545, 95 % CI 3.188-442.187, P= 0.004). Conclusions MRI and CT examinations are of great value in diagnosis and prognostic assessment of NK/TCL. Clinicians can make correct and timely diagnosis by comprehensive clinical, radiological and pathological features and can make a detailed clinical assessment to give patients appropriate treatment,thus improving the outcome of the NK/TCL patients.

Study on the relationship between protein expression and gene abnormalities of myc, bcl-2 and bcl-6in diffuse large B-cell lymphoma / 白血病·淋巴瘤

Objective To study the frequency of diffuse large B-cell lymphoma (DLBCL) with multi-genetic alteration, and its correlation with c-myc, bcl-2 and bcl-6 protein expression. Methods 50 cases diagnosed with DLBCL from January 2012 to December 2016 were collected. The expression of c-myc, bcl-2 and bcl-6 was analyzed by immunohistochemistry. Interphase fluorescence in situ hybridization (I-FISH) analysis was performed to identify the genetic alteration of c-myc, bcl-2 and bcl-6. Results In all cases, there were 27 males and 23 females with a median age of 50 years (range: 3-85 years). 23 (46.00 %) cases were defined as primary nodal DLBCL and 27 (54.00 %) cases were primary extra-nodal DLBCL, with gastrointestinal tract (48.15 %, 13/27) being the most common site of involvement. c-myc protein expression was detected in 94.00 % (47/50) cases, in which 82.00 % (41/47) cases exhibited high levels of c-myc expression with positive nuclear staining observed in over 40.00 % of tumor cells. The positive rate of bcl-2 protein was 84.00 % (42/50), 76 % (38/50) cases presented with high-level bcl-2 expression. Concurrent high expression of c-myc and bcl-2 were presented in 18 cases (36.00%). FISH analysis demonstrated c-myc gene rearrangement in 7 cases (14.00 %) and amplification in 2 cases (4.00 %). bcl-2 gene rearrangement was detected in 6 cases (12.00 %) and 4 cases (8.00 %) exhibited gene amplification. bcl-6 gene rearrangement was identified in 8 cases (16.00%), amplification in 3 cases (6.00%), and 1 case concomitantly harbored the rearrangement and amplification of bcl-6. Multi-genetic alterations were defined in 4 cases with 3 cases fulfilling the criteria for double-hit lymphoma (DHL) and 1 case for triple-hit lymphoma (THL). For the cases with concomitant high-level expression of c-myc and bcl-2 proteins, 3 cases (16.67 %) was detected with multi-genetic alterations, including 2 cases for DHL and 1 case for THL. Conclusions The proportion of DLBCL with multi-genetic alterations is 8.00 % in this study. The genetic alterations are not consistently correlated with the protein expression. The molecular genetic testing is reliable for the identification of DHL.

Analysis of clinicopathological features of 21 patients with enteropathy-associated T-cell lymphoma / 肿瘤研究与临床

Objective To investigate the clinicopathological and prognosis features of enteropathy-associated T-cell lymphoma (EATL).Methods 21 cases of EATL,6 cases of peripheral T-cell lymphoma (PTCL) and 11 cases of natural-killer/T-cell lymphoma (NKTCL) were collected from January 2008 to May 2015.The immunophenotype of the tumor cell was tested by EnVision and as well as EBV-EBER for EB virus.Some patients were performed with follow-up data.Results 21 EATL patients included 14 males,7 females and the middle age was 55 years old (40-79 years old).15 patients affected the small bowel,4 cases affected colon,2 cases affected more than one site.18 cases were mono-morpholohic EATL while 3 cases were classical EATL.The expression rates of neoplastic cells for CD3ε,CD4,CD8,CD56,Granzyme B,TIA-1 were 95.24 ％ (20/21),20.00 ％ (3/15),73.68 ％ (14/19),85.71％ (18/21),64.71％ (11/17),88.89 ％ (16/18) respectively.The expression of EBER in EATL patients (0,0/21) was obviously lower than that in NKTCL patients (100 ％,11/11).17 EATL patients had follow-up data,and the middle survival time was 15 months.No different prognosis was found in the three kinds of T-NHL (P =0.697).Conclusions EATL usually occurs in elder male and jejunum.The diagnosis of EATL needs a lot of information,including clinical history,endoscopy,histomorphology,immunophenotype and EBV-EBER result.EATL has low mobidity and high malignancy,it still lacks impactful therapeutic regimen.

Review of the 2016 WHO classification of lymphoid neoplasms / 白血病·淋巴瘤

There are some major changes win the revised 2016 WHO Classification of Lymphoid Neoplasms.Based on the clinicopathological changes and genetic/molecular findings in the past years,the new classification clarified the diagnosis and clinical management of some very early stages of lymphoproliferative disorders,refined the diagnostic criteria for some lymphoid neoplasms,and further emphasized the significance of genetic/molecular studies in the diagnosis and clinical treatment of lymphomas.A small number of new provisional entities were added to the 2016 edition.

Expression of microRNA-191 in T lymphoblastic leukemia/lymphoma and its underlying mechanism / 中华血液学杂志

<p><b>OBJECTIVE</b>To evaluate the correlation between MicroRNA-191 (miR-191) and T lymphoblastic leukemia/lymphoma (T-ALL/LBL) to probe its underlying molecular mechanism.</p><p><b>METHODS</b>The expression of miR-191 was examined by real-time PCR (RT-PCR) in 20 T-ALL/LBL tissue samples and 20 lymphoid reactive hyperplasia (LRH) tissue samples. The correlation between miR-191 and the clinicopathological feature of T-ALL/LBL was analyzed. Antisense miR-191 lentiviral vectors was constructed and transfected into T-ALL/LBL Jukat cells. After transfection, the expression of miR-191 was examined by RT-PCR. The cell activity was evaluated by CCK-8 asssy. The cell cycle and apoptosis were determined by flow cytometry.</p><p><b>RESULTS</b>Compared with LRH samples, the results of RT-PCR showed significant upregulation of miR-191 in 20 T-ALL/LBL tissue samples (1.875±0.079 vs 1.000, P<0.05). The expression level of miR-191 was negatively associated with prognosis. Compared with LV-NC-GFP and control groups, the expression of miR-191 significantly decreased after transfection of antisense miR-191 lentiviral vectors (0.578±0.012 vs 1.011±0.053 and 1.000, P<0.05), the percentages of apoptotic cells and the cell in G0/G1 phase significantly increased (P<0.05).</p><p><b>CONCLUSIONS</b>miR-191 might play a significant role in the development of T-ALL/LBL, implicating a new target for therapy.</p>

Characteristics and prognosis of EBV-positive gastric diffuse large B-cell lymphoma / 中国肿瘤临床

Objective:To investigate pathological characteristics and prognosis of patients with Epstein-Barr virus (EBV) positive gastric diffuse large B-cell lymphoma (DLBCL). Methods:Through retrospective study, we collected 75 cases of patients with DLBCL that oc-curs in the stomach. The patients were divided into two groups consisting of 60 cases of EBV negative control group and 15 cases of EBV positive group. To analyze the pathological characteristics and prognosis of patients with EBV positive gastric DLBCL, immunohisto-chemical and Epstein-Barr encoding region (EBER) in situ hybridization methods were used to detect Bcl-2, c-myc protein expression, and EBV-encoded RNA (EBER). Results:In certain aspects of clinical manifestations, such as age, gender, and origin, the comparison be-tween EBV-positive and EBV-negative groups had no statistically significant difference. The same results were obtained for Bcl-2 and c-myc protein expression. However, a statistically significant difference (P=0.01) was observed under the R-CHOP regimen where the me-dian overall survival (OS) of the EBV-positive and EBV-negative groups were 15.1 and 31.4 months, respectively. Conclusion:In pa-tients with DLBCL of the stomach, the EBV infection had no obvious effects in terms of clinical manifestation, origin, morphology, and protein expression of tumor cells. EBV-positive DLBCL was not limited to elderly patients. Under the R-CHOP regimen, the prognosis of EBV-positive patients was worse than that of EBV-negative patients.

Testicular lymphoma: a clinicopathological study of 65 cases / 中华血液学杂志

<p><b>OBJECTIVE</b>To summarize the clinicopathological features of testicular lymphomas (TL).</p><p><b>METHODS</b>The medical records of 65 patients diagnosed with TL between 2008.1.1 and 2014.11.30 were retrospectively reviewed.</p><p><b>RESULTS</b>TL was classified as primary (PTL) when there's no prior diagnosis of an extara-testicular lymphoma/leukemia and no concurrent widespread disease, except for the concomitant involvement of ipsilateral inguinal lymph nodes; otherwise it was classified as secondary (STL). Of our patients group, 46 (70.8%) cases were classified primary TL as and the other 19 (29.2%） cases were secondary TL. All patients presented with painless testicular swelling. The median age of STL was significantly younger than that of PTL [65 (12-88) ys vs 13 (1-75) ys, P<0.001]. Additionally, a striking difference in the distribution of histological subtypes was observed between the PTL and STL patients group.</p><p><b>CONCLUSION</b>Primary TLs were more common than secondary. Striking differences in the distribution of patients'age and histology were found between STL and PTL.</p>

Expression and prognostic value of COX- 2, p16(INK4A) and p53 in patients with classical Hodgkin lymphoma / 中华血液学杂志

<p><b>OBJECTIVE</b>To investigate the expression level of COX-2, p16(INK4A) and p53 in patients with classic Hodgkin's lymphoma (cHL), and to evaluate their correlation with prognosis.</p><p><b>METHODS</b>The clinical data and samples of 52 cHL cases were collected. Immunohistochemical staining was performed to analyze the proteins level mentioned above and in situ hybridization of EBV encoded RNA (EBER) to clarify the tumor EBV infection state. Correlation between the protein expression and prognosis of patients was analyzed.</p><p><b>RESULTS</b>Of 52 cases, the male and female ratio was 1.6∶1, the age was from 22 to 68 years old. All lesions located primarily in lymph nodes. All samples from 52 cases were stained with COX-2, p16(INK4A) and p53, and the positive expression of COX-2 was found in 28 cases (53.8%）, that of p16(INK4A) in 25 cases (48.1%)and p53 in 42 cases (80.8%）. All patients were divided into two groups according to differences in age (<40 years/ ≥ 40 years), gender (male/female), EBV infection (yes/no), B symptoms (yes/no), and the Ann Arbor staging (Ⅰ-Ⅱ/Ⅲ-Ⅳ), the correlation with COX-2, p16(INK4A) and p53 expression were analyzed, and only p53 expression was correlated with Ann Arbor staging (P=0.027). The statistical analysis of correlations between COX- 2, p16(INK4A) and p53 showed that the expression of COX-2 was strongly correlated with p53 (P=0.008), and p16 (INK4A) was not related to either COX-2 or p53 (P=0.246 and 0.958). Kaplan- Meier univariate OS analysis using SPSS17.0 software showed that only COX-2 expression was an adverse prognostic factor for patients'event free survival (EFS) (P=0.003). Meanwhile COX-2 expression was a unique independent prognostic factor analyzed by COX proportional hazards regression model (HR=0.091, 95% CI 0.017-0.505, P=0.006).</p><p><b>CONCLUSION</b>The expression rate of COX-2, p16 (INK4A) and p53 in the cHL were relatively high; and they were not statistically correlated with tumor EBV infection status; the COX-2 positive group had poor prognosis, but only event free survival time becomes statistically significant shorter. COX proportional hazard regression model was used to analyze the COX-2 expression as a independent adverse prognostic factors for EFS.</p>

Retrospective analysis of EB virus infection in patients with angioimmunoblastic T-cell lymphoma and its clinical significance / 白血病·淋巴瘤

Objective To investigate the EB virus (EBV) infection and its clinical significance in angioimmunoblastic T-cell lymphoma(AITCL).Methods 62 patients diagnosed as AITCL between 2008 Jan and 2011 Dec were retrospective analyzed.All cases were re-confirmed and classified with the histology and immunology according to 2008 WHO Classification.In situ hybridization of EBV encoded RNA (EBV-EBER) was performed.Clinical characteristics and follow-up data of patients were collected.Results 42 ％ (26/62) AITCL cases were EBER-positive.EBV infection was found to be significant correlation with age over 60 years and poor response to therapy (P =0.025,P =0.049,respectively).However,EBV infection had no relationship with the overall survival,the presence of B symptom and the Ann Arbor stage.Conclusion In AITCL,EBV infection seems not to be associated with the overall survival,B symptom and Ann Arbor stage,but it may have impacts on theraputic response.

Analysis of clinical pathological characteristics and prognosis of elderly patients with EB virus-positive diffuse large B-cell lymphoma / 白血病·淋巴瘤

Objective To analyze the clinical pathological characteristics and prognosis of elderly patients with EB virus-positive diffuse large B-cell lymphoma (EBV+ DLBCL).Methods 24 elderly patients with EBV + DLBCL were collected to evaluate their clinical pathological characteristics and prognosis by comparison with the EBV-DLBCL,NOS during the same period.Results 24 EBV + DLBCL cases demonstrated two morphologic subtypes:polymorphic and monomorphic.And polymorphic subtype showed geographic necrosis more frequently than that in monomorphic subtype.According to Hans and Choi models,the majority of EBV+ DLBCL of the elderly were classified as non-GCB subtype (91.3 ％ and 100.0 ％,respectively).55.0 ％ cases showed CD30 positive,which was significantly higher than that in EBV-DLBCL group (P ＜ 0.001).Under the treatment of R-CHOP regimen,the overall survival (OS) of the elderly EBV+ DLBCL patients showed no significant difference with the ＞50-year old EBV-DLBCL patients (the median OS were 44.2 months and 29.2 months,P =0.587).Conclusions The elderly EBV + DLBCL patients are normally presented with polymorphic and monomorphic patterns.And geographic necrosis are often seen in polymorphic cases.CD30 expression and non-GCB subtypes are high.With the R-CHOP regimen,the OS of the elderly EBV+ DLBCL patients is similar with that of ＞50-year old EBV-DLBCL patients.

Clinicopathologic features and prognosis of primary bone anaplastic large cell lymphoma / 中华病理学杂志

<p><b>OBJECTIVE</b>To study the clinicopathologic features, differential diagnosis and prognosis of primary bone anaplastic large cell lymphoma(ALCL).</p><p><b>METHODS</b>Twelve patients diagnosed with primary bone ALCL were retrospectively reviewed. The clinicopathologic features, immunohistochemic findings and results of in situ hybridization for EB virus were analyzed.</p><p><b>RESULTS</b>Of the 12 patients, the male-to-female was 7: 5 with a median age of 17.5 years (range from 9 to 64 years). Bone pain was the presenting symptom in all patients. Radiographic examination demonstrated solitary osteolytic lesion in 8 patients and multiple lesions in the rest 4 patients. Spine (7 cases) was the most common site to be involved, followed by ilium (5 cases), sacrum (2 cases), humerus (1 case) and collarbone (1 case). Ten patients were available with the follow-up data including 5 ALK-positive and 5 ALK-negative patients, and the follow-up time was 2 to 47 months. Interestingly, the 3 dead patients were ALK-negative whereas 5 of 7 ALK-positive patients achieved remission.</p><p><b>CONCLUSIONS</b>Primary bone ALCL is a rare type of non-Hodgkin lymphoma and it more frequently involves the axial skeleton. Boys and young males are more commonly affected. Patients usually present at an early stage and have a relatively favorable prognosis. Expression of ALK protein may be associated with a favorable prognosis in primary bone ALCL.</p>

Clinicopathological analysis of 64 case of angioimmunoblastic T-cell lymphoma / 中华血液学杂志

<p><b>OBJECTIVE</b>To explore the clinical and pathological characteristics of angioimmunoblastic T-cell lymphoma (AITL).</p><p><b>METHODS</b>Sixty-four cases of AITL were retrospectively analyzed by histopathological and immunohistochemical methods.</p><p><b>RESULTS</b>There were 35 men and 29 women, the median age was 59 years (range, 25-84 ys). AITL typically presented with advanced stage, generalized lymphadenopathy, hepatosplenomegaly and systemic symptoms. Morphologically, the lymph nodes showed partial or total obliteration of the normal architecture by a polymorphic infiltration of lymphocytes, and by proliferation of follicular dendritic cells and that of high endothelial venules. Most cases contained a monoclonal T-cell population as well as clonal cytogenetic abnormalities. Immunophenotype analysis showed that neoplastic cells expressed the following markers: CXCL13 (positive rate 95.3%), PD-1 (positive rate 75.0%), CD10 (positive rate 25.0%), Bcl- 6 (positive rate 40.0%), CD2 (positive rate 96.0%), CD3 (positive rate 95.0%), CD4 (positive rate 84.0%), CD5 (positive rate 73.0%), EBER (positive rate 39.5%) and Ki-67 (average positive rate 55.0%), and frequently showed aberrant loss or reduced expression of CD7 and CD8.</p><p><b>CONCLUSION</b>The neoplastic cells of AITL showed features of CD4+ TFH, with peculiar clinical features. Peripheral T-cell lymphomas with a follicular growth pattern may show overlapping features with focal AITL.</p>

Clinicopathological analysis of 273 cases of primary intestinal non-Hodgkin's lymphoma / 中华血液学杂志

<p><b>OBJECTIVE</b>To study the clinicopathological characteristics and prognostic factors in Chinese patients with primary intestinal non-Hodgkin's lymphomas (PINHL).</p><p><b>METHODS</b>The clinical symptoms, pathological features, diagnostic and prognostic factors of 273 cases diagnosed with PINHL from our center were analyzed.</p><p><b>RESULTS</b>Among 273 cases, 189 were male and 84 female, the male to female ratio was 2.3:1. The age of patients ranged from 2 to 85 years old with the median age of 46. The most frequent site of the lesions was ileocecus (n=83, 30.4%). The clinical symptoms of PINHL were unspecific with abdominal mass frequently seen in B-cell lymphoma, and perforation, hypogastric pain and "B" symptoms more common in T-cell lymphoma. Endoscopic biopsy diagnosis rate was 90.3%. Of 273 cases, B-cell lymphoma (n=232, 85.0%) dominated PINHL with the most common subtype of diffuse large B-cell lymphoma-not otherwise specified (DLBCL-NOS) (n=132, 48.4%), while the T-cell lymphoma (n=41, 15.0%) were much less seen with the most common subtype of enteropathy-associated T-cell lymphoma (EATL) (n=15, 36.6%). There were 245 cases were followed up, including 206 cases of B-cell lymphoma and 39 cases of T-cell lymphoma, it was found that the prognosis of B-cell lymphoma is much better than that of T-cell lymphoma (P<0.05). Operation had no significant effect for the overall survival rate. But for patients with aggressive lymphoma, operation can improve the survival rate.</p><p><b>CONCLUSION</b>It indicates that PINHL often occurs as B cell type, DLBCL-NOS is the most common histological type. Ileocecus is the most common site involved and enteroscope biopsy is a good method of diagnosis. Compared with T-cell lymphoma, B-cell lymphoma has different clinical manifestations and a better prognosis. Patients with aggressive lymphoma can benefit from operation.</p>

Primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome in a child / 中华皮肤科杂志

A 13-year-old girl presented with multiple recurrent cutaneous plaques for more than six months,which had been aggravated with intermittent fever for five months.No obvious systemic abnormality was found.Dermatological examination revealed multiple,non-ulcerative,painless,infiltrative,indurated,poorly marginated,purple subcutaneous plaques measuring 3-1 1 cm in diameter with slight squamation in bilateral buttocks and lower limbs.Laboratory investigations showed bicytopenia with the white blood cell count being (0.03-3.7) × 109/L and red blood cell count being (2.8-4.4) × 1012/L,a normal platelet count,hypofibrinogenemia (1.79 g/L) and low proportion of natural killer cells (4.6％).Bone marrow smear showed active proliferation of cells,decreased proportion of granulocytes,presence of a few indefinitely classified cells,and phagocytosis.Reticulocytes were easily seen in the bone marrow smear.Pathologically,no obvious abnormality was observed in the epidermis or dermis,while the subcutaneous adipose tissue,especially fat lobules and some interlobular septa,was extensively infiltrated by large-to medium-sized lymphoid cells with pleomorphic and twisted nuclei as well as a small amount of cytoplasm; necrosis and phagocytosis of nuclear debris and lymphocytes were visible.The atypical lymphoid cells stained positive for CD3,T-cell intracellular antigen-1,granzyme B and TCRγδ with partial loss of CD5 and CD7,but negative for CD56,CD4,CD8 and TCRαβ.No Epstein-Barr virus-encoded RNA (EBER) was detected by in situ hybridization.Based on these findings,a diagnosis of primary cutaneous γδ-T cell lymphoma with hemophagocytic syndrome was made.