1.Clinicopathological features of primary mucosal CD30-positive T-cell lymphoproliferative disorders
Na DONG ; Lina ZHANG ; Yuanyuan ZHENG ; Yanlin ZHANG ; Xiaoge ZHOU ; Jianlan XIE
Chinese Journal of Pathology 2024;53(7):667-671
Objective:To investigate the clinicopathological features and differential diagnosis of primary mucosal CD30-positive T-cell lymphoproliferative disorders (pmCD30 +TLPD). Methods:Eight cases of pmCD30 +TLPD diagnosed from 2013 to 2023 at the Department of Pathology, Beijing Friendship Hospital Affiliated to Capital Medical University and Beijing Ludaopei Hospital were retrospectively collected. The immunophenotype, EBV infection status and T-cell receptor (TCR) clonability of tumor cells were examined. The clinicopathological features were analyzed and related literatures were reviewed. Results:There were 5 females and 3 males, aged 28 to 73 years, without B symptoms, lack of trauma and autoimmune diseases. Seven cases occurred in oral mucosa and one in anal canal mucosa. Submucosal nodules with ulcerations were presented in all cases except one, which only submucosal nodule. Morphologically, there was different distribution of allotypic lymphocytes in inflammatory background. Four cases showed “kidney-shaped”, “embryonic” and “horseshoe-shaped” cells, and one case resembled Hodgkin and Reed/Sternberg (HRS) cells. Allotypic lymphocytes expressed CD3 (7/8), CD4+/CD8-(7/8) and CD4-/CD8-(1/8). CD30 was uniformly strongly positive while ALK and CD56 were negative. In situ hybridization of EBER was negative in five cases (5/5). Clonal TCR gene rearrangement was positive in two cases. Four patients did not receive radiotherapy or chemotherapy. All the seven patients survived without disease except one died due to concurrent leukopenia.Conclusions:pmCD30 +TLPD had a broad morphological spectrum and could be easily confused with primary cutaneous CD30 +TLPD and systemic ALK-negative anaplastic large cell lymphoma involving mucosa, which may lead to misdiagnosis. Although the majority of the cases had a favorable prognosis, a few cases relapsed or progressed to lymphoma.
2.Expression of CXCR5 and its significance of clinical and pathological in classical Hodgkin lymphoma
Zhixiong XIA ; Xiaoge ZHOU ; Yuanyuan ZHENG ; Yanlin ZHANG ; Jianlan XIE
Chinese Journal of Clinical and Experimental Pathology 2024;40(1):35-39
Purpose To investigate the expression of Che-mokine(C-X-C Motif)receptor 5(CXCR5)and its clinico-pathological significance in classic Hodgkin lymphoma(CHL).Methods The expression of CXCR5 was assessed in 33 pa-tients by immunohistochemistry(IHC),and retrospectively ana-lyzed the expression and clinical significance of CXCR5 in the four subtypes of CHL.Meanwhile,10 cases of ALK-positive an-aplastic large cell lymphoma(ALCL)and 10 cases of ALK-neg-ative ALCL were collected as the control group.ResultsThere were 31 cases with CXCR5-positive in all 33 cases(93.94%),including 15/16(93.75%)in nodular sclerosis CHL,12/13(92.31%)in mixed cellularity CHL,2/2 in lymphocyte-rich CHL,and 2/2 in lymphocyte-depleted CHL.The positive ex-pressions of CXCR5 in different immunophenotypes of CHL were as follow,31/33(93.94%)in CD30 positive and PAX5 weakly positive CHL.12/14(85.71%)in CD15 negative CHL,24/26(92.31%)in CD20 negative CHL,10/11(90.91%)in EBER-negative CHL and 5/6 in LMP1-negative CHL.CXCR5 were not expressed in all 20 cases of ALCL.Conclusion The positive expression rate of CXCR5 in CHL is high.When the tumor cells are negative for CD15,LMP1 and CD20 or EBER,CXCR5 also has a high positive expression rate,which is helpful for the diagnosis of CHL.CXCR5 can be used to differentiate CHL from ALCL,especially the cases lacking typical morpholo-gy and immunohistochemistry.
3.Translocations of MYC, bcl-2 and bcl-6 genes and Epstein-Barr virus infection in primary cardiac large B-cell lymphoma
Jianlan XIE ; Xiaojing TENG ; Yuanyuan ZHENG ; Yanlin ZHANG ; Xiaoge ZHOU
Chinese Journal of Pathology 2021;50(7):785-790
Objective:To investigate the translocations of MYC, bcl-2 and bcl-6 genes, the Epstein-Barr virus (EBV) status and the clinicopathological features of primary cardiac large B cell lymphoma (LBCL).Methods:Seven cases of primary cardiac LBCL were collected at Beijing Friendship Hospital, Capital Medical University, China from February 2013 to May 2019. The clinical feature, pathological morphology and immunophenotype were analyzed. The detections of EBV and gene rearrangements of MYC, bcl-2 and bcl-6 were conducted. The 2017 WHO classification of tumors of haematopoietic and lymphoid tissues was used to classify the tumors.Results:Four patients with right atrial lesions showed diffuse infiltration of medium size lymphoid cells with small vascular hyperplasia, without evidence of EBV infection. Without detectable gene rearrangements of MYC and bcl-2, 2 of the patients showed bcl-6 gene break-apart. The diagnosis was revised from diffuse LBCL to high-grade B-cell lymphoma, not otherwise specified (HGBL-NOS). There was a case of CD5 + diffuse LBCL involving the right atrium and ventricle and 2 cases of fibrin-associated diffuse LBCL located at left atrium without gene rearrangements of MYC, bcl-2 and bcl-6. However, EBER and EBNA2 were highly expressed in fibrin-associated diffuse LBCL. The patients were followed up for 10-71 months. Four cases of HGBL-NOS and a case of CD5 + diffuse LBCL received R-CHOP with/without autologous stem cell transplantation. All but two patients survived. Two cases of fibrin-associated diffuse LBCL were disease free without adjuvant chemotherapy and radiotherapy. Conclusions:Primary cardiac LBCL is heterogeneous, including at least HGBL-NOS. Primary cardiac HGBL-NOS most frequently occurs in the right atrium. Tumor cells of primary cardiac LBCL have the morphological characteristics similar to Burkitt lymphoma, lacking MYC and bcl-2 gene rearrangements, but usually show bcl-6 gene disruption. Fibrin-associated diffuse LBCL has a good prognosis and postoperative chemotherapy seems unnecessary.
4.Clinicopathological features and prognosis of the skeletal-muscle cytotoxic T-cell lymphoma
Weihua HOU ; Xiaoge ZHOU ; Jianlan XIE ; Yuanyuan ZHENG ; Yanlin ZHANG ; Xu WANG
Chinese Journal of Pathology 2021;50(11):1246-1251
Objective:To investigate the clinicopathological features and prognosis of skeletal-muscle cytotoxic T-cell lymphoma (CTL).Methods:The clinical data of 14 cases of skeletal muscle CTL and 47 cases of non-skeletal muscle extranodal CTL patients in Beijing Friendship Hospital Affiliated to Capital Medical University and the 989 Hospital of the joint logistics support force of the people′s Liberation Army (the former 152 hospital) from 2008 to 2019 were collected retrospectively. Immunophenotype, EBV infection status and T-cell receptor (TCR) clonality of tumor cells were evaluated. The clinicopathological features and prognosis of the two groups were compared.Results:Skeletal-muscle CTL accounted for 23.0% (14/61) of extranodal CTL in the same period. The median age of the patients was 42.3 years (range 11-76 years), including six males and eight females. The main clinical manifestations were painless masses. Two patients (2/14) had B symptoms. The tumors occurred in the cheek (7 cases), the tongue (4 cases), the lower lip (3 cases) and the left upper arm (2 cases), and in two cases had two sites. Ten cases were of stage ⅠE and four cases stage ⅡE. Compared with non-skeletal-muscle extranodal CTL, many patients of skeletal-muscle CTL did not have B symptoms, the clinical stage was lower, and the tumor mainly involved the oral cavity (cheek, tongue and lip, P<0.05). Morphologically, the tumor showed diffuse infiltration of heterotypic lymphocytes in skeletal muscle. Immunohistochemistry showed that in 11/14 cases, there were reduced or loss of expression of some the T cell antigens (CD2, CD3, CD5, CD7). TIA-1, Gr B and CD8 (CD8 +>CD4 +) were expressed in all cases, and CD56 was negative. The median Ki-67 proliferation index was 35.0% (range 5%-60%). EBER in situ hybridization was negative in all cases. The results of TCR clonality analysis showed clonal TCR gene rearrangement were detected in eight cases. The median follow-up time was 40 months (range 10-67 months). Ten patients were tumor free; the 5-year survival rate of skeletal-muscle CTL was 100%. Compared with non-skeletal-muscle extranodal CTL (5-year overall survival rate was 35.9%), the difference was statistically significant (χ2=8.277, P=0.004). Conclusions:Skeletal-muscle CTL mostly occurs in the skeletal muscle of cheek and mouth. Tumor cells show morphologic characteristics of muscle invasion and myositis-like feature. It also shows CD8 +>CD4 +immunophenotype, cytotoxic molecular pattern and is associated with low clinical stage and good prognosis.
5.Construction of a medical service experience system based on staff role play
Xiaomin CHEN ; Dongping XU ; Min DONG ; Shengchun QIU ; Jianlan ZHENG ; Xu ZHANG ; Danni ZHANG ; Jiaxin ZHOU ; Yaping ZHANG ; Dongsheng HUANG
Chinese Journal of Hospital Administration 2020;36(8):649-653
Objective:To build a medical service experience system based on role play of hospital staff, and explore its contribution to upgrading medical service, elevating medical service quality, and improving patients′ satisfaction.Methods:An indicator system for medical service experience was developed via literature review. 20 newly recruited hospital employees in 2019 were sampled randomly to form a service experience team, who were arranged to experience the full medical service process of outpatients and inpatients based on role play. The questionnaire for service quality based on patient perception and expectation was used to survey the expectation and perception values of those role players on their medical service experiences, and summarize the defects found on such six dimensions as hospital reliability, responsiveness, tangibility, assurance, empathy and cost-effectiveness. After due corrections of these defects, further experiences and patient satisfaction survey were made to assess the effects of such actions taken. Paired t test was used respectively in statistical analysis of service experience value and actual perception value, as well as the perception values before and after the actions taken, and χ2 test was used for a statistical analysis of patient satisfaction. Results:Role players experienced respectively at the emergency, outpatient and inpatient departments of the hospital for a week in August 2019. Statistics of their experiences indicated≥0.50 points difference between the mean expectation and perception values on doctor-patient communication, patient help responsiveness, outpatient process, examination report delivery duration, service attitude, patient trust on medical workers, medical environment and patient privacy protection.Following a 3-month reform, role players found improvements in such aspects as hospital reliability, responsiveness, tangibility, assurance, empathy and cost-effectiveness; patient satisfaction improved sizably in December 2019 over January of the same year( P<0.05). Conclusions:The construction and application of the medical service experience system based on role play of hospital staff prove highly useful in upgrading medical service actions, improving medical service quality, and improving patient satisfaction.
6.Clinicopathological characteristics of pediatric-type follicular lymphomas: an analysis of 37 cases
Yuanyuan ZHENG ; Jianlan XIE ; Yanlin ZHANG ; Xiaoge ZHOU
Chinese Journal of Pathology 2020;49(7):681-685
Objective:To study the clinicopathologic features, immunophenotype and prognosis of pediatric-type follicular lymphoma (PTFL).Methods:Thirty-seven cases of PTFL at the Beijing Friendship Hospital, Capital Medical University, from January 2012 to March 2018 were analyzed using light microscopy, immunohistochemistry, and polymerase chain reaction (PCR), and 13 cases were also examined using fluorescence in situ hybridization (FISH).Results:The male to female ratio was 35∶2. The median age was 16 years. Thirty-one patients were clinical stage Ⅰ and 6 were stage Ⅱ, displaying enlargement of lymph node in the head and neck regions. Follow-up information was available in 32 patients. Only two patients received low-dose chemotherapy, and none of these patients had relapse or disease progression at the latest follow-up (ranging from 16 to 79 months; median, 37 months). Morphologically, the lymph node architecture was partially or totally effaced by expansile follicles lacking polarization, with a prominent "starry sky" appearance. The cytologic composition was dominated by monotonous medium to large-sized blastoid cells with round to oval nuclei, finely clumped chromatin, small nucleoli, and scant cytoplasm. Immunophenotypically, all cases were positive for CD20, CD10, and bcl-6, but negative for bcl-2, MUM1 and C-MYC. Tumor cells were restricted to the follicles. The Ki-67 immunohistochemistry demonstrated a high proliferation (50% to 90%). Moreover, the tumor cells in the examined 28 cases were negative for CD43, CD5 and CD23. PCR analysis revealed monoclonal Ig gene rearrangements in all specimens. Thirteen cases being subjected to the FISH testing lacked bcl-2 and bcl-6 translocations.Conclusion:PTFL is different from conventional follicular lymphoma in their distinct morphology, immunophenotypic and molecular features, and behaves like an highly indolent or benign tumor.
7. Paediatric nodal marginal zone lymphoma: a clinicopathological study of seven cases
Dongyu BAI ; Jianlan XIE ; Yuanyuan ZHENG ; Yanlin ZHANG ; Dengyun MA ; Xiaoge ZHOU
Chinese Journal of Pathology 2019;48(5):369-372
Objective:
To investigate the pathological features and clinical manifestation of pediatric nodal marginal zone lymphoma(NMZL).
Methods:
Histological morphology and immunophenotype of 7 cases of pediatric NMZL were retrospectively reviewed at Beijing Friendship Hospital Affiliated to Medical University between January 2008 to October 2018. Clonal rearrangement analysis was performed. Clinical information including patient follow-up data were analyzed.
Results:
All 7 patients were male with a median age of 15 years aged from 10 to 26 years. All patients presented with only lymph node enlargement without B symptoms, including cervical lymph node (5 cases), preauricular lymph node (1 case) and retroauricular lymph node (1 case). Histologically, all cases showed irregular large follicles on the edges with widened marginal areas and intervesicular areas, and lesional cells were uniform with progressive transformation of germinal center centers along with a small amount of intrinsic lymphoid tissue. All 7 cases showed diffuse CD20 positivity both follicle and interfollicular region along with 30%-40% positivity in the interfollicular region (pathological region). Markers of other B-cell lymphomas werenot expressed. All 7 cases were positive for immunoglobulin(Ig) gene rearrangement. None of the patients showed no recurrence up on after follow-up for an average of 13 months.
Conclusions
Pediatric NMZL is a rare type of lymphoma that has a unique morphology and occurs almost exclusively in male children and young adults and often in head and neck lymph nodes. It has an excellent prognosis. Therefore, awareness of the disease with accurate diagnosis is important.
9.Paediatric nodal marginal zone lymphoma:a clinicopathological study of seven cases
Dongyu BAI ; Jianlan XIE ; Yuanyuan ZHENG ; Yanlin ZHANG ; Dengyun MA ; Xiaoge ZHOU
Chinese Journal of Pathology 2019;48(5):369-372
Objective To investigate the pathological features and clinical manifestation of pediatric nodal marginal zone lymphoma(NMZL).Methods Histological morphology and immunophenotype of 7 cases of pediatric NMZL were retrospectively reviewed at Beijing Friendship Hospital Affiliated to Medical University between January 2008 to October 2018. Clonal rearrangement analysis was performed. Clinical information including patient follow?up data were analyzed. Results All 7 patients were male with a median age of 15 years aged from 10 to 26 years. All patients presented with only lymph node enlargement without B symptoms, including cervical lymph node (5 cases), preauricular lymph node (1 case) and retroauricular lymph node (1 case). Histologically, all cases showed irregular large follicles on the edges with widened marginal areas and intervesicular areas, and lesional cells were uniform with progressive transformation of germinal center centers along with a small amount of intrinsic lymphoid tissue. All 7 cases showed diffuse CD20 positivity both follicle and interfollicular region along with 30%-40% positivity in the interfollicular region (pathological region). Markers of other B?cell lymphomas werenot expressed. All 7 cases were positive for immunoglobulin(Ig) gene rearrangement. None of the patients showed no recurrence up on after follow?up for an average of 13 months. Conclusions Pediatric NMZL is a rare type of lymphoma that has a unique morphology and occurs almost exclusively in male children and young adults and often in head and neck lymph nodes. It has an excellent prognosis. Therefore, awareness of the disease with accurate diagnosis is important.
10. EB virus-positive T/NK lymphoproliferative diseases: an analysis of 156 patients
Yanlin ZHANG ; Jianlan XIE ; Yuanyuan ZHENG ; Ping WEI ; Yuhua HUANG ; Xiaodan ZHENG ; Xiaojing TENG ; Wei LIU ; Xiaoge ZHOU
Chinese Journal of Pathology 2018;47(6):407-411
Objective:
To investigate the clinicopathological features of EBV-positive T/NK cell lymphoproliferative diseases (EBV+ T/NK-LPD).
Methods:
The clinical characteristics of 156 cases of EBV+ T/NK-LPD were collected from August 2002 to March 2015 at Beijing Friendship Hospital, Capital Medical University. Immunohistochemical staining, EBER in situ hybridization and clonal analysis of TCR gene were performed. All patients were followed up.
Results:
There were 106 male and 50 female patients; patients′ age ranged from 1 to 75 years (median 20 years). The course of the diseases before diagnosis ranged from 2 to 540 months (median 20 months). Fever was noted in 122 patients (78.2%), 108 patients had lymphadenopathy (69.2%), and 75 patients had hepatosplenomegaly (48.1%). Thirty-three cases were grade 1, 68 cases were grade 2, and 55 cases were grade 3. TCR gene arrangement analysis was performed in 45 cases, and 33 cases (73.3%) showed clonal rearrangement. The follow-up period ranged from 1-134 months, and 44 patients (28.2%) died. There was a trend of increased death rate associated with increasing grade (

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