2.Angioimmunoblastic T-cell lymphoma with fever, arthritis and skin pigmentation: A case report.
Gong CHENG ; Xia ZHANG ; Fei YANG ; Jia Yu CHENG ; Yan Ying LIU
Journal of Peking University(Health Sciences) 2020;52(6):1150-1152
Angioimmunoblastic T-cell lymphoma is a rare T-cell lymphoma. The clinical manifestations are not specific. In addition to the common clinical manifestations of lymphomas such as fever, weight loss, night sweats and lymphadenopathy, it may also have skin rashes, arthritis, multiple serous effusions, eosinophilia and other systemic inflammatory or immune symptoms. The lymphoma cells of angioimmunoblastic T-cell lymphoma originates from follicular helper T cells, and the follicular structure of lymph nodes disappears. In the tumor microenvironment, in addition to tumor cells, there are a large number of over-activated immune cells, such as abnormally activated B cells, which produce a series of systemic inflammation or immune-related symptoms. This disease is rare and difficult to diagnose. This article reports a 36-year-old female. She got fever, joint swelling and pain, skin pigmentation, accompanied by hepatomegaly, splenomegaly, lymphadenopathy, anemia and other multiple-systems manifestations. The clinical manifestations of this patient were similar to autoimmune diseases such as adult onset Still's disease, rheumatoid arthritis, and systemic sclerosis, which made the diagnosis difficult. At the beginning of the disease course, the patient got arthritis and fever. And her white blood cells were significantly increased. Adult onset Still's disease should be considered, but her multiple-systems manifestations could not be explained by adult onset Still's disease. And her arthritis of hands should be distinguished with rheumatoid arthritis. However, the patient's joint swelling could get better within 3-7 days, and there was no synovitis and bone erosion on joint imaging examination. The rheumatoid factor and anti-CCP antibody were negative. The diagnostic evidence for rheumatoid arthritis was insufficient. The patient's skin pigmentation and punctate depigmentation were similar to those of systemic sclerosis. But the patient had no Raynaud's phenomenon, and her sclerosis-related antibody was negative. The diagnostic evidence for systemic sclerosis was also insufficient. After 3 years, she was finally diagnosed with angioimmunoblastic T-cell lymphoma by lymph node biopsy aspiration. This case suggests that the clinical manifestations of angioimmunoblastic T-cell lymphoma are diverse, and some symptoms similar to immune diseases may appear. When the patient's clinical symptoms are atypical and immune diseases cannot explain the patient's condition, and further evidence should be sought to confirm the diagnosis.
Adult
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Diagnosis, Differential
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Female
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Humans
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Immunoblastic Lymphadenopathy/diagnosis*
;
Lymphoma, T-Cell/diagnosis*
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Skin Pigmentation
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Tomography, X-Ray Computed
;
Tumor Microenvironment
3.Angioimmunoblastic T-cell lymphoma with autoimmune hemolytic anemia and pure red cell aplasia.
Fu-Xu WANG ; Xue-Jun ZHANG ; Ling PAN ; Shu-Kai QIAO ; Xiao-Ling GUO ; Zuo-Ren DONG
Journal of Experimental Hematology 2007;15(4):862-865
Angioimmunoblastic T-cell lymphoma (AILT) is a peripheral T-cell lymphoma often complicated autoimmune phenomena such as autoimmune cytopenia, and is a truly rare type of NHL. In order to investigate the clinical features, pathological manifestation of this lymphoma, and to explore its therapy protocol, a 37-years old patient with AILT was investigated. The routine blood examination, bone marrow smear, lymphonodus biopsy, Coombs test, flow cytometry for bone marrow mononuclear cells, serological test, immunochemistry method etc were performed for this patient. The results showed that the systemic lymphadenectasis and hepatosplenomegaly were seen in patient, the cervical lymphonode biopsy revealed AITL. The hematoglobin level and number of reticulocytes were very low. Coombs test was positive. Simultaneously, the bone marrow aspirate revealed erythroid aplasia. The warm type autoimmune hemolytic anemia (AIHA) and pure red cell aplasia (PRCA) were co-existed. After one course of chemotherapy with CHOP-E, infiltration sign of AITL patient with AIHA and PRCA disappeared. In conclusion, the AITL patient complicated with AIHA and PRCA was successfully diagnosed, the lymphonode biopsy and bone marrow smear showed more significant, the chemotherapy protocol of CHOP-E can give some effect to cure such angioimmunoblastic T cell lymphoma.
Adult
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Anemia, Hemolytic, Autoimmune
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complications
;
diagnosis
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Humans
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Immunoblastic Lymphadenopathy
;
complications
;
diagnosis
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Lymphoma, T-Cell
;
complications
;
diagnosis
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Male
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Red-Cell Aplasia, Pure
;
complications
;
diagnosis
4.Clinicopathological analysis of 64 case of angioimmunoblastic T-cell lymphoma.
Congyou GU ; Nan LI ; Min LI ; Xuemin XUE ; Zifen GAO
Chinese Journal of Hematology 2014;35(1):24-28
OBJECTIVETo explore the clinical and pathological characteristics of angioimmunoblastic T-cell lymphoma (AITL).
METHODSSixty-four cases of AITL were retrospectively analyzed by histopathological and immunohistochemical methods.
RESULTSThere 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.
CONCLUSIONThe 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.
Adult ; Aged ; Aged, 80 and over ; Diagnosis, Differential ; Female ; Humans ; Immunoblastic Lymphadenopathy ; diagnosis ; pathology ; Lymphoma, Follicular ; pathology ; Lymphoma, T-Cell, Peripheral ; diagnosis ; pathology ; Male ; Middle Aged ; Retrospective Studies
5.Clinicopathologic features and diagnosis of 18 patients with angioimmunoblastic T-cell lymphoma.
Xiaoyu YUAN ; Fangping CHEN ; Dandong BI ; Xielan ZHAO ; Qiongqiong HE ; Qun LI
Journal of Central South University(Medical Sciences) 2009;34(6):523-526
OBJECTIVE:
To explore the clinical and pathologic features of angioimmunoblastic T-cell lymphoma(AITL) and provide evidence for diagnosis.
METHODS:
Eighteen AITL patients (9 males and 9 females aged from 14 to 70 years) were retrospectively analyzed in Xiangya Hospital of Central South University from July 2002 to September 2007.
RESULTS:
Characteristic features at the presentation of AITL included generalized lymphadenopathy, fever, splenomegaly, and skin rashes with polyclonal hyper-gammaglobulinemia and other hematological abnormalities (such as Coombs-positive hemolytic anemia), which often involved the bone marrow and had well-described histologic features. The positive rate for CXCL13 was 93.3%.
CONCLUSION
Repeated lymphadenbiopsy is helpful for AITL diagnosis. Routine histological and immunohistochemical examinations (especially including CXCL13) play significant role in the diagnosis and differential diagnosis of AITL.
Adolescent
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Adult
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Aged
;
Chemokine CXCL13
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metabolism
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Female
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Humans
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Immunoblastic Lymphadenopathy
;
diagnosis
;
metabolism
;
pathology
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Lymphoma, T-Cell, Peripheral
;
diagnosis
;
metabolism
;
pathology
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Male
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Middle Aged
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Retrospective Studies
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Young Adult
6.Clinical analysis of 12 patients with angioimmunoblastic T cell lymphoma.
Shu-Hong WANG ; Quan-Shun WANG ; Lu SUN ; Hong-Hua LI ; Yu ZHAO ; Bo-Jun JIA ; Xiao-Ling ZHANG ; Li YU
Journal of Experimental Hematology 2010;18(5):1208-1210
To evaluate the clinical, pathological characters and prognosis of patients with angioimmunoblastic T cell lymphoma (AITL), the clinicopathologic features, immunophenotypes, therapy and survival rate of 12 AITL patients which were confirmed by pathologic examination were retrospectively studied. The results indicated that main symptom was observed as general lymphadenopathy, however, 9 patients had fever. The diagnosis of AITL was based on lymph-node biopsy. The histopathologic characteristics of AITL showed the damage of normal lymphnode structure, the proliferation of immunoblastic cells and arborescent super vascularization. All immunophenotypes were mature peripheral T-cellular. CVP regimen was the most common chemotherapy regimen used for patients. 58% patients have a good initial response to chemotherapy. 3-year survival was 25%, with median survival time of 25 months. In conclusion, most cases of AITL display an aggressive course, therefore, the disease progresses rapidly and has unfavorable prognosis, further studies are required to improve its therapy regimen.
Aged
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Female
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Humans
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Immunoblastic Lymphadenopathy
;
diagnosis
;
pathology
;
therapy
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Lymph Nodes
;
pathology
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Lymphoma, T-Cell, Peripheral
;
diagnosis
;
pathology
;
therapy
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Male
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Middle Aged
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Survival Rate
7.Clinicopathologic analysis of angioimmunoblastic T-cell lymphoma with Hodgkin/Reed-Sternberg-like cells.
Xue GAO ; Wenyong HUANG ; Wensheng LI ; Jianlan XIE ; Yuanyuan ZHENG ; Xiaoge ZHOU
Chinese Journal of Pathology 2015;44(8):553-558
OBJECTIVETo study the clinicopathologic features and pathologic diagnosis and differential diagnosis of angioimmunoblastic T-cell lymphoma with HRS-like cells.
METHODSSix cases of angioimmunoblastic T-cell lymphoma with HRS-like cells were examined histologically and immunohistochemically (EliVision method) and in-situ hybridization for Epstein-Barr virus-encoded RNA (EBER), and the literature was reviewed.
RESULTSThe cytologic and microscopic features of these imprints and lymph node samples showed a heterogeneous population of hematolymphoid cells, including small to intermediate lymphoid cells, immunoblasts, plasma cells, dendritic cells, and eosinophils, as well as small vessels that were surrounded by some of the abnormal cells. The neoplastic T-cells expressed CD3 and CD5 and partly positive for CD10 and bcl-6, CD21 showed expanded and irregular follicular dendritic cell (FDC) meshworks that surrounding the high HEV. The HRS-like cells were positive for MUM-1 and Ki-67, variable intensity positive for CD30, CD20, and PAX-5, but negative for CD15. EBV-positive cells included HRS-like cells and small to large-sized neoplastic T-cells, which formed small clusters or scattering in the background of the disease.
CONCLUSIONSThe clinical course of angioimmunoblastic T-cell lymphoma with HRS-like cells is aggressive. Which present with histomorphology overlap with classical Hodgkin lymphoma (CHL), similar to CHL in EBER and immunophenotype, however, it is easy to misdiagnosis as HL. Thus, angioimmunoblastic T-cell lymphoma pathology diagnosis should comprehensive analysis of different kinds of materials, including clinical features, and histological structure, and EBER, and immunophenotype, and gene rearrangement.
Diagnosis, Differential ; Herpesvirus 4, Human ; Hodgkin Disease ; pathology ; Humans ; Immunoblastic Lymphadenopathy ; diagnosis ; pathology ; Immunohistochemistry ; Immunophenotyping ; In Situ Hybridization ; Lymphoma, T-Cell ; diagnosis ; pathology ; RNA, Viral ; Reed-Sternberg Cells ; pathology ; T-Lymphocytes ; pathology
8.Expressions of CXCL13, CD10 and bcl-6 in angioimmunoblastic T-cell lymphoma and peripheral T-cell lymphoma, not otherwise specified.
Xue-feng TANG ; Gan-di LI ; Ya-lin LI ; Dong-ni LIANG ; Tian XIA ; Ji-yong ZHOU ; Yu-qi YAO ; Wen-qiao WU ; Zhan-gui WANG ; Yong-hong YANG ; Xian-bin TANG ; Yan-qiong BAI ; Qiang DING
Chinese Journal of Pathology 2009;38(4):224-230
OBJECTIVETo study the value of immunomarkers CXCL13, CD10, bcl-6 in pathologic diagnosis of angioimmunoblastic T-cell lymphoma (AITL).
METHODSOne hundred and fifteen cases of AITL, 30 cases of peripheral T-cell lymphoma, not otherwise specified (PTCL, NOS) and 30 cases of reactive lymph nodes with paracortical hyperplasia (RH) encountered during the period from January, 1990 to January, 2008 were retrieved from the archival files of the Department of Pathology, West China Hospital of Sichuan University, China. The morphologic features were reviewed and compared. Immunohistochemical study was performed by SP method for CXCL13, CD10, bcl-6, CD21, CD3epsilon, CD3, CD45RO, CD20 and Ki-67. TCR-gamma gene rearrangement study was also carried out.
RESULTSRegressed follicles were evident in 7.8% (9/115) of AITL cases, 6.7% (2/30) of PTCL, NOS cases and 83.3% (25/30) of RH cases, respectively. A marked increase of number of arborizing venules was shown in 98.3% (113/115) of AITL cases, 63.3% (19/30) of PTCL, NOS cases and 76.7% (23/30) of RH cases, respectively. In lymph nodes with paracortical hyperplasia, the expression of CXCL13, CD10 and bcl-6 were restricted to the germinal centers. In AITL, 96.5% (111/115) of cases showed CXCL13 expression, in contrast to 26.7% (8/30) of PTCL, NOS. Expression of CD10 and bcl-6 were found in the neoplastic cells in 50.4% (58/115) and 78.3% (90/115) of AITL, and 3.3% (1/30) and 3.3% (1/30) of PTCL, NOS, respectively. Irregular meshworks of CD21-positive follicular dendritic cells were found in all the AITL cases. Clonal TCR-gamma rearrangement was detected in 83% (83/100) of the AITL cases.
CONCLUSIONSAITL is a type of lymphoma originated from the follicular helper T cells. Detailed morphologic assessment and use of immunohistochemical markers are essential for accurate diagnosis.
Adult ; Aged ; Aged, 80 and over ; Chemokine CXCL13 ; metabolism ; Diagnosis, Differential ; Female ; Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor ; Humans ; Immunoblastic Lymphadenopathy ; metabolism ; pathology ; Lymph Nodes ; metabolism ; pathology ; Lymphoma, T-Cell, Peripheral ; metabolism ; pathology ; Male ; Middle Aged ; Neprilysin ; metabolism ; Proto-Oncogene Proteins c-bcl-6 ; metabolism ; Pseudolymphoma ; metabolism ; pathology
9.Drug-induced lymphadenitis.
Wei-hua YIN ; Hong-yu ZHANG ; Xue-feng LI ; Ya MA
Chinese Journal of Pathology 2010;39(3):192-194
Adult
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Anti-Inflammatory Agents, Non-Steroidal
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adverse effects
;
therapeutic use
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CD3 Complex
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metabolism
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Colitis, Ulcerative
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drug therapy
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Diagnosis, Differential
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Drug Hypersensitivity
;
etiology
;
metabolism
;
pathology
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Female
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Gastrointestinal Agents
;
adverse effects
;
therapeutic use
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Humans
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Immunoblastic Lymphadenopathy
;
metabolism
;
pathology
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Ki-1 Antigen
;
metabolism
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Lymphadenitis
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chemically induced
;
metabolism
;
pathology
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Lymphoma, Large-Cell, Anaplastic
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metabolism
;
pathology
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Lymphoma, T-Cell
;
metabolism
;
pathology
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Receptors, Complement 3d
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metabolism
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Sulfasalazine
;
adverse effects
;
therapeutic use