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Chen XY, Yang JY, Chen YH, Liu AN, Wu SS, Ji Zhi SN, Zheng SM. Primary duodenal T/histiocyte-rich large B-cell lymphoma complicated with obstructive jaundice: A case report and review of literature. World J Gastrointest Surg 2025; 17:99758. [PMID: 39872781 PMCID: PMC11757195 DOI: 10.4240/wjgs.v17.i1.99758] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/30/2024] [Revised: 10/07/2024] [Accepted: 10/31/2024] [Indexed: 12/27/2024] Open
Abstract
BACKGROUND T/histiocyte-rich large B-cell lymphoma (T/HRBCL) is a highly aggressive subtype of diffuse large B-cell lymphoma characterized histologically by the presence of a few neoplastic large B cells amidst an abundant background of reactive T lymphocytes and/or histiocytes. T/HRBCL commonly affects the lymph nodes, followed by extranodal sites, such as the spleen, liver, and bone marrow, with rare occurrences in the gastrointestinal tract. Primary gastrointestinal T/HRBCL lacks specific clinical and endoscopic manifestations, and it is difficult to differentiate from inflammatory diseases, nodular lymphocyte predominant Hodgkin lymphoma, and other diseases on a histological basis, thereby hindering early diagnosis. CASE SUMMARY A 63-year-old man was hospitalized with a one-month history of jaundice and weight loss of approximately 3 kg. Laboratory tests revealed increased hepatic parameters in a cholestatic pattern and elevated carbohydrate antigen 19-9 levels. An abdominal computed tomography scan revealed a low-density mass within the descending duodenum and dilation of the bile and pancreatic ducts. He was clinically diagnosed with a duodenal tumor. During surgery, a 7.0 cm × 8.0 cm mass was identified within the descending duodenum, so pancreaticoduodenectomy and cholecystectomy were performed. Following operative biopsy, the tumor was diagnosed as primary duodenal T/HRBCL. The patient refused postoperative chemotherapy and died four months after surgery. CONCLUSION Primary duodenal T/HRBCL is an extremely rare and highly aggressive malignancy. The initial treatment strategies should be based on the original site of the tumor, the disease stage, and the patient's physical condition. Chemotherapy-based comprehensive treatment is still the main treatment method for primary gastrointestinal T/HRBCL.
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Affiliation(s)
- Xing-Yu Chen
- Department of Gastroenterology and Hepatology, North Sichuan Medical College, Nanchong 637000, Sichuan Province, China
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - Jia-Yi Yang
- Department of Radiology, Wuxi People’s Hospital, Nanjing Medical University, Nanjing 214023, Jiangsu Province, China
| | - Yi-Hua Chen
- Department of Pathology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - A-Niu Liu
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - Shan-Shan Wu
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - Se-Niu Ji Zhi
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
| | - Shu-Mei Zheng
- Department of Gastroenterology and Hepatology, The General Hospital of Western Theater Command, Chengdu 610083, Sichuan Province, China
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Okeke RK, Harmon GA, Okeke IG, Schuler JW, Sangappa SJ, Harmon JS, Angelova E, Sun X, Chinnici AA. Catch the Calcium: T-Cell Histiocyte-Rich B-Cell Lymphoma Presenting as Hypercalcemia. World J Oncol 2023; 14:570-574. [PMID: 38022410 PMCID: PMC10681787 DOI: 10.14740/wjon1610] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/27/2023] [Accepted: 09/14/2023] [Indexed: 12/01/2023] Open
Abstract
T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is an extremely rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL) that typically presents in middle-aged patients and carries a poor prognosis. Hypercalcemia presenting as the initial manifestation of the disease is rare, with only one other case reported in the literature. We report a case of a 90-year-old male who presented with progressive lethargy and unintentional weight loss. Initial workup showed elevated serum calcium of 14.6 mg/dL, corrected for albumin, and creatinine of 1.51 mg/dL. He had a suppressed iPTH of 6.3 pg/mL and normal PTHrP (13 pg/mL). Computed tomography (CT) scan of the abdomen and pelvis was performed to rule out underlying malignancy, which showed splenomegaly and enlarged retrocrural and porta hepatis lymph nodes. Bone marrow biopsy was performed to evaluate for hematological malignancy, which revealed findings diagnostic of THRLBCL. While rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) is one of the mainstay therapies for DLBCL and has been shown to have comparable outcomes in THRLBCL, there are documented concerns with its toxicity profile limiting the ability of older patients (60 years and older) to complete therapy. Our patient was treated with R-mini-CHOP, which is much better tolerated in this patient demographic. R-mini-CHOP features decreased doses of cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) with the conventional dose of rituximab. This case discusses a rare subtype of non-Hodgkin lymphoma presenting with a unique manifestation of hypercalcemia. We highlight the importance of thorough investigation for causes of hypercalcemia as well as the efficacy and tolerability of R-mini-CHOP in this elderly patient demographic.
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Affiliation(s)
- Richard K. Okeke
- Department of Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, USA
| | - Gabriella A. Harmon
- Department of Hematology/Oncology, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, USA
| | - Ijeoma G. Okeke
- Department of Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, USA
| | - Jake W. Schuler
- Saint George’s University School of Medicine, Grenada, West Indies
| | | | - Jonathan S. Harmon
- Department of Medicine, Einstein Medical Center Montgomery, East Norriton, PA, USA
| | - Evgeniya Angelova
- Department of Pathology, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, USA
| | - Xiu Sun
- Department of Pathology, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, USA
| | - Angelo A. Chinnici
- Department of Medicine, Jersey Shore University Medical Center, Hackensack Meridian Health, Neptune, NJ, USA
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The identification of TCF1+ progenitor exhausted T cells in THRLBCL may predict a better response to PD-1/PD-L1 blockade. Blood Adv 2022; 6:4634-4644. [PMID: 35767735 PMCID: PMC9636403 DOI: 10.1182/bloodadvances.2022007046] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/13/2022] [Accepted: 06/21/2022] [Indexed: 11/23/2022] Open
Abstract
T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is a rare and aggressive variant of diffuse large B-cell lymphoma (DLBCL) that usually affects young to middle-aged patients, with disseminated disease at presentation. The tumor microenvironment (TME) plays a key role in THRLBCL due to its peculiar cellular composition (<10% neoplastic B cells interspersed in a cytotoxic T-cell/histiocyte-rich background). A significant percentage of THRLBCL is refractory to rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (RCHOP)-based regimens and to chimeric antigen receptor T-cell therapy; thus, the development of a specific therapeutic approach for these patients represents an unmet clinical need. To better understand the interaction of immune cells in THRLBCL TME and identify more promising therapeutic strategies, we compared the immune gene expression profiles of 12 THRLBCL and 10 DLBCL samples, and further corroborated our findings in an extended in silico set. Gene coexpression network analysis identified the predominant role of the programmed cell death protein 1 (PD-1)/programmed cell death ligand 1 (PD-L1) axis in the modulation of the immune response. Furthermore, the PD-1/PD-L1 activation was flanked by the overexpression of 48 genes related to the functional exhaustion of T cells. Globally, THRLBCL TME was highly interferon-inflamed and severely exhausted. The immune gene profiling findings strongly suggest that THRLBCL may be responsive to anti–PD-1 therapy but also allowed us to take a step forward in understanding THRLBCL TME. Of therapeutic relevance, we validated our results by immunohistochemistry, identifying a subset of TCF1+ (T cell–specific transcription factor 1, encoded by the TCF7 gene) progenitor exhausted T cells enriched in patients with THRLBCL. This subset of TCF1+ exhausted T cells correlates with good clinical response to immune checkpoint therapy and may improve prediction of anti–PD-1 response in patients with THRLBCL.
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Schmitz C, Rekowski J, Reinke S, Müller SP, Hüttmann A, Klapper W, Dührsen U. Metabolic tumor volume, cancer cell fraction, and prognosis - the case of T-cell/histiocyte-rich large B-cell lymphoma. Leuk Lymphoma 2020; 61:1372-1379. [PMID: 32022621 DOI: 10.1080/10428194.2020.1713319] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/08/2023]
Abstract
T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is a rare aggressive lymphoma characterized by a paucity of neoplastic B-cells and a majority of reactive T-cells with or without histiocytes. In the 'Positron Emission Tomography-Guided Therapy of Aggressive Non-Hodgkin Lymphomas' trial (clinicaltrials.gov NCT00554164; EudraCT 2006-001641-33), its frequency was less than 3%. While cancer cell content by quantitative histology was 10-times lower, baseline total metabolic tumor volume (TMTV) by 18-fluorodesoxyglucose positron emission tomography was 10-times higher in THRLBCL than in diffuse large B-cell lymphoma (DLBCL). When THRLBCL and DLBCL populations were matched for TMTV, the survival curves were superimposable. However, when the populations were matched for cancer cell volume by multiplying TMTV by cancer cell fraction, outcome in THRLBCL was worse than in DLBCL. Whether genetic differences between cancer cells, tumor-promoting properties of non-neoplastic cells, or both are responsible for inferior cancer cell volume-related outcome in THRLBCL, remains to be elucidated.
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Affiliation(s)
- Christine Schmitz
- Department of Hematology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Jan Rekowski
- Institute for Medical Informatics, Biometry, and Epidemiology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Sarah Reinke
- Department of Pathology, Hematopathology Section and Lymph Node Registry, University Hospital Schleswig-Holstein, Kiel, Germany
| | - Stefan P Müller
- Department of Nuclear Medicine, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Andreas Hüttmann
- Department of Hematology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
| | - Wolfram Klapper
- Department of Pathology, Hematopathology Section and Lymph Node Registry, University Hospital Schleswig-Holstein, Kiel, Germany
| | - Ulrich Dührsen
- Department of Hematology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany
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Kommalapati A, Tella SH, Go RS, Nowakowski GS, Goyal G. T cell/histiocyte-rich large B cell lymphoma: incidence, demographic disparities, and long-term outcomes. Br J Haematol 2018; 185:140-142. [PMID: 29845599 DOI: 10.1111/bjh.15391] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/30/2022]
Affiliation(s)
- Anuhya Kommalapati
- Department of Internal Medicine, University of South Carolina School of Medicine, Columbia, SC, USA
| | - Sri Harsha Tella
- Department of Internal Medicine, University of South Carolina School of Medicine, Columbia, SC, USA
| | - Ronald S Go
- Division of Hematology, Mayo Clinic, Rochester, MN, USA
| | | | - Gaurav Goyal
- Division of Hematology, Mayo Clinic, Rochester, MN, USA
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Zheng SM, Zhou DJ, Chen YH, Jiang R, Wang YX, Zhang Y, Xue HL, Wang HQ, Mou D, Zeng WZ. Pancreatic T/histiocyte-rich large B-cell lymphoma: A case report and review of literature. World J Gastroenterol 2017; 23:4467-4472. [PMID: 28706431 PMCID: PMC5487512 DOI: 10.3748/wjg.v23.i24.4467] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/18/2017] [Revised: 04/26/2017] [Accepted: 06/01/2017] [Indexed: 02/06/2023] Open
Abstract
Primary pancreatic lymphoma (PPL) is an extremely rare form of extranodal malignant lymphoma. The most common histological subtype of PPL is diffuse large B cell lymphoma (DLBCL). In rare cases, PPL can also present as follicular lymphoma, small lymphocytic lymphoma, and T cell lymphoma either of non-Hodgkin’s lymphoma or of Hodgkin’s lymphoma. T-cell/histiocyte-rich large B-cell lymphoma (T/HRBCL) is an uncommon morphologic variant of DLBCL with aggressive clinical course, it is predominantly a nodal disease, but extranodal sites such as bone marrow, liver, and spleen can be involved. Pancreatic involvement of T/HRBCL was not presented before. Herein, we report a 48-year-old male who was hospitalized with complaints of jaundice, dark brown urine, pale stools, and nausea. The radiological evaluation revealed a pancreatic head mass and, following operative biopsy, the tumor was diagnosed as T/HRBCL. The patient achieved remission after six cycles of CHOP chemotherapy. Therefore, T/HRBCL can be treated similarly to the stage-matched DLBCL and both of them get equivalent outcomes after chemotherapy.
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MESH Headings
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Bile Ducts/diagnostic imaging
- Bile Ducts/surgery
- Biopsy
- Chemotherapy, Adjuvant/methods
- Cholangiopancreatography, Endoscopic Retrograde
- Choledochostomy
- Cyclophosphamide/therapeutic use
- Diagnosis, Differential
- Doxorubicin/therapeutic use
- Gastroenterostomy
- Histiocytes/pathology
- Hodgkin Disease/diagnosis
- Humans
- Jaundice/etiology
- Jaundice/surgery
- Jejunum/surgery
- Liver Function Tests
- Lymph Nodes/pathology
- Lymphatic Metastasis
- Lymphoma, Large B-Cell, Diffuse/complications
- Lymphoma, Large B-Cell, Diffuse/diagnosis
- Lymphoma, Large B-Cell, Diffuse/pathology
- Lymphoma, Large B-Cell, Diffuse/therapy
- Male
- Mesentery/pathology
- Middle Aged
- Nausea/etiology
- Nausea/surgery
- Pancreas/diagnostic imaging
- Pancreas/pathology
- Pancreatic Neoplasms/complications
- Pancreatic Neoplasms/diagnosis
- Pancreatic Neoplasms/pathology
- Pancreatic Neoplasms/therapy
- Pancreatitis/diagnosis
- Prednisone/therapeutic use
- Stomach/surgery
- Tomography, X-Ray Computed
- Vincristine/therapeutic use
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EBV-positive large B-cell lymphomas in young patients: a nodal lymphoma with evidence for a tolerogenic immune environment. Blood 2015; 126:863-72. [PMID: 25999451 DOI: 10.1182/blood-2015-02-630632] [Citation(s) in RCA: 205] [Impact Index Per Article: 20.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/23/2015] [Accepted: 05/15/2015] [Indexed: 12/22/2022] Open
Abstract
Few studies have reported Epstein-Barr virus-positive (EBV(+)) large B-cell lymphomas (LBCLs) in young patients without immunodeficiency. We identified 46 such cases in patients ≤45 years of age and analyzed the clinical and pathological characteristics. EBV(+) LBCLs affected predominantly males (male:female = 3.6:1), with a median age of 23 years (range, 4-45 years). All patients presented with lymphadenopathy and 11% also had extranodal disease. Morphologically, 3 patterns were identified: T-cell/histiocyte-rich large B-cell lymphoma-like (n = 36), gray zone lymphoma (n = 7), and diffuse LBCL-not otherwise specified (n = 3). Tumor cells (EBV(+) in >90% of cells) expressed B-cell antigens, were often CD30 and PD-L1 positive, and showed a nongerminal center immunophenotype. A total of 93% expressed EBV latency type II and 7% latency type III. Indoleamine 2,3-dioxygenase was expressed on background accessory cells. The most common treatment regimen was rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (58%), with local radiation therapy added in 21%. With a median follow-up of 22 months, 82% of patients are in clinical remission and only 8% died of disease. Younger patients achieved a significantly higher overall survival than prior series of EBV(+) LBCLs reported in the elderly (P < .0001). In conclusion, EBV(+) LBCLs are not restricted to the elderly. Young patients present with nodal disease and have a good prognosis.
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Blackmur JP, Stewart GD, Egong EA, Cutress ML, Tolley DA, Riddick AC, McNeill SA. Matched-Pair Analysis of Open versus Laparoscopic Nephroureterectomy for Upper Urinary Tract Urothelial Cell Carcinoma. Urol Int 2014; 94:156-62. [DOI: 10.1159/000364833] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/02/2014] [Accepted: 05/24/2014] [Indexed: 11/19/2022]
Abstract
Objective: Laparoscopic nephroureterectomy (LNU) offers a superior morbidity profile compared with open nephroureterectomy (ONU) in treating upper urinary tract urothelial cell carcinoma. Evidence of oncological equivalence between LNU and ONU is limited. We compare operative and oncological outcomes for LNU and ONU using matched-pair analysis. Methods: Of 159 patients who underwent a nephroureterectomy at a single institution between April 1992 and April 2010, 13 pairs of ONU and LNU patients were matched for gender, age, tumour location, tumour grade and stage. Operative details, post-operative characteristics and recurrences were collated and survival rates analysed using the Kaplan-Meier method. Results: There was no significant difference in mean operation time between LNU (191 min) and ONU (194 min, p = 0.92). There was no significant difference in the 5-year survival rate between LNU and ONU (overall survival 59.1% vs. 73.5%, p = 0.18; progression-free survival 24.0% vs. 56.0%, p = 0.14; cancer-specific survival 60.9% vs. 73.5%, p = 0.56; bladder cancer recurrence-free survival 8.7% vs. 0.0%, p = 0.09). Conclusion: Amidst limited RCT and comparative studies, this study presents further evidence of oncological equivalence between LNU and ONU. There was a trend towards poorer outcomes following LNU though, which merits further study.
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