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1.
J Assoc Physicians India ; 72(1): 99-102, 2024 Jan.
Article in English | MEDLINE | ID: mdl-38736082

ABSTRACT

BACKGROUND: Peripheral T cell lymphoma (PTCL), not otherwise specified (NOS) is a heterogenous group of predominantly nodal T cell lymphomas that generally presents with lymphadenopathy with or without extra nodal involvement. Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and acral cyanosis. Although, this condition is commonly associated with connective tissue disorder, smoking and vasculitis, its association with lymphoid malignancy is very rare. Here, we present a case report of a patient with digital gangrene of all toes and fingers as a presenting symptom of PTCL-NOS. CASE DESCRIPTION: A 62 year old male presented with digital ischemia associated with pain, low grade fever, loss of appetite and significant weight loss of 6 kilograms over a period of 3 months. On examination, he was found to have bilateral inguinal and axillary lymph nodes with gangrenous changes over toes and fingers but peripheral pulses were palpable. On evaluation he had anemia, elevated ESR and CRP. CT angiogram revealed thinned out digital arteries with multifocal areas of narrowing. Patient was screened for other causes of digital gangrene and was tested negative for ANCA, ANA, cryoglobulins and viral markers. Lymph node biopsy with IHC was suggestive of peripheral T-cell lymphoma-NOS and was started on CHOP regimen. Lymph nodes size decreased and gangrenous changes resolved. CONCLUSION: Though digital ischemia is a rare paraneoplastic presentation of lymphoma, it should be considered if there is a rapid progression of gangrene. Early initiation of chemotherapy may result in the reduction of further progression of digital gangrene and thus prevent permanent disability. In our patient, progression of gangrene was prevented even though it was an aggressive variant of T cell lymphoma.


Subject(s)
Fingers , Gangrene , Lymphoma, T-Cell, Peripheral , Paraneoplastic Syndromes , Toes , Humans , Male , Gangrene/etiology , Gangrene/diagnosis , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/complications , Middle Aged , Fingers/pathology , Paraneoplastic Syndromes/diagnosis , Paraneoplastic Syndromes/etiology , Toes/pathology , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Cyclophosphamide/therapeutic use , Doxorubicin/therapeutic use , Vincristine/therapeutic use , Prednisone/therapeutic use
2.
Br J Haematol ; 204(5): 1862-1871, 2024 May.
Article in English | MEDLINE | ID: mdl-38613165

ABSTRACT

Peripheral T-cell lymphomas (PTCL) are morphologically and biologically heterogeneous and a subset expresses CD30, including anaplastic large cell lymphomas (ALCL) and a minority of PTCL, not otherwise specified (PTCL, NOS). ALCL with ALK translocations (ALCL, ALK+) are readily identified by routine diagnostic methods, but differentiating ALCL without ALK translocation (ALCL, ALK-) and PTCL, NOS expressing CD30 (PTCL CD30+) can be challenging. Furthermore, rare PTCL co-express CD30 and CD15 (PTCL CD30+CD15+); some resemble ALCL, ALK- while others resemble classic Hodgkin lymphoma. To explore the relationship between PTCL CD30+CD15+ and ALCL, ALK-, we analysed 19 cases of PTCL with CD30 expression, previously diagnosed as ALCL, ALK- (nine cases) and PTCL CD30+CD15+ (10 cases) for DUSP22/IRF4 rearrangements, coding RNA expression and selected transcriptome analysis using the NanoString nCounter gene expression analysis platform. Unsupervised clustering showed no clear segregation between ALCL, ALK- and PTCL CD30+CD15+. Three cases previously classified as PTCL CD30+CD15+ showed DUSP22/IRF4 rearrangements, favouring a diagnosis of ALCL, ALK-. Our results suggest that cases previously designated PTCL CD30+CD15+, likely fall within the spectrum of ALCL, ALK-; additionally, a subset of ALCL, ALK- with DUSP22/IRF4 rearrangement expresses CD15, consistent with previous reports and expands the immunophenotypic spectrum of this lymphoma subgroup.


Subject(s)
Anaplastic Lymphoma Kinase , Ki-1 Antigen , Lewis X Antigen , Lymphoma, Large-Cell, Anaplastic , Lymphoma, T-Cell, Peripheral , Humans , Lymphoma, Large-Cell, Anaplastic/genetics , Lymphoma, Large-Cell, Anaplastic/pathology , Lymphoma, Large-Cell, Anaplastic/diagnosis , Ki-1 Antigen/metabolism , Ki-1 Antigen/genetics , Ki-1 Antigen/analysis , Lymphoma, T-Cell, Peripheral/genetics , Lymphoma, T-Cell, Peripheral/metabolism , Lymphoma, T-Cell, Peripheral/pathology , Lymphoma, T-Cell, Peripheral/diagnosis , Male , Female , Middle Aged , Adult , Anaplastic Lymphoma Kinase/genetics , Anaplastic Lymphoma Kinase/metabolism , Lewis X Antigen/analysis , Lewis X Antigen/metabolism , Aged , Dual-Specificity Phosphatases/genetics , Interferon Regulatory Factors/genetics , Interferon Regulatory Factors/metabolism , Mitogen-Activated Protein Kinase Phosphatases/genetics , Young Adult , Gene Rearrangement
3.
J Clin Exp Hematop ; 64(1): 52-58, 2024.
Article in English | MEDLINE | ID: mdl-38538319

ABSTRACT

We report the case of a 52-year-old male who presented to our hospital with cervical lymphadenopathy. Lymph node biopsy revealed small atypical lymphoid cells positive for CD3 and CD5 and negative for CD56 and Epstein-Barr virus (EBV)-encoded small RNA (EBER) by in situ hybridization. CD4-positive cells and CD8-positive cells were mixed in almost equal numbers. He was diagnosed with peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). The patient received one cycle of chemotherapy, resulting in severe sepsis. While undergoing treatment in the intensive care unit with an antimicrobial agent and prednisone, ascitic fluid appeared. Abdominal aspiration revealed neutrophil-predominant ascites and microbiological studies revealed Candida albicans. However, ascites did not improve when treated with micafungin for Candida peritonitis. Abdominal aspiration was re-performed, and atypical lymphoid cells that were positive for CD3 and CD56 were detected. EBV-DNA levels in whole blood were significantly elevated. Atypical lymphoid cells were positive for EBER by in situ hybridization and Southern blot analysis showed EBV terminal repeat monoclonal patterns. Bone marrow examination revealed the same atypical lymphoid cells. Therefore, the patient was diagnosed with extranodal natural killer/T-cell lymphoma (ENKTL) with bone marrow involvement 3 months after the diagnosis of PTCL-NOS. Complications associated with PTCL-NOS and ENKTL are rare. PTCL-NOS, chemotherapy, sepsis, and prednisone might have led to immunodeficiency and reactivation of EBV, which might be one of the pathophysiologies for developing ENKTL. Our case indicates that measuring EBV-DNA in the blood is a simple and prompt examination to detect complications of EBV-associated lymphoma.


Subject(s)
Epstein-Barr Virus Infections , Lymphoma, Extranodal NK-T-Cell , Lymphoma, T-Cell, Peripheral , Male , Humans , Middle Aged , Epstein-Barr Virus Infections/complications , Herpesvirus 4, Human , Lymphoma, T-Cell, Peripheral/complications , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/drug therapy , Prednisone , Lymphoma, Extranodal NK-T-Cell/complications , Lymphoma, Extranodal NK-T-Cell/diagnosis , Ascites/complications , Ascites/pathology , Killer Cells, Natural/pathology , DNA
4.
Exp Oncol ; 45(4): 474-482, 2024 Feb 03.
Article in English | MEDLINE | ID: mdl-38328842

ABSTRACT

BACKGROUND: The peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is the most common subtype of peripheral T-cell lymphoma (PTCL). It constitutes approximately 25% of all PTCLs and accounts for more than 15% of all lymphomas. The results of the first Ukrainian prospective study of patients with PTCL-NOS are presented in the article. The aim of the study was to analyze the morbidity of PTCL patients and the treatment performed, to evaluate overall survival and progression-free survival, and to determine the factors that predict the treatment response. PATIENTS AND METHODS: An analysis was performed on the data of 31 patients diagnosed with peripheral PTCL-NOS from February 2018 to the present. T-cell lymphoid neoplasms were diagnosed according to the 2016 WHO classification. The treatment regimens were in alignment with ESMO and NCCN guidelines. More than 90% of patients were prescribed anthracycline-based regimens (CHOP; CHOEP - cyclophosphamide, doxorubicin, etoposide, vincristine, prednisone). An initial treatment was performed with CHOP-based regimens in 38.70% (n = 12) of patients, with the addition of etoposide in 58.06% of patients (n = 18). RESULTS: The response was assessed according to the response criteria for malignant lymphoma (Cheson, 2008, 2014). The overall response to therapy was 58.06% (n = 18), with complete responses in 29.03% of patients and partial responses in 29.03% of patients. The stabilization of the disease occurred in 3.44%, while the disease progression in 41.37% of patients. The 12-month and 24-month survival rates were 75.44% and 50.81%, respectively. The 12-month and 24-month progression-free survivals were 47.68% and 33.1%, respectively. Ki-67 overexpression (> 65%) was a negative prognostic factor. CONCLUSIONS: The results of the treatment of PTCL obtained in a Ukrainian population study are similar to those in other European studies, all of which remain unsatisfactory. Further research is required to develop a new strategy for examination and therapy to improve treatment outcomes. The emphasis should be placed on the pragmatic clinical trials comparing the efficacy of first-line treatment in PTCL patients with both favorable and unfavorable clinical factors.


Subject(s)
Lymphoma, T-Cell, Peripheral , Humans , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/drug therapy , Lymphoma, T-Cell, Peripheral/pathology , Prospective Studies , Etoposide/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/adverse effects , Treatment Outcome , Cyclophosphamide/therapeutic use , Cyclophosphamide/adverse effects , Doxorubicin/therapeutic use , Disease Progression
5.
Br J Haematol ; 204(5): 1752-1756, 2024 May.
Article in English | MEDLINE | ID: mdl-38176400

ABSTRACT

Peripheral T-cell lymphomas (PTCLs) have a poor prognosis and, to date, there are no reliable predictive biomarkers of response. In this work we explored the prognostic impact of cell-free DNA (cfDNA) concentration in 75 newly diagnosed patients enrolled in a prospective multicenter study. Pre-treatment cfDNA was strongly associated with clinical risk factors and was identified as a superior predictor for shorter progression-free survival in multivariable analysis, outweighing canonical risk parameters. Furthermore, we identified a cfDNA value above which survival worsens. In conclusion, pre-treatment cfDNA concentration represents an easily usable predictive biomarker that is highly associated with survival of PTCL patients.


Subject(s)
Cell-Free Nucleic Acids , Lymphoma, T-Cell, Peripheral , Humans , Lymphoma, T-Cell, Peripheral/mortality , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/blood , Lymphoma, T-Cell, Peripheral/genetics , Male , Female , Middle Aged , Aged , Cell-Free Nucleic Acids/blood , Prognosis , Adult , Biomarkers, Tumor/blood , Prospective Studies , Aged, 80 and over , Antineoplastic Combined Chemotherapy Protocols/therapeutic use
6.
Medicine (Baltimore) ; 103(3): e36974, 2024 Jan 19.
Article in English | MEDLINE | ID: mdl-38241538

ABSTRACT

INTRODUCTION: Fever of unknown origin (FUO) remains one of the most challenging clinical conditions. It demands an exhaustive diagnostic approach, considering its varied etiologies spanning infectious, autoimmune, inflammatory, and malignant causes. PATIENT CONCERNS: This report shows the journey of diagnosing a 28-year-old male who presented with persistent fever and lower-extremity weakness over 9 months. Despite seeking care at multiple hospitals, a definitive diagnosis remained elusive. DIAGNOSIS: The patient underwent a series of evaluations in various specialties, including gastroenterology, infectious diseases, rheumatology, hematology, and cardiology. Multiple tests and treatments were administered, including antiviral therapy for hepatitis B and antibiotics for suspected infections. INTERVENTIONS: After an initial misdiagnosis and unsuccessful treatments, a positron emission tomography-computed tomography scan and lymph node biopsy ultimately led to the diagnosis of peripheral T-cell lymphoma-T follicular helper type (PTCL-TFH) lymphoma. The patient was referred to the hematology clinic and initiated on CHOEP (cyclophosphamide, vincristine, etoposide, and prednisone) chemotherapy. OUTCOMES: The patient showed a positive response to CHOEP therapy, as indicated by a posttreatment positron emission tomography-computed tomography scan. He reported a significant improvement in his quality of life. Additional rounds of the same regimen were planned to further manage the lymphoma. CONCLUSION: This case emphasizes the importance of a comprehensive and persistent diagnostic approach in managing FUO. Initially, the focus on infectious causes led to extensive treatments, but the disease's progression and complications shifted attention to other specialties. The eventual diagnosis of PTCL-TFH lymphoma highlights the significance of advanced imaging techniques and multidisciplinary collaboration in uncovering elusive diagnoses. Thorough surveillance, timely reassessments, and repeated testing can uncover definitive changes critical for diagnosis. PTCL-TFH lymphoma, although rare, should be considered in the differential diagnosis of FUO, especially when initial evaluations are inconclusive.


Subject(s)
Fever of Unknown Origin , Lymphoma, T-Cell, Peripheral , Male , Humans , Adult , Lymphoma, T-Cell, Peripheral/complications , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/pathology , Fever of Unknown Origin/diagnosis , Fever of Unknown Origin/etiology , Quality of Life , Positron Emission Tomography Computed Tomography , T-Lymphocytes, Helper-Inducer
7.
BMC Cancer ; 24(1): 18, 2024 Jan 02.
Article in English | MEDLINE | ID: mdl-38166662

ABSTRACT

BACKGROUND: Peripheral T-cell lymphoma (PTCL) refers to a heterogenous group of T-cell neoplasms with poor treatment responses and survival times. Canine PTCL clinically and immunophenotypically resembles the most common human subtype, PTCL-not otherwise specified (PTCL-NOS), leading to interest in this canine disease as a naturally occurring model for human PTCL. Gene expression profiling in human PTCL-NOS has helped characterize this ambiguous diagnosis into distinct subtypes, but similar gene expression profiling in canine PTCL is lacking. METHODS: Bulk RNA-sequencing was performed on tumor samples from 33 dogs with either CD4+ (26/33), CD8+ (4/33), or CD4-CD8- (3/33) PTCL as diagnosed by flow cytometry, and sorted CD4+ and CD8+ lymphocytes from healthy control dogs. Following normalization of RNA-seq data, we performed differential gene expression and unsupervised clustering methods. Gene set enrichment analysis was performed to determine the enrichment of canine CD4+ PTCL for human PTCL-NOS, oncogenic pathways, and various stages of T-cell development gene signatures. We utilized gene set variation analysis to evaluate individual canine CD4+ PTCLs for various human and murine T-cell and thymocyte gene signatures. Cultured canine PTCL cells were treated with a pan-PI3K inhibitor, and cell survival and proliferation were compared to DMSO-treated controls. Expression of GATA3 and phosphorylated AKT was validated by immunohistochemistry. RESULTS: While the canine CD4+ PTCL phenotype exhibited a consistent gene expression profile, the expression profiles of CD8+ and CD4-CD8- canine PTCLs were more heterogeneous. Canine CD4+ PTCL had increased expression of GATA3, upregulation of its target genes, enrichment for PI3K/AKT/mTOR signaling, and downregulation of PTEN, features consistent with the more aggressive GATA3-PTCL subtype of human PTCL-NOS. In vitro assays validated the reliance of canine CD4+ PTCL cells on PI3K/AKT/mTOR signaling for survival and proliferation. Canine CD4+ PTCL was enriched for thymic precursor gene signatures, exhibited increased expression of markers of immaturity (CD34, KIT, DNTT, and CCR9), and downregulated genes associated with the T-cell receptor, MHC class II associated genes (DLA-DQA1, DLA-DRA, HLA-DQB1, and HLA-DQB2), and CD25. CONCLUSIONS: Canine CD4+ PTCL most closely resembled the GATA3-PTCL subtype of PTCL-NOS and may originate from an earlier stage of T-cell development than the more conventionally posited mature T-helper cell origin.


Subject(s)
Lymphoma, T-Cell, Peripheral , Humans , Dogs , Animals , Mice , Lymphoma, T-Cell, Peripheral/genetics , Lymphoma, T-Cell, Peripheral/diagnosis , Transcriptome , Phosphatidylinositol 3-Kinases/metabolism , Proto-Oncogene Proteins c-akt/metabolism , TOR Serine-Threonine Kinases/metabolism
9.
Clin Lymphoma Myeloma Leuk ; 24(2): 65-76, 2024 02.
Article in English | MEDLINE | ID: mdl-37973458

ABSTRACT

Although a rare subset of non-Hodgkin lymphomas, peripheral T-cell lymphomas (PTCL) account for a disproportionate proportion of patient mortality. Conventional therapies are derived from experience treating aggressive B-cell lymphomas and center around CHOP-based chemotherapy. However, due to the unique biology and diverse subtypes of PTCL, most patients fail to durably respond to this approach and 5-year survival is only 20% to 30%. There have been multiple attempts to improve outcomes for patients with PTCL. Among the more successful strategies are the use of consolidative autologous stem cell transplant, the augmentation of CHOP with etoposide (CHOEP), and the use of brentuximab vedotin in CD30-positive PTCL. Advances in the understanding of histology-specific biology has cultivated enthusiasm to evaluate hypomethylating agents, histone deacetylate inhibitors, and phosphoinositol-3-kinase inhibitors in the frontline setting. Improvements in monitoring disease response and prognostication including the use of cell-free DNA, mutational profiling, and interim PET/CT imaging are also on the horizon. For patients with acute T-cell leukemia/lymphoma, the use of mogamulizumab-based therapy in the frontline setting may lead to advances in care. The true impact of these new-era therapies will only be elucidated as clinical practices incorporate the rapidly changing evidence.


Subject(s)
Lymphoma, T-Cell, Peripheral , Humans , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/drug therapy , Positron Emission Tomography Computed Tomography , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Brentuximab Vedotin/therapeutic use , Stem Cell Transplantation
11.
Haematologica ; 109(4): 1163-1170, 2024 Apr 01.
Article in English | MEDLINE | ID: mdl-37794805

ABSTRACT

Peripheral T-cell lymphomas (PTCL) comprise a heterogeneous group of mature T-cell neoplasms with an unfavorable prognosis; presentation with stage I(E) disease is uncommon. In clinical practice, an abbreviated chemotherapy treatment regimen combined with radiotherapy (combined modality treatment [CMT]) is commonly used, although evidence from clinical trials is lacking. The aim of this nationwide population-based cohort study is to describe first-line treatment and outcome of patients with stage I(E) PTCL. All newly diagnosed patients ≥18 years with stage I(E) anaplastic large cell lymphoma (ALCL), angioimmunoblastic T-cell lymphoma (AITL) and peripheral T-cell lymphoma NOS (PTCL not otherise specified [NOS]) in 1989-2020 were identified in the Netherlands Cancer Registry. Patients were categorized according to treatment regimen, i.e., chemotherapy (CT), radiotherapy (RT), CMT, other therapy and no treatment. The primary endpoint was overall survival (OS). Patients with stage I(E) ALCL, AITL and PTCL NOS (n=576) were most commonly treated with CMT (28%) or CT (29%), 2% underwent SCT. RT only was given in 18%, and 8% received other therapy and 16% no treatment. Overall, the 5-year OS was 59%. According to subtype, 5-year OS was superior for ALCL as compared to PTCL NOS and AITL (68% vs. 55% and 52%, respectively; P=0.03). For patients treated with CMT, 5-year OS was significantly higher (72%) as compared to patients treated with either CT or RT alone (55% and 55%, respectively; P<0.01). In multivariable analysis, age per year increment (hazard ratio [HR] =1.06, 95% confidence interval [CI]: 1.05-1.07), male sex (HR=1.53, 95% CI: 1.23-1.90), and CT, or no treatment (HR=1.64, 95% CI: 1.21-2.21, and HR=1.55, 95% CI: 1.10-2.17, respectively) were associated with a higher risk of mortality. For stage I(E) ALCL, AITL and PTCL NOS, 5-year OS is 59%, comparing favorably to historical outcome in advanced-stage disease. Superior outcome estimates were observed in patients treated with CMT.


Subject(s)
Immunoblastic Lymphadenopathy , Lymphoma, Large-Cell, Anaplastic , Lymphoma, T-Cell, Peripheral , Humans , Male , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/epidemiology , Lymphoma, T-Cell, Peripheral/therapy , Cohort Studies , Netherlands/epidemiology , Combined Modality Therapy , Prognosis
12.
Ann Hematol ; 103(3): 869-883, 2024 Mar.
Article in English | MEDLINE | ID: mdl-38040859

ABSTRACT

Peripheral T cell lymphoma, not otherwise specified (PTCL, NOS), is a heterogeneous and aggressive type of non-Hodgkin's lymphoma with a bleak prognosis. This study was designed to assess the value of EFS24 as an alternative clinical endpoint and identify prognosis-related factors in PTCL, NOS. Patients diagnosed with PTCL, NOS were retrospectively collected and slides were reviewed by two hematopathologists. EFS was defined as the time from diagnosis to the occurrence of disease progression after initial treatment, retreatment, or death. Subsequent overall survival (OS) was defined from EFS24 or time of progression, if it occurred within 24 months, to the last follow-up or death. 97 cases with complete follow-up were selected. Approximately 66 patients (68.04%) failed to achieve ES24, with the median OS of 12.17 months, and 5-year OS rate of 15.17%. While patients who reached EFS24 had a median OS of 60.57 months and a 5-year OS rate of 68.77%. Multivariate Cox analysis indicated that bone marrow involvement and elevated ß2 Microglobulin (ß2-MG) were associated with a poor prognosis. B symptoms, extranodal involvement more than one site, and a high Ki67 index were significant factors in predicting the failure of EFS24. EFS24 can help stratify the subsequent outcomes of PTCL, NOS. Patients who achieve EFS24 have a favorable prognosis, although it does not reach that of the general population. On the other hand, patients who do not achieve EFS24 have an extremely poor prognosis. Therefore, EFS24 can be used for patient risk stratification, patient counseling, and study design.


Subject(s)
Lymphoma, T-Cell, Peripheral , Humans , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/therapy , Lymphoma, T-Cell, Peripheral/pathology , Progression-Free Survival , Retrospective Studies , Clinical Relevance , Prognosis , Risk Factors
13.
Histopathology ; 84(3): 556-564, 2024 Feb.
Article in English | MEDLINE | ID: mdl-37988271

ABSTRACT

AIMS: Follicular helper T-cell (TFH) lymphoma of the angioimmunoblastic-type (AITL), one of the most prevalent T-cell lymphomas, typically encompasses proliferation of high endothelial venules and Epstein-Barr virus-positive immunoblasts, but neither infection with HHV8 nor association with Kaposi's sarcoma (KS) have been described. The aims of this study are to characterise the association between AITL and HHV8 infection or KS. METHODS AND RESULTS: Three male patients aged 49-76 years, HIV-negative, with concurrent nodal involvement by AITL and KS, were identified from our files and carefully studied. Two patients originated from countries where endemic KS occurs, including one with cutaneous KS. The lymphomas featured abundant vessels, expanded follicular dendritic cells and neoplastic TFH cells [PD1+ (three of three), ICOS+ (three of three), CXCL13+ (three of three), CD10+ (two of three), BCL6 (two of three)] but lacked EBV+ immunoblasts. The foci of KS consisted of subcapsular proliferations of ERG+, CD31+ and/or CD34+ , HHV8+ spindle cells. High-throughput sequencing showed AITL-associated mutations in TET2 (three of three), RHOA (G17V) (three of three) and IDH2 (R172) (two of three), which were absent in the microdissected KS component in two cases. Relapses in two patients consisted of AITL, without evidence of KS. No evidence of HHV8 infection was found in a control group of 23 AITL cases. CONCLUSION: Concurrent nodal involvement by AITL and KS is rare and identification of both neoplastic components may pose diagnostic challenges. The question of whether the association between AITL and KS may be fortuitous or could reflect the underlying immune dysfunction in AITL remains open.


Subject(s)
Epstein-Barr Virus Infections , Immunoblastic Lymphadenopathy , Lymphoma, T-Cell, Peripheral , Lymphoma, T-Cell , Sarcoma, Kaposi , Humans , Male , Herpesvirus 4, Human , Neoplasm Recurrence, Local , Immunoblastic Lymphadenopathy/complications , Immunoblastic Lymphadenopathy/genetics , Lymphoma, T-Cell/pathology , Lymphoma, T-Cell, Peripheral/complications , Lymphoma, T-Cell, Peripheral/diagnosis
15.
Haematologica ; 108(12): 3244-3260, 2023 Dec 01.
Article in English | MEDLINE | ID: mdl-38037801

ABSTRACT

While all peripheral T-cell lymphomas are uncommon, certain subtypes are truly rare, with less than a few hundred cases per year in the USA. There are often no dedicated clinical trials in these rare subtypes, and data are generally limited to case reports and retrospective case series. Therefore, clinical management is often based on this limited literature and extrapolation of data from the more common, nodal T-cell lymphomas in conjunction with personal experience. Nevertheless, thanks to tremendous pre-clinical efforts to understand these rare diseases, an increasing appreciation of the biological changes that underlie these entities is forming. In this review, we attempt to summarize the relevant literature regarding the initial management of certain rare subtypes, specifically subcutaneous panniculitis-like T-cell lymphoma, hepatosplenic T-cell lymphoma, intestinal T-cell lymphomas, and extranodal NK/T-cell lymphoma. While unequivocally established approaches in these diseases do not exist, we make cautious efforts to provide our approaches to clinical management when possible.


Subject(s)
Lymphoma, Extranodal NK-T-Cell , Lymphoma, T-Cell, Peripheral , Humans , T-Lymphocytes/pathology , Retrospective Studies , Killer Cells, Natural/pathology , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/therapy , Lymphoma, T-Cell, Peripheral/pathology , Lymphoma, Extranodal NK-T-Cell/diagnosis , Lymphoma, Extranodal NK-T-Cell/therapy
16.
Pathologie (Heidelb) ; 44(Suppl 3): 128-135, 2023 Dec.
Article in English | MEDLINE | ID: mdl-38047948

ABSTRACT

In this review focus article, we highlight the main modifications introduced in the latest 2022 International Consensus Classification and World Health Organization classification (ICC and WHO-HAEM5) of mature T (and NK) cell neoplasms (PTCLs) and consequent implications for diagnostic practice. The changes result from recent advances in the genomic and molecular characterization of PTCLs and enhanced understanding of their pathobiology. Specifically, consideration is given to the following groups of diseases: Epstein-Barr virus (EBV)-associated neoplasms; follicular helper T cell lymphoma; anaplastic large cell lymphomas; primary intestinal T and NK cell lymphomas and lymphoproliferative disorders; and PTCL, not otherwise specified.


Subject(s)
Epstein-Barr Virus Infections , Lymphoma, Large-Cell, Anaplastic , Lymphoma, T-Cell, Peripheral , Humans , Lymphoma, T-Cell, Peripheral/diagnosis , Epstein-Barr Virus Infections/diagnosis , Herpesvirus 4, Human/genetics , Killer Cells, Natural/pathology , Lymphoma, Large-Cell, Anaplastic/diagnosis
17.
J Cancer Res Ther ; 19(6): 1680-1684, 2023 Dec 01.
Article in English | MEDLINE | ID: mdl-38156938

ABSTRACT

Peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) is a highly aggressive lymphoma with a poor response to chemotherapy, frequent relapses, low overall survival, and poor prognosis, and is the most common form of PTCL. For relapsed/refractory (R/R) PTCLs, the efficacy of traditional chemotherapy is even worse, so clinical trials and new drugs become their therapeutic hope. The patient was a 43-year-old woman who complained of enlarged superficial lymph nodes (submandibular, neck, axillary, epitrochlear, and groin) and progressive aggravation of skin lesions, facial and limb edema, and subcutaneous masses. Histological analyses of lymph nodes and skin biopsy were suggestive of PTCL-NOS. The patient experienced failure of six lines of therapy, including multiple cycles of chemotherapy, chidamide, and BCL-2 inhibitors therapy, surprisingly, has a good response to PD-1 inhibitor combined with decitabine. We intend to provide some references for clinical practice.


Subject(s)
Antineoplastic Agents , Lymphoma, T-Cell, Peripheral , Female , Humans , Adult , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/drug therapy , Lymphoma, T-Cell, Peripheral/pathology , Decitabine/therapeutic use , Programmed Cell Death 1 Receptor , Neoplasm Recurrence, Local/drug therapy , Antineoplastic Agents/therapeutic use , Antineoplastic Combined Chemotherapy Protocols/therapeutic use
18.
Probl Radiac Med Radiobiol ; 28: 504-512, 2023 Dec.
Article in English, Ukrainian | MEDLINE | ID: mdl-38155144

ABSTRACT

Peripheral T-cell lymphomas (PTCLs) are a heterogeneous group of lymphoproliferative disorders, accounting for about 10 % of all non-Hodgkin lymphomas. The most common subtype is peripheral T-cell lymphoma, unclassified (PTCL-NOS), accounting for about 26 % of all PTCLs. PTCL-NOS is associated with less favorable overall survival (OS) and progression-free survival (PFS) compared with aggressive B-cell lymphomas. The role of radiation therapy in the treatment of PTCL-NOS is still not definitively defined. The results of many studies show that the addition of radiation therapy to the treatment regimen is associated with a significant improvement in survival in patients with early-stage PTCL-NOS, but in the later stages, the benefit of radiation therapy is not obvious. OBJECTIVE: peripheral T-cell lymphoma, unspecified is a clinically and biologically heterogeneous disease with a poor prognosis. Since the role of radiation therapy is still unclear, a study was conducted to evaluate the effectiveness of radiation therapy in peripheral T-cell lymphoma, unspecified. MATERIALS AND METHODS: The work is based on clinical observations and treatment results of patients who were diagnosed between 2013 and 2023 at the National Cancer Institute (in the period from 2020 to 2023, patients were observed and treated as part of research). 56 patients were included in the study. RESULTS: The work analyzed the immediate results of the treatment of patients with peripheral T-cell lymphoma, unspecified depending on the stage and type of treatment, as well as the overall survival of these patients. When analyzing the overall response to the treatment of patients with I/II stages of the disease, it was proven that this indicator is higher in the group of patients who received chemoradiotherapy, compared to patients who received only chemotherapy (100 % versus 83.3 %), and this indicator was higher due to patients who demonstrated a complete response to therapy (75 % vs. 50 %). Analyzing the response to treatment of patients with III/IV stages of the pathological process, it was established that there was no difference in the overall response to treatment, the level of complete and partial response to treatment. Analysis of the overall survival of patients with I/II stages of the disease, with a median follow-up of 60 months, demonstrated a significant improvement in overall survival in the group of patients who received chemoradiotherapy compared to the group of patients who received only radiation therapy (median 48 vs. 22 months). Overall 1-year (78 % vs. 69 %), 3-year (64 % vs. 40 %), and 5-year (48 % vs. 35 %) were also higher in the chemoradiotherapy group. In the group of patients with III/IV stages of the disease, there was no difference in overall survival between patients who received chemoradiotherapy and patients who received only chemotherapy (median 16 vs. 13 months, 1-year survival 54 vs. 52, 3-year survival 33 vs. 30 and 5-year overall survival of 23 vs. 20 %. CONCLUSIONS: The addition of radiation therapy to the treatment plan demonstrated a significant improvement in the overall response and overall survival of patients with peripheral T-cell lymphomas, unspecified with I and II stages of the pathological process, but in III and IV stages of the disease, the benefit of radiation therapy has not been proven.


Subject(s)
Lymphoma, T-Cell, Peripheral , Humans , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/drug therapy , Lymphoma, T-Cell, Peripheral/pathology , Treatment Outcome , Antineoplastic Combined Chemotherapy Protocols/therapeutic use , Chemoradiotherapy , Pathologic Complete Response
19.
Leuk Lymphoma ; 64(14): 2258-2268, 2023 Dec.
Article in English | MEDLINE | ID: mdl-37706491

ABSTRACT

We conducted a population-based study of patients >65 years, diagnosed 2008-2017, with peripheral T-cell lymphoma (PTCL) using SEER-Medicare. Associations between PTCL subtype, treatment regimen, comorbidity, and mortality were assessed using the Kaplan-Meier method and multivariable Cox regression. Amongst the 2,546 patients, the median age was 77 years (interquartile range, 71-83). 5-year overall survival (OS) ranged from 22.2% to 37.3% depending on PTCL subtype. The most common frontline regimen was cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP). 5-year OS rate was 47.0% for patients treated with etoposide + CHOP (N = 67; CHOEP), 33.7% for those treated with CHOP (N = 732), and 23.8% for patients treated with non-anthracycline-containing regimens (N = 105; p < 0.001). In patients without comorbidities, CHOEP remained independently associated with improved OS (HR 0.52, 95% CI,0.30-0.91). Median OS was 1.2 years from initiation of second-line therapy (N = 228) independent of treatment regimen. Frontline but not second-line treatment regimen is associated with OS in older patients with PTCL.


Subject(s)
Lymphoma, T-Cell, Peripheral , Humans , Aged , United States , Lymphoma, T-Cell, Peripheral/diagnosis , Lymphoma, T-Cell, Peripheral/drug therapy , Lymphoma, T-Cell, Peripheral/epidemiology , Medicare , Doxorubicin/adverse effects , Vincristine/adverse effects , Cyclophosphamide , Prednisone/adverse effects , Comorbidity , Antineoplastic Combined Chemotherapy Protocols/adverse effects
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