Primary nodal peripheral T-cell lymphomas: diagnosis and therapeutic considerations

Lage, Luís Alberto de Pádua Covas; Cabral, Tamara Carvalho dos Santos; Costa, Renata de Oliveira; Gonçalves, Marianne de Castro; Levy, Débora; Zerbini, Maria Cláudia Nogueira; Pereira, Juliana

doi:10.1016/j.bjhh.2015.03.017

Cited by 12 publications

(15 citation statements)

References 40 publications

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“…Even for the most expert hematopathologists, the differentiation between PTCL/NOS and ALK-negative ALCL or AITL is quite problematic [1]. This analysis can be more troublesome particularly if tumor sample is scarce, preventing analysis of the complete architecture of the lymph node that is commonly found in samples obtained by core-biopsy [1,23,24].…”

Section: Discussionmentioning

confidence: 99%

GATA 3 Gene Overexpression Is A Biomarker of Adverse Prognosis for Peripheral T-Cell Lymphoma, Not Otherwise Specified and for ALK-Negative Anaplastic Large Cell Lymphoma: An Exploratory Analysis of 80 Latin American Cases of nodal PTCLs

Lage¹,

Brito²,

Levy³

et al. 2020

Preprint

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Background: Nodal peripheral T-cell lymphomas (nPTCLs) encompass a heterogeneous group of mature and aggressive lymphoid malignancies, including peripheral T-cell lymphoma, not otherwise specified (PTCL/NOS), angioimmunoblastic T-cell lymphoma (AITL) and anaplastic large cell lymphoma (ALCL) ALK-positive and ALK-negative. Their differential diagnosis and prognosis are an issue in the clinical practice. Accurate biomarkers to refine the different subtypes of nPTCLs and to stratify their prognosis are essential to improve their treatment approach. The aim of this study was to test the prognostic impact of GATA3 gene expression, and its capability to discriminate the different subtypes of nPTCLs. Patients and Methods: From 2000 to 2017, 80 patients with nPTCLs were eligible for GATA3 gene expression analysis that was assessed retrospectively by quantitative real time PCR (qRT-PCR) of neoplastic biopsies in Formalin-Fixed Paraffin-Embedded samples (FFPE). Results: Median age was 49 years old (IqR 34-59), 43/80 (53.7%) were male. Median follow-up was 1.72 years. Of them, 36.3% were classified as PTCL/NOS, 31.2% ALK-negative ALCL, 21.2% ALK-positive ALCL and 11.3% AITL. The majority of cases had advanced stage (III/IV). Two-year estimated overall survival (OS) and progression-free survival (PFS) were 52.2% and 39.5%, respectively. The median GATA3 gene expression level was 0.49% (range 0 – 7.07) in all cohort, it was 0.11% for ALK-positive ALCL, 0.46% for ALK-negative ALCL, 0.86% for PTCL/NOS and 0.67% for AITL. The difference of GATA3 gene expression among distinct variants of nPTCLs was statistically significant (p < 0.001). GATA3 gene expression levels ≥ 0.71% discriminate PTCL/NOS from ALK-negative ALCL and AITL with sensitivity of 62% and specificity of 80.3%. GATA3 gene expression levels ≥ median was associated with poor 2-year OS for PTCL/NOS (46.7% x 21.4%, p=0.04) and for ALK-negative ALCL (85.7% x 54.5%, p=0.04). Conclusion: Despite the relative small and heterogeneous group of patients with nPTCLs, GATA3 gene overexpression may be an important biomarker associated with poor prognosis in PTCL/NOS and ALK-negative ALCL. Moreover it may also discriminate different subtypes of nPTCLs. Further studies with larger series of patients should confirm our findings.

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Section: Discussionmentioning

confidence: 99%

GATA 3 Gene Overexpression Is A Biomarker of Adverse Prognosis for Peripheral T-Cell Lymphoma, Not Otherwise Specified and for ALK-Negative Anaplastic Large Cell Lymphoma: An Exploratory Analysis of 80 Latin American Cases of nodal PTCLs

Lage¹,

Brito²,

Levy³

et al. 2020

Preprint

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“…They most frequently present at an advanced stage, and especially the aggressive types have a short survival [3]. Current standard chemotherapy regimens such as CHOP, has insufficient efficacies, and no definitive curative therapy has been shown to be successful in treating PTCL-NOS [7,8]. According to the International T-cell Lymphoma Study, the overall survival (OS) and failure-free survival (FFS) with PTCL-NOS at 10 to 15 years is 10% [9].…”

Section: Discussionmentioning

confidence: 99%

Peripheral T- cell lymphoma not otherwise specified - a patient with extranodal pancreatic involvement detected by FDG PET/CT: a case report and review of the literature

Demirel¹,

Efeturk²,

Uçmak³

et al. 2018

Nucl Med Biomed Imaging

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Peripheral T-cell lymphomas, (PTCL) are rare and heterogeneous group of lymphoid malignancies arising from mature or precursor T-cells. The most common sites of extranodal involvement in peripheral T-cell lymphomas are the spleen, liver, skin, lungs and subcutaneous tissue. The frequency of primary pancreatic involvement has been reported as 2.2% and secondary pancreatic involvement 0.2-2% in NHL patients. FDG PET/CT has been found to efficiently identify more sites of extranodal disease compared to contrast enhanced CT scan. The sensitivity and specificity of PET/CT in detecting extranodal lymphoma is 88% and 100% respectively. To our knowledge, no cases with pancreatic involvement of peripheral T-cell lymphoma detected by PET/CT have been reported in the literature.

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“…The 3-year progression-free survival for pirarubicin-CHOP was 75% versus 33% with DOXO-CHOP [19] . The 5-year survival rate with CHOP is only 19% in PCTL-NOS [20] . Adjuvant radiation therapy to DOXO-based chemotherapy in early stage PCTL-NOS may improve the 3-year survival significantly, that is, 49.7% versus 23.1% (DOXO-based chemotherapy alone) [21] …”

Section: Discussionmentioning

confidence: 99%

Pegylated liposomal-encapsulated doxorubicin in cutaneous composite lymphoma

Wollina¹,

Langner²,

Hansel³

et al. 2016

Medicine

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Background:Cutaneous composite lymphomas are very rare. Their treatment depends upon the different contributing lymphoma entities. Peripheral T-cell lymphoma, not otherwise specified, (PTCL-NOS) represents an aggressive lymphoma subtype. Follicular cutaneous B-cell lymphoma (FCBCL) runs an indolent course. Treatment with pegylated liposomal encapsulated doxorubicin (PLE-DOXO) has yet not been reported in this entity.Case presentation:A 73-year-old male patient presented with 3 rapidly growing, painful nodules on his left leg. He was diagnosed as composite cutaneous lymphoma consisting of PTCL-NOS and FCBCL. All lesions had been surgically removed. Staging was unremarkable. After 4 months a relapse occurred with involvement of inguinal lymph nodes and systemic treatment with PEL-DOXO 20 mg/ m2 every 3 weeks was initiated. After 6 cycles PLE-DOXO, which were well tolerated without grade 3 or 4 toxicities, a mixed response was obtained with complete remission of cutaneous lesions.Lymph nodes were treated by radiotherapy. A second relapse occurred after 8 months and various polychemotherapy regimens were applied without remission. The overall survival was 28 months.Conclusion:PEL-DOXO is a possible initial systemic treatment in case of PCTL-NOS. Whether polychemotherapy offers an advantage for survival remains questionable but further investigations are needed.

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Primary nodal peripheral T-cell lymphomas: diagnosis and therapeutic considerations

Cited by 12 publications

References 40 publications

<strong><em>GATA 3</em> Gene Overexpression Is A Biomarker of Adverse Prognosis for Peripheral T-Cell Lymphoma, Not Otherwise Specified and for ALK-Negative Anaplastic Large Cell Lymphoma: An Exploratory Analysis of 80 Latin American Cases of nodal PTCLs</strong>

<strong><em>GATA 3</em> Gene Overexpression Is A Biomarker of Adverse Prognosis for Peripheral T-Cell Lymphoma, Not Otherwise Specified and for ALK-Negative Anaplastic Large Cell Lymphoma: An Exploratory Analysis of 80 Latin American Cases of nodal PTCLs</strong>

Peripheral T- cell lymphoma not otherwise specified - a patient with extranodal pancreatic involvement detected by FDG PET/CT: a case report and review of the literature

Pegylated liposomal-encapsulated doxorubicin in cutaneous composite lymphoma

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