scholarly journals Lack of PRAME Expression in Cutaneous T-Cell Lymphomas

2021 ◽  
Vol 9 (1) ◽  
pp. 11-16
Author(s):  
Chau M. Bui ◽  
Sumire Kitahara ◽  
Wonwoo Shon ◽  
Tatsiana Pukhalskaya ◽  
Bruce R. Smoller
Keyword(s):  
T Cell ◽  
Poor Prognosis ◽  
Lymphoproliferative Disorder ◽  
Cell Lymphoma ◽  
Hematologic Malignancies ◽  
T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Chemotherapeutic Response ◽  
Malignant Lesions ◽  
Cancer Testis

Cutaneous T-cell lymphomas (CTCLs) are rare tumors with no established markers that can reliably distinguish between benign and malignant lesions. Preferentially Expressed Antigen in Melanoma (PRAME) is a cancer/testis antigen that is found in many solid and hematologic malignancies. PRAME overexpression typically portends a poor prognosis and lower chemotherapeutic response. To date, no studies have established a role for PRAME in CTCL. An analysis was performed on 47 cases definitively diagnosed as CTCL: 25 cases of mycosis fungoides, 2 of Sezary syndrome, 5 of CD30+ lymphoproliferative disorder, 7 of primary cutaneous anaplastic large T-cell lymphoma, 3 of primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder, 1 of subcutaneous panniculitis-like T-cell lymphoma, and 4 of angiocentric T-cell lymphoma. PRAME immunohistochemistry was completely negative in all cases. PRAME expression was not found in any CTCL subtypes, suggesting that the pathogenesis of CTCL is not mediated by PRAME. Further study is required to identify biomarkers that might aid in the diagnosis and prognostication of CTCLs.

scholarly journals A rare case of peripheral T cell lymphoma, not otherwise specified with cutaneous involvement and diffuse systemic metastasis

2021 ◽  
Vol 7 (3) ◽  
pp. 476
Author(s):  
Pandharinath K. Khade ◽  
Agni K. Bose ◽  
Vidya D. Kharkar
Keyword(s):  
Gastrointestinal Tract ◽  
T Cell ◽  
Poor Prognosis ◽  
Cell Lymphoma ◽  
Palliative Radiotherapy ◽  
T Cell Lymphoma ◽  
Lower Limbs ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas

<p class="abstract">Peripheral T-cell lymphoma (PTCL), a subdivision of T-cell non-Hodgkin lymphomas (NHLs), is rare and different from the more common cutaneous T-cell lymphomas. PTCL is a diverse group of disorders and carries a poor prognosis. Peripheral T cell lymphoma not otherwise specified (PTCL-NOS) is the most common and aggressive disorder out of the group, which involves the lymph nodes followed by the skin, liver, and gastrointestinal tract. We hereby report a case of a 60 years old female who presented to us with complaints of multiple painful dark-coloured nodular lesions and indurated plaques over bilateral upper limbs, lower limbs and trunk, for 3 months. After detail laboratory investigations, histopathological and immunohistochemistry which was positive for CD30 antigen we confirmed the diagnosis as PTCL-NOS.  Radiological imaging showed involvement of gastrointestinal tract, adrenal gland and vertebrae. After thorough workup the patient was started on palliative radiotherapy, unfortunately she succumbed to death. In conclusion, PTCL-NOS with CD30 positivity is rare and aggressive with a poor prognosis, it is important to identify such cases and work in conjugation with an oncologist for proper management.</p>

scholarly journals Transformation of T-Cell Acute Lymphoblastic Lymphoma to Peripheral T-Cell Lymphoma: A Report of Two Cases

2018 ◽  
Vol 2018 ◽  
pp. 1-11
Author(s):  
Michael Markow ◽  
Abu-Sayeef Mirza ◽  
Lia Perez ◽  
Haipeng Shao ◽  
Pedro Horna ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Lymphoblastic Leukemia ◽  
Rare Event ◽  
Cell Lineage ◽  
Hematologic Malignancies ◽  
T Cell Lymphoma ◽  
Molecular Characteristics ◽  
Peripheral T Cell Lymphoma ◽  
T Cell Lymphomas

Nonhepatosplenic/noncutaneous γδ peripheral T-cell lymphoma (NHNCγδ PTCL) represents a miscellaneous group of unrelated T-cell lymphomas of which only isolated cases have been reported. We describe two cases of transformation from T-lymphoblastic leukemia/lymphoma to NHNCγδ PTCL. Transformation into more aggressive disease is a rare event in T-cell lineage-derived hematologic malignancies compared to B-cell neoplasms. Nevertheless, both of our cases involved relapse as PTCL manifested with skin involvement and an overt shift from blastic morphology to large granular leukemia-like mature T cells. Among other notable molecular characteristics, expression of immature markers such as TdT was lost in both cases. Based on cytogenetics, phenotype, and morphology, both patients represent a novel phenomenon of clonal transformation from T-ALL to PTCL which has rarely been reported in the literature. Such transformation may carry important diagnostic and biological implications.

scholarly journals Overexpression of MYC and BCL2 Predicts Poor Prognosis in Patients with Extranodal NK/T-cell Lymphoma, Nasal Type

2017 ◽  
Vol 8 (5) ◽  
pp. 793-800 ◽  
Author(s):  
Jing-hua Wang ◽  
Xi-wen Bi ◽  
Peng-fei Li ◽  
Zhong-jun Xia ◽  
Hui-qiang Huang ◽  
...  
Keyword(s):  
T Cell ◽  
Poor Prognosis ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Nasal Type ◽  
Nk T Cell

Peripheral T-cell lymphomas unspecified presenting in the skin: analysis of prognostic factors in a group of 82 patients

Blood ◽  
2003 ◽  
Vol 102 (6) ◽  
pp. 2213-2219 ◽  
Author(s):  
Marcel W. Bekkenk ◽  
Maarten H. Vermeer ◽  
Patty M. Jansen ◽  
Ariënne M. W. van Marion ◽  
Marijke R. Canninga-van Dijk ◽  
...  
Keyword(s):  
T Cell ◽  
Cell Size ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Skin Lesions ◽  
T Cell Lymphoma ◽  
Peripheral T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Nor Phenotype ◽  
Peripheral T Cell Lymphomas

Abstract In the present study the clinicopathologic and immunophenotypic features of 82 patients with a CD30– peripheral T-cell lymphoma, unspecified, presenting in the skin were evaluated. The purpose of this study was to find out whether subdivision of these lymphomas on the basis of cell size, phenotype, or presentation with only skin lesions is clinically relevant. The study group included 46 primary cutaneous CD30– large cell lymphomas and 17 small/medium-sized T-cell lymphomas as well as 17 peripheral T-cell lymphomas with both skin and extracutaneous disease at the time of diagnosis. Patients with primary cutaneous small- or medium-sized T-cell lymphomas had a significantly better prognosis (5-year-overall survival, 45%) than patients with primary cutaneous CD30– large T-cell lymphomas (12%) and patients presenting with concurrent extracutaneous disease (12%). The favorable prognosis in this group with primary cutaneous small- or medium-sized T-cell lymphomas was particularly found in patients presenting with localized skin lesions expressing a CD3+CD4+CD8– phenotype. In the primary cutaneous T-cell lymphoma (CTCL) group and in the concurrent group, neither extent of skin lesions nor phenotype had any effect on survival. Our results indicate that peripheral T-cell lymphomas, unspecified, presenting in the skin have an unfavorable prognosis, irrespective of the presence or absence of extracutaneous disease at the time of diagnosis, cell size, and expression of a CD4+ or CD8+ phenotype. The only exception was a group of primary cutaneous small- or medium-sized pleomorphic CTCLs with a CD3+CD4+CD8– phenotype and presenting with localized skin lesions.

scholarly journals Successful Treatment of Advanced Primary Cutaneous Peripheral T-Cell Lymphoma with Oral Bexarotene Monotherapy

2018 ◽  
Vol 11 (1) ◽  
pp. 212-215 ◽  
Author(s):  
Yota Sato ◽  
Taku Fujimura ◽  
Yumi Kambayashi ◽  
Akira Hashimoto ◽  
Setsuya Aiba
Keyword(s):  
T Cell ◽  
Successful Treatment ◽  
Cell Lymphoma ◽  
Skin Lesions ◽  
Retinoid X Receptor ◽  
T Cell Lymphoma ◽  
Third Generation ◽  
Peripheral T Cell Lymphoma ◽  
Not Otherwise Specified ◽  
T Cell Lymphomas

Bexarotene is a third-generation retinoid X receptor-selective retinoid that is widely used for the early treatment of advanced-stage cutaneous T-cell lymphomas. In this report, we describe a case of successful treatment of advanced primary cutaneous peripheral T-cell lymphoma not otherwise specified (PTCL-NOS) with oral bexarotene monotherapy. After the administration of oral bexarotene at a dose of 300 mg/m2/day, all skin lesions and lymph nodes regressed, and complete remission was achieved for 1 year. Our case suggested that bexarotene monotherapy could be one of the possible therapies for the treatment of primary cutaneous PTCL-NOS.

Results From a Prospective, Open-Label, Phase II Trial of Bendamustine in Refractory or Relapsed T-Cell Lymphomas: The BENTLY Trial

2013 ◽  
Vol 31 (1) ◽  
pp. 104-110 ◽  
Author(s):  
Gandhi Damaj ◽  
Rémy Gressin ◽  
Krimo Bouabdallah ◽  
Guillaume Cartron ◽  
Bachra Choufi ◽  
...  
Keyword(s):  
T Cell ◽  
Response Rate ◽  
Cell Lymphoma ◽  
Single Agent ◽  
T Cell Lymphoma ◽  
Open Label ◽  
Previous Response ◽  
Angioimmunoblastic Lymphadenopathy ◽  
Prior Chemotherapy ◽  
T Cell Lymphomas

Purpose To determine the efficacy and safety of bendamustine as a single agent in refractory or relapsed T-cell lymphomas. Patients and Methods Patients with histologically confirmed peripheral T-cell lymphoma (PTCL) or cutaneous T-cell lymphoma who progressed after one or more lines of prior chemotherapy received bendamustine at 120 mg/m2 per day on days 1 through 2 every 3 weeks for six cycles. The primary end point was overall response rate (ORR). Secondary end points were duration of response (DOR), progression-free survival (PFS), and overall survival (OS). Results Of the 60 patients included, 27 (45%) were refractory to their last prior chemotherapy, and the median duration of the best previous response was 6.6 months. Histology was predominantly angioimmunoblastic lymphadenopathy and PTCL not otherwise specified. The disease was disseminated in the majority of patients (87%). The median number of previous lines of chemotherapy was one (range, one to three). Twenty patients (33%) received fewer than three cycles of bendamustine, mostly because of disease progression. In the intent-to-treat population, the ORR was 50%, including complete response in 17 patients (28%) and partial response in 13 patients (22%). Bendamustine showed consistent efficacy independent of major disease characteristics. The median values for DoR, PFS, and OS were 3.5, 3.6, and 6.2 months, respectively. The most frequent grade 3 to 4 adverse events were neutropenia (30%), thrombocytopenia (24%), and infections (20%). Conclusion Bendamustine showed an encouraging high response rate across the two major PTCL subtypes, independent of age and prior treatment, with acceptable toxicity in refractory or relapsed T-cell lymphoma.

The T-Cell Activation Markers CD30 and OX40/CD134 Are Expressed in Nonoverlapping Subsets of Peripheral T-Cell Lymphoma

Blood ◽  
1999 ◽  
Vol 93 (10) ◽  
pp. 3487-3493 ◽  
Author(s):  
Dan Jones ◽  
Christopher D.M. Fletcher ◽  
Karen Pulford ◽  
Aliakbar Shahsafaei ◽  
David M. Dorfman
Keyword(s):  
T Cells ◽  
T Cell ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Tnf Receptor ◽  
T Cell Lymphomas ◽  
Tumor Types ◽  
Tnf Receptor Family ◽  
Receptor Family

The tumor necrosis factor (TNF) receptor family includes several important markers of activation in T cells. We examined expression patterns of two T-cell-associated members of these receptors, namely CD30 and OX40/CD134, in 148 cases of T-cell lymphoma to identify possible objective immunohistochemical criteria for subclassification of these tumors. CD30 expression was characteristic of tumors with an anaplastic (46/47 cases [98%]) or large-cell (10/21 [48%]) morphology and was seen in only scattered cells in other tumor types. In contrast, large numbers of OX40/CD134+ tumors cells were typical of angioimmunoblastic lymphoma (15/16 [94%]), angiocentric lymphoma (4/4), a subset of large-cell lymphomas (10/21 [48%]), and lymphomas with a prominent histiocytic component (6/7 [86%]). Strong OX40/CD134 and CD30 coexpression was seen in only 4% of tumors, typically those with an anaplastic/Hodgkin’s-like appearance. OX40/CD134 expression was characteristic of tumors composed of activated CD4+ T cells and was not seen in small-cell T-cell lymphomas, lymphoblastic lymphomas, or other tumor types, including B-cell lymphomas or carcinomas. These results suggest that immunostaining for OX40/CD134 may be helpful in subclassification of peripheral T-cell lymphomas and that the patterns of TNF receptor family expression in these tumors may parallel those seen within nonneoplastic helper T-cell subsets.

scholarly journals Non Hodgkin T cell lymphoma: an atypical clinical presentation

2013 ◽  
Vol 88 (2) ◽  
pp. 264-267
Author(s):  
Paula Maio ◽  
Diogo Bento ◽  
Raquel Vieira ◽  
Ana Afonso ◽  
Fernanda Sachse ◽  
...  
Keyword(s):  
T Cell ◽  
Clinical Presentation ◽  
Cell Lymphoma ◽  
Systemic Disease ◽  
Skin Lesions ◽  
T Cell Lymphoma ◽  
Lymphocyte Population ◽  
T Cell Lymphomas ◽  
Atypical Clinical Presentation ◽  
New Skin

Cytotoxic lymphomas comprise a spectrum of peripheral T-cell lymphomas that can have a initial or late cutaneous presentation. We describe a 46-year-old man from Cape Verde, with a dermatosis involving his face and trunk, consisting of monomorphic papules with a smooth surface and both motor and sensory polyneuropathy.The hypothesis of leprosy was supported by the clinical and initial hystopathological findings and the patient was referred to our hospital with suspected Hansen's disease. In the new skin and lymph node biopsies a lymphocyte population was identified whose immunohystochemistry study allowed the diagnosis of T-cell lymphoma with expression of cytotoxic markers. The patient was started on chemotherapy with initial remission of the skin lesions but, subsequently, progression of systemic disease.

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