Primary Cutaneous T-Cell Lymphoma: Review and Current Concepts

2000 ◽  
Vol 18 (15) ◽  
pp. 2908-2925 ◽  
Author(s):  
Richard S. Siegel ◽  
Tomi Pandolfino ◽  
Joan Guitart ◽  
Steven Rosen ◽  
Timothy M. Kuzel
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Gene Rearrangement ◽  
Medical Literature ◽  
Cell Lymphoma ◽  
Large Cell ◽  
Cutaneous T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Skin Abnormalities ◽  
Indolent Disease

PURPOSE: Primary cutaneous T-cell lymphomas (CTCLs) encompass a wide variety of lymphomas that are characterized by the localization of the malignant lymphocytes to the skin at presentation. Advances in molecular biologic techniques, including immunophenotyping and gene rearrangement studies to determine clonality, have led to more frequent diagnosis of CTCL as well as more consistent subclassification of these entities. However, there continues to be confusion in the classification, prognosis, and management of patients with CTCL. The purpose of this review is to present a summary of the diagnosis, prognosis, and treatment of CTCL, with specific emphasis on mycosis fungoides (MF) and Sézary syndrome (SS). We also present a detailed discussion of the entities that make up the differential diagnosis of CTCL. DESIGN: We reviewed the medical literature on CTCL and other diseases that make up the differential diagnosis of CTCL. Results and CONCLUSION: MF and SS are the most common forms of CTCL. The etiology of this disease is still unknown. Patients may go for months to years with skin abnormalities before being diagnosed. MF/SS is an indolent disease and patients with T1 disease have a normal life expectancy. Patients who undergo transformation to large-cell lymphoma (8% to 23% of patients) have a poor prognosis, with mean survival ranging from 2 to 19 months. Treatment for MF/SS continues to be palliative. There are many new therapies that are currently being investigated in clinical trials, and the DAB389IL-2 fusion protein was recently approved for the treatment of refractory MF/SS.

scholarly journals Primary Cutaneous CD30-Positive Large T-Cell Lymphoma in an 80-Year-Old Man: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Rehan Hussain ◽  
Amir Bajoghli
Keyword(s):  
T Cell ◽  
Cell Lymphoma ◽  
Large Cell ◽  
T Cell Lymphoma ◽  
Cutaneous T Cell Lymphoma ◽  
Left Forearm ◽  
Favorable Prognosis ◽  
History Of ◽  
Indolent Disease ◽  
Rule Out

Primary cutaneous CD30-positive large cell lymphoma (CD30+ PCLCL) is a rare subtype of cutaneous T-cell lymphoma (CTCL) that can present in a variety of ways. We report a patient with a three-month history of an enlarging, exophytic mass with two smaller satellite lesions on the left forearm. Biopsy of the skin stained positive for CD30, and, after thorough systemic evaluation, a diagnosis of CD30+ PCLCL was made. When PCLCL is suspected, it is important to perform immunohistological studies for CD30 types and conduct a thorough workup to rule out systemic LCL. These measures will reduce the use of unnecessarily aggressive chemotherapy regimens for CD30+ PCLCL, an indolent disease with a favorable prognosis.

scholarly journals Keratoacanthoma Centrifugum Marginatum with Spontaneous Regression and Its Possible Differential Diagnosis

2018 ◽  
Vol 11 (3) ◽  
pp. 671-675 ◽  
Author(s):  
Ryo Amagai ◽  
Taku Fujimura ◽  
Yumi Kambayashi ◽  
Sadanori Furudate ◽  
Yota Sato ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Cd8 T Cells ◽  
Spontaneous Regression ◽  
Cell Lymphoma ◽  
Tumor Infiltrating Lymphocytes ◽  
Inflammatory Cells ◽  
Cutaneous T Cell Lymphoma ◽  
T Cell Lymphomas ◽  
Infiltrating Lymphocytes

Keratoacanthoma centrifugum marignatum (KCM) is a rare variant of keratoacanthoma, which is characterized by the dense infiltration of inflammatory cells throughout the dermis, especially around the keratinocytic islands. Therefore, it is sometimes difficult to differentiate between KCM and cutaneous T-cell lymphomas. In this report, we describe a case of KCM with spontaneous regression that showed dense infiltration of CD3+CD8+ T cells. Our present case suggested the importance of investigating tumor-infiltrating lymphocytes to avoid the misdiagnosis of KCM as cutaneous T-cell lymphoma.

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.

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.

A Young Man with Fever and Skin Nodules

2008 ◽  
Vol 12 (6) ◽  
pp. 299-301
Author(s):  
Mark A. Lomaga ◽  
Scott Walsh
Keyword(s):  
Differential Diagnosis ◽  
T Cell ◽  
Clinical Presentation ◽  
Rare Variants ◽  
Recent Literature ◽  
Cell Lymphoma ◽  
T Cell Lymphoma ◽  
Cutaneous T Cell Lymphoma ◽  
Subcutaneous Nodules ◽  
Skin Nodules

Background: There are two rare variants of cutaneous T-cell lymphoma (CTCL) that primarily involve the subcutis. These include subcutaneous panniculitis-like T-cell lymphoma (SPTL) and cutaneous γ/δ T-cell lymphoma. Objective: This case report describes the clinical presentation, diagnosis, and treatment of a young man with probable SPTL. A review of recent literature outlining the differences between SPTL and cutaneous γ/δ T-cell lymphoma is discussed. Conclusion: The differential diagnosis in patients presenting with subcutaneous nodules and constitutional symptoms should include CTCL.

Brentuximab vedotin in real life, a seven year experience in patients with refractory/relapsed CD30+ T cell lymphoma

2020 ◽  
pp. 107815522096861
Author(s):  
Lucie Oberic ◽  
Faustine Delzor ◽  
Caroline Protin ◽  
Sophie Perriat ◽  
Camille Laurent ◽  
...  
Keyword(s):  
T Cell ◽  
Combination Treatment ◽  
Cell Lymphoma ◽  
Real Life ◽  
Progression Free Survival ◽  
Large Cell ◽  
Complete Response ◽  
Brentuximab Vedotin ◽  
T Cell Lymphoma ◽  
T Cell Lymphomas

Introduction Brentuximab vedotin (Bv) has been approved for the treatment of Refractory/Relapsed (R/R) Anaplastic Large Cell Lymphomas (ALCL) and cutaneous T-Cell Lymphomas, but is also effective in other CD30+ malignancies. We report here the outcomes of patients with various R/R Peripheral T Cell Lymphoma (PTCL) treated with Bv in real life practice. Method This was a retrospective, single-center study based on medical records of patients with R/R PTCL treated either with Bv alone or in combination with chemotherapy. Results Among 27 patients treated with Bv, neutropenia was the main serious adverse event observed in particular when Bv was used as combination treatment. The complete Response Rates (CRR) was 40.7%; it was significantly improved when Bv was used as combination treatment. The majority of eligible patients (7/10) underwent Stem Cell Transplantation. Median Progression Free Survival (PFS) and Overall Survival (OS) were 5.2 months and 12.5 months respectively. Conclusion Our current study shows that Bv used in combination with chemotherapy provides a high CRR and thereby allows SCT in R/R PTCL. The use of Bv treatments in this setting warrants further investigation.

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