Primary cutaneous aggressive epidermotropic CD8 positive cytotoxic T-cell lymphoma of the ear

We report a case of an epidermotropic CD8+ cutaneous T-cell lymphoma which initially presented as an ulcerated lesion of the pinna. Although T-cell lymphomas may present as cutaneous lesions, the ear is rarely involved. This uncommon presentation and the need for multiple biopsies means that the diagnosis of these lesions may be delayed or missed. A high index of suspicion is required when evaluating cutaneous lesions in the head and neck area.

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Initial Report of a Cutaneous T-Cell Lymphoma Appearing on the Auricular Helix

Ear Nose & Throat Journal ◽

10.1177/014556130007900513 ◽

2000 ◽

Vol 79 (5) ◽

pp. 391-396 ◽

Cited By ~ 7

Author(s):

Brian J. Baumgartner ◽

Vincent Eusterman ◽

Jerome Myers ◽

Phillip Massengill

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

Skin Lesions ◽

T Cell Lymphoma ◽

Cutaneous T Cell Lymphoma ◽

Initial Report ◽

Delay In Diagnosis ◽

Multiple Biopsies ◽

Key Factor ◽

High Index Of Suspicion

We report a case of cutaneous T-cell lymphoma in which the first sign of disease was involvement of the superior auricular helix. A review of the literature reveals that T-cell lymphoma often presents cutaneously, but it usually does not involve the ear, and an auricular lesion is rarely the first sign. The uncommon nature of this presentation, in addition to the potential need for multiple biopsies for a tissue diagnosis, can lead to a delay in diagnosis. When evaluating skin lesions in the head and neck, a high index of suspicion for cutaneous T-cell lymphoma is a key factor in its early diagnosis.

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Primary Cutaneous Peripheral T-Cell Lymphoma Not Otherwise Specified: A Rapidly Progressive Variant of Cutaneous T-Cell Lymphoma

Case Reports in Oncological Medicine ◽

10.1155/2015/429068 ◽

2015 ◽

Vol 2015 ◽

pp. 1-3 ◽

Cited By ~ 4

Author(s):

Kimberly Aderhold ◽

Lisa Carpenter ◽

Krysta Brown ◽

Anthony Donato

Keyword(s):

T Cell ◽

Mycosis Fungoides ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Rapid Progression ◽

Entire Body ◽

Cutaneous T Cell Lymphoma ◽

Peripheral T Cell Lymphoma ◽

Multiple Biopsies ◽

Skin Conditions

Primary Cutaneous Peripheral T-Cell Lymphoma NOS (PTL-NOS) is a rare, progressive, fatal dermatologic disease that presents with features similar to many common benign plaque-like skin conditions, making recognition of its distinguishing features critical for early diagnosis and treatment (Bolognia et al., 2008). A 78-year-old woman presented to ambulatory care with a single 5 cm nodule on her shoulder that had developed rapidly over 1-2 weeks. Examination was suspicious for malignancy and a biopsy was performed. Biopsy results demonstrated CD4 positivity, consistent with Mycosis Fungoides with coexpression of CD5, CD47, and CD7. Within three months her cancer had progressed into diffuse lesions spanning her entire body. As rapid progression is usually uncharacteristic of Mycosis Fungoides, her diagnosis was amended to PTL-NOS. Cutaneous T-Cell Lymphoma (CTCL) should be suspected in patients with patches, plaques, erythroderma, or papules that persist or multiply despite conservative treatment. Singular biopsies are often nondiagnostic, requiring a high degree of suspicion if there is deviation from the anticipated clinical course. Multiple biopsies are often necessary to make the diagnosis. Physicians caring for patients with rapidly progressive, nonspecific dermatoses with features described above should keep more uncommon forms of CTCL in mind and refer for early biopsy.

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Treg phenotype in T-cell lymphomas

Journal of Clinical Oncology ◽

10.1200/jco.2007.25.18_suppl.18513 ◽

2007 ◽

Vol 25 (18_suppl) ◽

pp. 18513-18513

Author(s):

D. Morales ◽

B. E. Beltran ◽

C. Castañeda ◽

A. Carrasco ◽

P. Quiñones ◽

...

Keyword(s):

T Cells ◽

T Cell ◽

Cell Lymphoma ◽

Foxp3 Expression ◽

Tumour Cells ◽

T Cell Lymphoma ◽

Immunohistochemical Method ◽

Cytotoxic T Cell ◽

T Cell Lymphomas ◽

Treg Phenotype

18513 Background: Foxp3 is a key regulatory gene required for the development and function of: regulatory CD4+CD25high T cells (Treg) specialized in maintaining the balance between immunity and tolerance and activated conventional CD4+CD25low T cells without suppressive activity. Previous studies had reported the origin of Adult T-cell Leukemia/Lymphoma cells (ATLL) in Foxp3 T cells and in other lymphomas types the FOXP3 expression was only detected in the reactive T-cell background. Our objetive was to determine the presence of Treg phenotype cells by the FOXP3 expression in T-cell lymphomas. Methods: A retrospective study was performed on 48 samples collected from diverse T-cell lymphomas in our institution. A highly sensitive immunohistochemical method was used to demonstrate Treg phenotype by FOXP3 protein expression with a mouse monoclonal antibody (clone 236A/E7ABCAM) in most formalin-fixed paraffin-embedded tissue sections from lymph nodes, skin, bone marrow and extranodal sites samples as cavum and stomach. We did not co-stained with CD25 and considered a FOXP3+ tissue when positivity was > 20% of tumor cells. The statistical method was descriptive and survival was calculated using the Kaplan-Meier method. Results: Among the 48 evaluable T-cell lymphomas collected, 33 were ATLL, 8 unspecified peripheral T-cell lymphomas (U-PTCL), 6 mycosis fungoides (MF) and 1 cutaneous aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma. Among the 33 ATLL: lymphomatous=17, acute=11, smoldering=1, chronic=1, cutaneous=1 and undefined=2. FOXP3 expression in tumour cells was detected in 24% (8/33) of ATLL cases and in 37% (3/8) of U-PTCL. It was negative in MF tumour cells and aggressive epidermotropic CD8(+) cytotoxic T-cell lymphoma Among the ATLL cases FOXP3 positivity were obtained in 35% (6/17) of lymphomatous type; 18% (2/11) of acute ones and none in others ATLL types studied. Interestingly 3 U-PTCL had Treg phenotype and were related to EBV (LMP1 positive); two had extranodal primary ( parotide and cavum) and one was nodal. We failed to demonstrate any correlation between FOXP3 status and survival. Conclusions: Some ATLL and U-PTCL had Treg phenotype. In our work Foxp3 expression was not found to be a prognostic factor. No significant financial relationships to disclose.

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Detection of the human T cell lymphoma virus p19 in cells of some patients with cutaneous T cell lymphoma and leukemia using a monoclonal antibody.

Journal of Experimental Medicine ◽

10.1084/jem.154.6.1957 ◽

1981 ◽

Vol 154 (6) ◽

pp. 1957-1964 ◽

Cited By ~ 149

Author(s):

M Robert-Guroff ◽

F W Ruscetti ◽

L E Posner ◽

B J Poiesz ◽

R C Gallo

Keyword(s):

Monoclonal Antibody ◽

T Cells ◽

T Cell ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Cutaneous T Cell Lymphoma ◽

Natural Reservoir ◽

T Cell Lymphomas ◽

Human T Cell ◽

Type C

A monoclonal antibody specific for the internal p19 protein of a type-C retrovirus (HTLV) isolated from human neoplastic T cells has been developed. Its specificity has been shown by radioimmune precipitation and by affinity chromatography of iodinated HTLV proteins. By indirect immune fluorescence this antibody recognizes only HTLV-producing cells. Examination of cells from patients with cutaneous T cell lymphomas and leukemias and with other types of lymphomas and leukemias indicated that HTLV p19 expression is rare. The monoclonal antibody will be useful in determining the natural reservoir of HTLV, possibly in a subset of mature T cell neoplasias.

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Assessment of FDG-PET Imaging in T-Cell and NK-Cell Lymphomas

Blood ◽

10.1182/blood.v112.11.5305.5305 ◽

2008 ◽

Vol 112 (11) ◽

pp. 5305-5305

Author(s):

Juan Chalapud-Revelo ◽

Pedro Sobrevilla-Calvo ◽

Cadena-Eumaña Carlos ◽

Rivas-Vera Silvia ◽

Lopez-Navarro Omar ◽

...

Keyword(s):

T Cell ◽

Fdg Pet ◽

Nk Cell ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Histological Subtypes ◽

Cutaneous Lesions ◽

Nasal Type ◽

T Cell Lymphomas ◽

Nasal Region

Abstract BACKGROUND: Malignant T-cell lymphomas are a heterogeneous group of disorders that frequently involve extranodal regions. Treatment and prognosis depend on accurate staging and histological evaluation. PET is widely used in staging and response evaluation of B-cell lymphomas, however, the use of FDG-PET have not been well studied for T-cell and natural killer cell lymphomas, and there is controversy if PET can detect accurately extranodal tumor activity. PATIENTS AND METHODS: We retrospectively evaluated FDG-PET in 38 patients with active T-cells, and T/NK-cells lymphoma diagnosed according to the World Health Organization (WHO) classification, from May 2007 to May 2008. We also evaluated the Standard Uptake values (SUV) according the different subtypes and the accuracy of PET in detecting tumor activity in the skin and nasal region. RESULTS: Histological subtypes included were extranodal NK/T-cell lymphoma nasal type (ENKL, n=12); Anaplastic T-cell lymphoma (ATCL, n=11); peripheral T-cell lymphoma (PTL, n=7); mycosis fungoides and Sezary syndrome (MF/SS, n=5), angioimmunoblastic (An=3). FDG-PET detected a lymphoma lesion in at least one site in 35 out of 38 patients (91%). The positivity rate was high in all histological subtypes ENKL 92%, ATCL 100%, PTL 86%, MF/SS 80%, A 100%. Among 13 patients who had cutaneous lesions only 7 had FDG-avid skin lesions (sensitivity =53.8% and specificity=100%) and among 11 patients with nasal region lesions, 91% had FDG-avid cutaneous lesions (sensibility = 91%, specificity=100%). The SUV Value in the different subtypes is shown in the table and there was not statistical differences between the groups (p= 0.067) Lymphoma N Mean SD(+/−) Range An 3 4.1 1.7 2.7–6 MF/SS 5 4.2 3.0 1.9–8.6 ATCL 11 8.3 5.3 1.6–20.8 PTL 7 3.5 1.6 2.2–6.0 ENKL 12 7.2 4.1 2.2–13.8 Total 38 6.35 4.3 1.6–20.8 CONCLUSIONS: Most of the T and T/NK cell lymphomas were detected by FDGPET. All cases, except one, of ENKL nasal type were FDG-avid, but only half of cutaneous lesions were detected by PET. In the whole T-Cell lymphomas group the mean SUV was 6.35, ATCL had the highest SUV, and we did not find any statistically differences between the groups. We conclude that PET has a high sensitivity for detection of active ENNK nasal type lymphomas and this information should be the basis for designing further studies to assess its value in staging, tumor response and as a prognostic factor.

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2-Chlorodeoxyadenosine: an active agent in the treatment of cutaneous T- cell lymphoma

Blood ◽

10.1182/blood.v80.3.587.587 ◽

1992 ◽

Vol 80 (3) ◽

pp. 587-592 ◽

Cited By ~ 78

Author(s):

A Saven ◽

CJ Carrera ◽

DA Carson ◽

E Beutler ◽

LD Piro

Keyword(s):

T Cell ◽

Cell Lymphoma ◽

Single Agent ◽

Active Agent ◽

T Cell Lymphoma ◽

Treatment Modalities ◽

Cutaneous T Cell Lymphoma ◽

T Cell Lymphomas ◽

Duration Of Response ◽

Combination Regimens

Abstract Cutaneous T-cell lymphomas are disfiguring malignant lymphoproliferative disorders for which standard therapy has been principally palliative. 2-Chlorodeoxyadenosine (2-CdA), a new purine analogue resistant to degradation by adenosine deaminase that has substantial activity against lymphoid neoplasms, was administered to 16 patients with cutaneous involvement by T-cell lymphoma. All patients had failed topical treatment modalities and/or systemic therapies. Fifteen patients were evaluable; one patient was not evaluable due to incomplete therapy and follow-up. The overall response rate was 47%. Three of 15 patients (20%) achieved complete responses and four of 15 patients (27%) achieved partial responses. The median duration of response was 5 months. One patient remains in unmaintained complete remission at 52+ months. Therapy was well tolerated. Myelosuppression was the principal toxicity encountered, occurring in 8 of 15 (53%) patients. 2-CdA is an effective new agent for the treatment of cutaneous T-cell lymphoma and warrants further study both as a single agent and in combination regimens.

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Galectin-1 inhibits the viability, proliferation, and Th1 cytokine production of nonmalignant T cells in patients with leukemic cutaneous T-cell lymphoma

Blood ◽

10.1182/blood-2011-12-396457 ◽

2012 ◽

Vol 119 (15) ◽

pp. 3534-3538 ◽

Cited By ~ 51

Author(s):

Filiberto Cedeno-Laurent ◽

Rei Watanabe ◽

Jessica E. Teague ◽

Thomas S. Kupper ◽

Rachael A. Clark ◽

...

Keyword(s):

T Cells ◽

T Cell ◽

Cytokine Production ◽

Immune Escape ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

Cutaneous T Cell Lymphoma ◽

Tumor Immune Escape ◽

T Cell Lymphomas ◽

Malignant T Cells

Tumor-derived galectin-1 (Gal-1), a β-galactoside–binding S-type lectin, has been shown to encourage T-cell death and promote T cell–mediated tumor immune escape. In this report, we show that patients with leukemic cutaneous T-cell lymphomas, known to have limited complexity of their T-cell repertoires, have a predominant T helper type-2 (Th2) cytokine profile and significantly elevated plasma levels of Gal-1 compared with healthy controls. Circulating clonal malignant T cells were a major source of Gal-1. The conditioned supernatant of cultured malignant T cells induced a β-galactoside–dependent inhibition of normal T-cell proliferation and a Th2 skewing of cytokine production. These data implicate Gal-1 in development of the Th2 phenotype in patients with advanced-stage cutaneous T-cell lymphoma and highlight the Gal-1–Gal-1 ligand axis as a potential therapeutic target for enhancing antitumor immune responses.

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Gemcitabine Treatment in Pretreated Cutaneous T-Cell Lymphoma: Experience in 44 Patients

Journal of Clinical Oncology ◽

10.1200/jco.2000.18.13.2603 ◽

2000 ◽

Vol 18 (13) ◽

pp. 2603-2606 ◽

Cited By ~ 165

Author(s):

Pier Luigi Zinzani ◽

Gianandrea Baliva ◽

Massimo Magagnoli ◽

Maurizio Bendandi ◽

Gino Modugno ◽

...

Keyword(s):

T Cell ◽

Phase Ii ◽

Cell Lymphoma ◽

Single Agent ◽

T Cell Lymphoma ◽

Hematologic Toxicity ◽

Cutaneous T Cell Lymphoma ◽

T Cell Lymphomas ◽

Previously Treated Patients ◽

Previously Treated

PURPOSE: To evaluate the efficacy and toxicity of gemcitabine, a novel pyrimidine antimetabolite with a low-toxicity profile and activity in several solid tumors, in patients with relapsed or refractory cutaneous T-cell lymphomas. PATIENTS AND METHODS: Between May 1997 and February 1999, 44 previously treated patients with mycosis fungoides (MF; n = 30) and peripheral T-cell lymphoma unspecified (PTCLU) with exclusive skin involvement (n = 14) were enrolled onto a two-institution, phase II trial and treated with gemcitabine. This drug was given on days 1, 8, and 15 of a 28-day schedule at a dose of 1,200 mg/m2 intravenously over 30 minutes for a total of three courses. RESULTS: Of the 44 patients, five (11.5%) achieved complete responses (CRs), 26 (59%) partial responses (PRs), and the remaining 13 showed no benefit from the treatment. Two of the CRs were histologically confirmed. The CR and PR rates were the same for patients with MF and those with PTCLU, respectively. No difference in terms of overall response rate was observed between relapsed and refractory patients. The median durations of CR and PR were 15 months (range, 6 to 22 months) and 10 months (range, 2 to 15 months), respectively. Treatment was well tolerated; hematologic toxicity was mild, and no nausea/vomiting or organ toxicity was recorded. CONCLUSION: The results of the present phase II study show activity of gemcitabine as a single agent in patients with pretreated cutaneous T-cell lymphoma. Further studies that use gemcitabine alone or in combination with other drugs in earlier stages of the disease are needed.

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Current and Emerging Therapeutics for Cutaneous T-Cell Lymphoma: Histone Deacetylase Inhibitors

Lymphoma ◽

10.1155/2012/290685 ◽

2012 ◽

Vol 2012 ◽

pp. 1-10 ◽

Cited By ~ 8

Author(s):

Annabelle L. Rodd ◽

Katherine Ververis ◽

Tom C. Karagiannis

Keyword(s):

T Cell ◽

Histone Deacetylase ◽

Histone Deacetylase Inhibitors ◽

Cell Lymphoma ◽

Therapeutic Option ◽

Hdac Inhibitors ◽

Lymph Node Involvement ◽

T Cell Lymphoma ◽

Cutaneous T Cell Lymphoma ◽

T Cell Lymphomas

Cutaneous T-cell lymphoma is a term that encompasses a spectrum of non-Hodgkin’s T-cell lymphomas with primary manifestations in the skin. It describes a heterogeneous group of neoplasms that are characterised by an accumulation of malignant T cells of the CD4 phenotype that have the propensity to home and accumulate in the skin, lymph nodes, and peripheral blood. The two most common variants of cutaneous T-cell lymphoma include mycosis fungoides and the leukemic variant, the Sézary syndrome. While numerous treatments are available for cutaneous T-cell lymphoma and have shown to have success in those with patch and plaque lesions, for those patients with tumour stage or lymph node involvement there is a significant decline in response. The relatively new therapeutic option with the use of histone deacetylase inhibitors is being advanced in the hope of decreasing morbidity and mortality associated with the disease. Histone deacetylase inhibitors have been shown to induce changes in gene expression, affecting cell cycle regulation, differentiation, and apoptosis. The aim of this paper is to discuss CTCL in the context of advances in CTCL treatment, specifically with HDAC inhibitors.

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Frequency and Survival of T-Cell Lymphomas in Peru: Initial Report of a Peruvian Registry Experience

Blood ◽

10.1182/blood-2021-154330 ◽

2021 ◽

Vol 138 (Supplement 1) ◽

pp. 1374-1374

Author(s):

Daniel J Enriquez ◽

Jhoisy Casas ◽

Gustavo Sandival ◽

Johan Espino ◽

Evelyn Espinoza-Morales ◽

...

Keyword(s):

Overall Survival ◽

T Cell ◽

Latin American ◽

Cell Lymphoma ◽

T Cell Lymphoma ◽

World Health ◽

Type I ◽

Cutaneous T Cell Lymphoma ◽

Initial Report ◽

T Cell Lymphomas

Abstract Introduction: T-cell lymphomas are a relatively rare and heterogeneous group of lymphoid neoplasms. Its incidence relies on viral infections incidence as Human T-cell lymphotropic virus type I-II (HTLV-I/II) and Ebstein Bar virus (EBV). Specifically, these viruses have a significantly higher incidence in Latin-American populations. Our objective was to calculate the incidence and survival of T-cell lymphomas in the largest Peruvian population based on a national registry. Methods: We conducted a multicenter, retrospective registry study of non-Hodgkin T cell lymphoma. The data was extracted from Instituto Nacional de Enfermedades Neoplasicas and Oncosalud-AUNA, Lima-Peru, from January 2010 to December 2019, a total of 948 patients who were diagnosed as mature T cell non-Hodgkin lymphoma based on the World Health Organization Classification 2008 were enrolled. T-lymphoblastic lymphoma/leukemia was excluded. Overall survival was calculated based on death dates from the Peruvian national identification registry (RENIEC). Results: The median age was 51 years (range, 1-94), and male and female patients were 512 (54%) and 436 (46%). Among the 948 patients enrolled, Peripheral T-cell lymphoma was the common neoplasm accounting for 23% (n=221), and Extra-Nodal NK T-lymphoma (22%, n=213), Adult T-cell lymphoma (22%, n=205), Anaplasic Large cell lymphoma (14%, n=131), Cutaneous T-cell lymphoma (14%, n=129) (Figure 1a). At the time of diagnosis, extranodal disease was found in 68.6% (650) of patients. By July 2021, only 15.3% of cases were in remission and 37% (350) were alive. Median global overall survival of T-cell lymphomas was 1 year (0.8-1.1), Cutaneous T-cell lymphoma had the highest survival and Adult T-cell lymphoma had the lowest survival (Table 1 and Figure 1b). Conclusion: This initial report shows a relatively high frequency of mature T-cell lymphomas in Latin-America real-world setting, and confirms that T-cell lymphomas patients had a dismal outcome. The clinical outcome for patients with T-cell lymphomas subtypes is poor with standard therapies, and novel agents and new modalities are needed to improve survival. Figure 1 Figure 1. Disclosures No relevant conflicts of interest to declare.

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