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Cutaneous T cell Lymphoma

Alejandro A Gru, Patrick J Voorhess
Anaplastic large-cell lymphoma (ALCL) was first described in 1985 by Stein et al and is a clinically, morphologically, and immunophenotypically heterogeneous neoplasm characterized by ALK expression, rearrangement of the ALK gene, and most characteristically its occurrence in children. Clinically, cutaneous ALK+ ALCL can be divided into primary (cutaneous forms) and the much more common, secondary dissemination by a systemic lymphoma. Systemic ALK+ ALCL represents 10%-15% of childhood non-Hodgkin lymphoma and generally presents with advanced systemic disease...
November 27, 2017: American Journal of Dermatopathology
Anne Steininger, Grit Ebert, Benjamin V Becker, Chalid Assaf, Markus Möbs, Christian A Schmidt, Piotr Grabarczyk, Lars R Jensen, Grzegorz K Przybylski, Matthias Port, Andreas W Kuss, Reinhard Ullmann
In classical models of tumorigenesis, the accumulation of tumor promoting chromosomal aberrations is described as a gradual process. Next-generation sequencing-based methods have recently revealed complex patterns of chromosomal aberrations, which are beyond explanation by these classical models of karyotypic evolution of tumor genomes. Thus, the term chromothripsis has been introduced to describe a phenomenon, where temporarily and spatially confined genomic instability results in dramatic chromosomal rearrangements limited to segments of one or a few chromosomes...
2018: Frontiers in Oncology
Christiane Querfeld, Samantha Leung, Patricia L Myskowski, Shane A Curran, Debra A Goldman, Glenn Heller, Xiwei Wu, Sung Hee Kil, Sneh Sharma, Kathleen J Finn, Steven Horwitz, Alison Moskowitz, Babak Mehrara, Steven T Rosen, Allan C Halpern, James W Young
Cutaneous T-cell lymphoma (CTCL) develops from clonally expanded CD4+ T cells in a background of chronic inflammation. Although dendritic cells (DCs) stimulate T cells and are present in skin, cutaneous T cells in CTCL do not respond with effective antitumor immunity. We evaluated primary T-cell and DC émigrés from epidermal and dermal explant cultures of skin biopsies from CTCL patients (n = 37) and healthy donors (n = 5). Compared with healthy skin, CD4+ CTCL populations contained more T cells expressing PD-1, CTLA-4, and LAG-3...
June 12, 2018: Cancer Immunology Research
Melissa Pulitzer, Shamir Geller, Erica Kumar, Denise Frosina, Allison Moskowitz, Steven Horwitz, Patricia Myskowski, Meenal Kheterpal, Alexander Chan, Ahmet Dogan, Achim Jungbluth
AIMS: The diagnosis of cutaneous gamma delta T-cell lymphoma (GDTCL) requires the identification of γδ chains of the T-cell receptor (TCR). Using a new mAb to TCRδ, we evaluated TCRδ expression in formalin fixed paraffin embedded (FFPE) skin tissue from TCRγ-positive cutaneous T-cell lymphoma (CTCL) and assessed TCRδ expression within a spectrum of other cutaneous lymphoproliferative disorders (CLPD). METHODS AND RESULTS: 12 cases (10 patients) with TCRγ-positive CTCL and 132 additional CLPD cases (127 patients) were examined including mycosis fungoides (MF, n=60), cutaneous GDTCL (n=15), subcutaneous panniculitis-like T-cell lymphomas (SPTCL, n=11), and CD30+ lymphoproliferative disorders (CD30+LPDs, n=24)...
June 12, 2018: Histopathology
Yixuan James Zheng, Albert Lee, Laura Pincus, Wilson Ho, Marin Vujic, Susana Ortiz-Urda
No abstract text is available yet for this article.
July 2018: JAAD Case Reports
Hossein Mortazavi, Leila Izadi Firouzabadi, Alireza Ghanadan, Hassan Seirafi, Seyed Naser Emadi, Safoura Shakoei
Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma. Persistent antigenic stimulation has been claimed to play a role in the development of this malignancy. We aimed to show the role of sulfur mustard in the pathogenesis of MF. A 45-year-old man with MF is introduced herein. He was a victim of chemical exposure in 1987 during the Iran-Iraq war. He developed skin lesions 3 years after exposure to sulfur mustard gas at the age of 21. Seven years after his exposure to sulfur mustard gas, a biopsy from the posterior distal part of his calf, which was injured and had bulla, revealed MF...
May 2018: Iranian Journal of Medical Sciences
F Liu, M Li, L Y Zhang, L Guo, W W Hu, H L Rao
Objective: To study clinicopathologic features, prognosis and differential diagnoses of primary mucosal CD30-positive T-cell lymphoproliferative disorders of the head and neck(mCD30(+) TLPD-head and neck). Methods: Three cases of mCD30(+) TLPD-head and neck were collected from January 2014 to April 2017 at Sun Yat-Sen University Foshan Hospital. A literature review of mCD30(+) TLPD of head and neck was provided. Results: All three cases presented with either bulging/exophytic nodule or mucosal ulcer/erosion...
June 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
Fang Yu, Ke Sun, Zhaoming Wang
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare disease with an aggressive clinical course resulting in median survival times of 12 to 14 months.1 It represents approximately 0.8% of primary cutaneous lymphomas.2 According to the World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissue, published in 2008, BPDCN is defined as an acute myeloid leukemia related precursor neoplasm, derived from precursors of the plasmacytoid dendritic cell. It is characterized microscopically by its dense monomorphous infiltrates of medium-sized blastoid morphology and expression of CD4 and CD56, as well as the absence of any common myeloid, T-lymphoid, B-lymphoid, and natural killer-lymphoid lineage markers...
May 3, 2018: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
D Nashan, C M Friedrich, E Geissler, A Schmitt-Graeff, F Klein, F Meiss
BACKGROUND: In addition to a broad and clinically diverse spectrum of known primary cutaneous lymphomas, for which an incidence of 1-3:100,000 is postulated, each year further entities are specified and defined. The goal is the presentation of a case series from daily clinical routine. METHODS: Over a period of 6 years and 2 months, patients consulting the Department of Dermatology, Medical Center University of Freiburg, were registered. Subsequently, collectives of mycosis fungoides (MF), Sezary syndrome (SS), CD30+ lymphoproliferative diseases, single cases with rare primary cutaneous lymphomas, and subcollectives of B‑cell lymphomas were examined...
June 7, 2018: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
Dylan E Lee, M Estela Martinez-Escala, Linda M Serrano, Xiaolong A Zhou, Jason B Kaplan, Barbara Pro, Jaehyuk Choi, Joan Guitart
Importance: Hemophagocytic lymphohistiocytosis (HLH) has been reported as a serious complication of cutaneous T-cell lymphoma (CTCL). Despite available diagnostic guidelines, it remains a diagnostic and therapeutic challenge in this patient population. Objectives: To examine the characteristics of CTCL associated with HLH and analyze the presenting signs and symptoms, therapeutic options, and outcome. Design, Setting, and Participants: In this case series, patients diagnosed with CTCL and HLH who were treated at a single institution from January 1, 2014, through December 31, 2017, were studied...
June 6, 2018: JAMA Dermatology
Rudolf Stadler, René Stranzenbach
Primary cutaneous T-cell lymphoma (CTCL) comprises the second most common group of extra-nodal non-Hodgkin lymphoma. They represent incurable primary extra-nodal lymphomas of major T-cells, uniformly present in the skin with 1-2% risk of systemic dissemination in mycosis fungoides (MF) which represents the most common subtype of CTCL. In general, long-term antigen stimulation is thought, through key cytokine signalling pathways, to induce an inflammatory response with T-cell proliferation, leading to a clonal malignant T-cell with continuous expansion...
June 4, 2018: Experimental Dermatology
S Geller, M Pulitzer, P L Myskowski
Follicular mucinosis (FM) can present as an acneiform eruption, and is usually a benign variant of primary FM unrelated to cutaneous T-cell lymphoma (CTCL). We report two cases of women in their twenties who presented with an acneiform rash on the face, arms and back. In both cases, pathological evaluation of the facial papules revealed predominantly mucinous degeneration of the follicular epithelium, with insufficient lymphocytic infiltration or atypia to diagnose mycosis fungoides. These cases are similar to previous reports of acneiform FM...
May 30, 2018: Clinical and Experimental Dermatology
Vanessa Szablewski, Valérie Costes-Martineau, Céline René, Aurélie Croci-Torti, Jean-Marie Joujoux
Composite lymphoma (CL) is a rare disease defined by the occurrence of 2 distinct lymphomas within a single tissue at the same time. We present the case of an 89-year-old male with a clinical history of immunoglobulin M monoclonal gammapathy of undetermined significance (IgM MGUS). The patient presented cutaneous eruption of nodules on the right bottom and arm. An excisional biopsy revealed cutaneous infiltration composed of 2 components. The first one consisted of large B-cell with CD20+/MUM1+/BCL2+ phenotype whereas the second one involved the subcutaneous fat in a panniculitis manner, was CD3+/CD8+/GranzymeB+/TCRβF1+...
May 30, 2018: Journal of Cutaneous Pathology
Natalia Buchely, Rami Al-Rohil, Phyu P Aung, George Jour, Carlos Torres-Cabala, Victor G Prieto, Doina Ivan
Mycosis fungoides (MF) is the most common variant of cutaneous T-cell lymphomas (CTCL). Large cell transformation of MF has been associated with disease progression and overall poor outcome. The expression of CD30, which defines anaplastic large cell lymphoma (ALCL) and lymphomatoid papulosis (LyP), might also occur in a subset of patients with MF, with or without large cell transformation. Brentuximab vedotin is an anti-CD30 monoclonal antibody which has been proven to be a safe and effective therapeutic agent in the treatment of CD30-positive lymphomas, such as Hodgkin lymphoma and ALCL...
May 27, 2018: Journal of Cutaneous Pathology
Chris Maminakis, Arin C Whitman, Nahida Islam
We present a case of a patient with cutaneous T-cell lymphoma started on bexarotene 300 mg/m2 due to progressing disease. The patient experienced good clinical response, but unfortunately, she developed rapid and profound hypertriglyceridemia. Although hypertriglyceridemia occurs in high incidence with bexarotene therapy, management recommendations are scarce. Due to the rise in triglycerides, atorvastatin 10 mg daily was initiated in combination with fenofibrate 120 mg daily. Triglycerides continued to increase, so the patient was instructed to take atorvastatin 40 mg, fenofibrate 120 mg, and to hold bexarotene for 2 weeks...
January 2018: Case Reports in Oncology
Lei Qiu, Fengjie Liu, Shengguo Yi, Xueying Li, Xiaoqing Liu, Cheng Xiao, Christine Guo Lian, Ping Tu, Yang Wang
DNA hydroxymethylation at the 5 position of cytosine (5-hmC) is a product of the ten-eleven translocation (TET) family of DNA hydroxylases. Accumulating evidence shows that loss of 5-hmC is critical for various biological and pathological processes. However, its level in cutaneous T cell lymphoma remains largely unknown. Here we report that the loss of 5-hmC is an epigenetic hallmark of cutaneous T cell lymphoma (CTCL), with diagnostic and prognostic implications. Immunohistochemistry staining on 90 mycosis fungoides (MF) cases demonstrated a significant decrease of 5-hmC staining in CD4+ T cells in patch and tumor stages, especially in MF-LCT, compared to benign inflammatory dermatoses...
May 24, 2018: Journal of Investigative Dermatology
Marcos Silva-Feistner, María Jesús Rojas-Lechuga, Elena Ortiz, Montserrat Molgó
INTRODUCTION: Extranodal natural killer/T-cell lymphoma (NK/T), nasal type, is an infrequent neoplasm with a high lethality, characterized by bone destruction around the sinus, nasal septum or obstruction of the airway. Also, may be primary skin involvement, airway and other organs. Objecti ve: Submit a rare condition in the pediatric population, in order to facilitate the diagnostic suspicion and quick recognition from specialists. CASE REPORT: a 14-year-old girl, who presented arm and leg lesions, painless, suggestive of subcutaneous panniculitis, which evolve to ulcerated purple maculae...
April 2018: Revista Chilena de Pediatría
Zhengbang Dong, Xiaomei Zhu, Yang Li, Lu Gan, Hao Chen, Wei Zhang, Jianfang Sun
Patients with mycosis fungoides (MF) developing tumors or extracutaneous lesions usually have a poor prognosis with no cure has so far been available. To identify potential novel biomarkers for MF at the tumor stage, a genomic mapping of 41 cutaneous lymphoma biopsies was used to explore for significant genes.The gene expression profiling datasets of MF were obtained from Gene Expression Omnibus database (GEO). Gene modules were simulated using Weighted Gene Co-expression Network Analysis (WGCNA) and the top soft-connected genes (hub genes) were filtrated with a threshold (0...
May 2018: Medicine (Baltimore)
Sreejata Raychaudhuri, Maral Rahvar, Jaroslaw Jedrych, Arivarasan Karunamurthy, Oleg Kruglov, Susan Rakfal, Kevin Kane, Oleg E Akilov
Cutaneous peripheral T-cell lymphoma, not otherwise specified represents a "waste basket" of all cases that cannot be put into another of the categories of mature cutaneous T-cell lymphoma. Previously, the sudden multifocal development of cutaneous CD4 tumors without preceding a patch or plaque stage was classified as d'emblée form of mycosis fungoides (MF). Currently, the term "MF" reserved only for the classic Alibert-Bazin type characterized by the evolution of patches, plaques, and tumors or for variants showing a similar clinical course...
May 14, 2018: American Journal of Dermatopathology
J H Chen, T T Wang, J Sun, X Y Lyu
No abstract text is available yet for this article.
May 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
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