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Cutaneous Lymphomas
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Emily Correia, Shalini Krishnasamy, Neda Nikbakht
Laboratory studies: Other categories of T-cell lymphomas must be excluded before diagnosis of primary cutaneous peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) can be made. Dermatopathology typically shows nodular or diffuse infiltrates with medium or large size pleomorphic or immunoblastic-like T-cell. Epidermotropism is typically mild or lacking. Generally, cases show an aberrant CD4+ T-cell expression. CD30 is usually mild or negative, while some cases may show CD56 co-expression.
Cutaneous Lymphoma
Published in Debjani Sahni, Adam Lerner, Bilal Fawaz, Advanced Skin Cancer, 2022
CTCL is classified into three main categories: (a) Classic CTCL (65%), which includes mycosis fungoides (MF) and Sézary syndrome (SS); (b) primary CD30+ lymphoproliferative disorders (25%), which include cutaneous anaplastic large-cell lymphoma (C-ALCL) and lymphomatoid papulosis (LyP); and (c) other CTCL (10%), which encompasses a group of often aggressive lymphomas, such as subcutaneous panniculitis-like T-cell lymphoma, extranodal NK/T-cell lymphoma nasal type, and cutaneous peripheral T-cell lymphoma, not otherwise specified.1–3 Men are almost twice as likely to develop CTCL than women, and incidence among blacks is higher than other racial groups.3 The disease presents at a median age of 55–60 years, but certain variants differ significantly in their age of onset (e.g., hypopigmented MF predominately presents in the pediatric population).3
Non-Melanoma Skin Cancer
Published in Pat Price, Karol Sikora, Treatment of Cancer, 2020
Irene De Francesco, Sean Whittaker, Stephen L. Morris
A Phase II study of gemcitabine monotherapy achieved an ORR of 70% (10% CR and 60% PR) in 30 previously treated patients with T3 or T4 disease. The median durations of CR and PR were 15 months (range: 6–22 months) and 10 months (range: 2–15 months), respectively. Treatment was well-tolerated; hematological toxicity was mild, and no nausea/vomiting or organ toxicity was recorded.248 The same group previously also reported 32 untreated patients (26 MF, 5 with peripheral T-cell lymphoma not otherwise specified [PTCLnos], 1 SS) showing a CR rate of 22% and PR rate of 53% with a median duration of response of 10 months (range: 4–22 months) with only mild toxicity.249 A U.K. multicenter trial of gemcitabine in combination with bexarotene has shown a low response rate and poor lipid control, and bexarotene did not improve the duration of response.250
The efficiency of autologous stem cell transplantation as the first-line treatment for nodal peripheral T-cell lymphoma: results of a systematic review and meta-analysis
Published in Expert Review of Hematology, 2022
Yixin Zhai, Jinhuan Wang, Yanan Jiang, Wenqi Wu, Yangyang Lv, Hong Xu, Linyan Tian, Huimeng Sun, Zhigang Zhao, Lanfang Li
Nodal PTCL, a type of non-Hodgkin lymphoma (NHL), is a group of highly heterogeneous, including peripheral T cell lymphoma, non-otherwise specified (PTCL-NOS), angioimmunoblastic T cell lymphoma (AITL), anaplastic large cell lymphoma, anaplastic lymphoma kinase positive (ALK+ALCL), and ALK−ALCL [1]. Except for ALK+ALCL, which can be targeted and an individual patient data pooled analysis of 263 patients found no benefit of upfront high-dose chemotherapy followed by autologous stem cell transplantation (HDT/ASCT) in PFS and OS [2], other types of PTCL generally have a poor prognosis, and no consensus has been reached on the first-line regimen [3,4]. CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone), CHOEP (cyclophosphamide, doxorubicin, vincristine, etoposide, and prednisone) or CHOP-like chemotherapy regimens is still the preferred treatment for most PTCL, but numbers of patients inevitably face disease progression during or shortly after treatment [5,6].
Prognostic significance of Twist, ZEB1 and Slug in peripheral T-cell lymphomas
Published in Hematology, 2020
Pyry M. Uotila, Siria A. Lemma, Kirsi-Maria Haapasaari, Katja Porvari, Sini Skarp, Ylermi Soini, Esa Jantunen, Taina Turpeenniemi-Hujanen, Outi Kuittinen
The study population consisted of 53 patients with newly diagnosed PTCL: 22 patients had angioimmunoblastic T-cell lymphoma (AITL), 9 had peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS), 7 had enteropathy associated T-cell lymphoma (EATL) and 15 had anaplastic large cell lymphoma (ALCL). The median patient age was 68 years. Diagnostic work-up included medical history and physical examination, blood chemistry, bone marrow biopsy and aspiration and whole-body computed tomography. Paraffin-embedded tissue blocks were available from all cases (53 in total). Most patients received cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) chemotherapy, or another CHOP-type regimen such as cyclophosphamide, vincristine and prednisone (COP) or CHOP plus etoposide (CHOEP). Clinical data were collected retrospectively from patient records. Full clinical data were available from 44 patients and is presented in Table 1. The study was conducted in accordance with the principles of the Declaration of Helsinki.
Advances in the treatment and prognosis of anaplastic lymphoma kinase negative anaplastic large cell lymphoma
Published in Hematology, 2019
Xiaoli Wang, Jingjing Wu, Mingzhi Zhang
The etiology and pathogenesis of ALK- ALCL is uncertain. ALK+ ALCL has 5 morphologic forms: common, lymphohistiocytic, small cell, Hodgkin-like, and composite [5]. The first form has 70% occurrence with characteristic embryoid, rosette-like and R-S-like giant cells commonly observed. The second form has 10% occurrence and is made up of reactive histiocytes. The third form has about 5% to 10% occurrence and is made up of small-to-medium sized cells. This pattern can be misdiagnosed as peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS). The fourth form has 3% occurrence, consists of tumor nodules surrounded by fibrous bands and this form can be misdiagnosed as Hodgkin Lymphoma (HL), nodular-sclerosis. The morphology of ALK- ALCL is similar to ALK+ ALCL with the absence of small cell pattern is absent. In ALK- ALCL, CD30 is expressed strongly in all tumor cells, usually in the cell membrane and the Golgi region. A majority of ALK- ALCL tumor cells are positive for CD3 and CD2 [6]. A substantial minority of cases are positive for EMA (epithelial membrane antigen) and granzyme B. ALK-ALCL is negative for CD15 and PAX5 [7].