Primary Cutaneous Peripheral T-cell Lymphoma Research Articles

Çocuklarda Primer Kutanoz T- Hücreli Lenfoma: İki Olgu Sunumu

Primary cutaneous lymphomas in children are rare and constitute a heterogeneous group in regard to histological subtype, clinical course and prognosis. Here, we present the clinical characteristics and outcomes of two children with primary cutaneous lymphoma with different histopathologic subtypes. They had been diagnosed as a skin abscess initially but had not responded to systemic antibiotherapy and dressing. After a biopsy and metastatic work up, one patient was diagnosed as primary cutaneous peripheral T-cell lymphoma, unspecified, and the other as primary cutaneous CD30 positive ALK negative anaplastic large cell lymphoma.

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

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.

CD8陽性末梢性T細胞リンパ腫と鑑別を要した皮膚原発節外性NK/T細胞リンパ腫，鼻型の1例

62歳，男性。初診の6ヵ月前から躯幹・四肢に環状の紅斑を生じた。皮膚生検でCD8陽性異型リンパ球が血管周囲および表皮内へ密に浸潤する像を認め，CD8陽性皮膚T細胞性リンパ腫が疑われ当科へ紹介された。初診時，全身に多発する環状の紅斑と紅色腫瘤を認めた。末梢血に異型細胞はなく，PET－CTを含む画像検査では，皮膚に多発する腫瘤への集積像を認めたが，他に異常集積は認めなかった。受診後より皮疹が急速に増悪し，末梢性T細胞リンパ腫，非特定としてCHOP療法を開始した。その後，追加の精査にて浸潤細胞はCD56陽性，EBER－ISH陽性であり，Southern blot hybridization法でTCR遺伝子再構成検査も陰性であることが判明し，皮膚原発節外性NK/T細胞リンパ腫と診断した。CHOP療法は無効でSMILE療法に変更し，2コースで完全寛解を得た。CD8陽性末梢性T細胞リンパ腫との鑑別が重要と考え報告した。

The Prognostic Value of the Neutrophil to Lymphocyte Ratio in 209 Patients with Peripheral T-Cell Lymphoma

Introduction: Peripheral T-cell lymphomas (PTCL) are rare hematologic malignancies with a poor prognosis when treated with current standard therapies. Several factors have been developed to prognosticate survival in PTCL patients; however, more refined, easy to use and reliable prognostic tools are needed. The neutrophil to lymphocyte ratio (NLR) has been reported prognostic in patients with diffuse large B-cell lymphoma (Troppan et al. BJC 2014). We have investigated the prognostic value of the NLR in the overall survival (OS) of patients with untreated PTCL.Methods: We included patients with a pathological diagnosis of PTCL who were diagnosed and treated at our institution between 2001-2013. We excluded cases with primary cutaneous PTCL, CNS involvement, and patients with >50% incomplete data. IRB approval was obtained prior to research. Pathological samples were reviewed by expert hematopathologists to confirm a diagnosis of PTCL. Pertinent clinicopathological data such as age, sex, performance status, LDH levels, stage, bone marrow involvement, extranodal sites of disease, PTCL subtype, and absolute neutrophil and lymphocyte countswere collected through chart review, and are presented using descriptive statistics. The NLR was calculated by dividing the absolute neutrophil by the lymphocyte count, and dichotomized in NLR>=4 and NLR<4. The Prognostic Index for PTCL (PIT) was estimated using the clinical data above. Survival curves were estimated using the Kaplan-Meier method and compared using the log-rank test. Multivariate analyses were performed using Cox proportional-hazard regression models.Results: A total of 209 patients were included in our analysis from which 93 (44.5%) were PTCL, not otherwise specified (NOS), 83 (40%) were adult T-cell leukemia/lymphoma (ATLL), 22 (10.5%) were anaplastic large cell lymphoma (ALCL), 7 (3%) were extranodal NK/T-cell lymphoma (NKTCL), and 4 (2%) angioimmunoblastic T-cell lymphoma (AITL). The median age was 57 years (range 3-87 years) with equal distribution among men (52%) and women (48%). Poor performance status (ECOG >1) was seen in 85 (49%), elevated LDH levels in 116 (64%), >1 extranodal site in 31 (19%), bone marrow involvement in 64 (31%), and advanced stage (stage 3 and 4) in 151 (74%) of patients. Based on the NLR, 91 patients (59%) had NLR<4 and 62 (n=41%) had NLR>=4. Patients with NLR >=4 were more likely men (67% vs. 44%, p=0.005), and tended to present with elevated LDH (73% vs. 47%, p=0.002), advanced stage (87% vs. 61%, p=0.001) but lower bone marrow involvement (19% vs. 37%, p=0.02). There were no differences in age, performance status and number of extranodal sites. NLR>=4 was associated with a worse OS (HR 2.27, 95% CI 1.40-3.69; p=0.001). Specifically, NLR>=4 was associated with a worse OS in patients with non-ATLL PTCL (HR 2.99, 95% CI 1.67-5.37; p<0.001) but not in patients with ATLL (HR 1.16, 0.48-2.81; p=0.75). In the multivariate analysis, NLR>=4 was independently associated with worse OS when adjusting for the PIT score in non-ATLL PTCL patients (HR 2.12, 95% CI 1.06-4.26; p=0.03).Conclusion: The NLR appears as a novel and easy to use prognostic factor for OS in patients with untreated non-ATLL PTCL, independent of the PIT score. The NLR did not seem to be prognostic in ATLL patients. Our findings support the need for validation of the NLR in larger retrospective or prospective studies in patients with PTCL. DisclosuresNo relevant conflicts of interest to declare.

Relative frequency and survival of primary cutaneous lymphomas: a retrospective analysis of 98 patients

Background The World Health Organization and European Organization for Research and Treatment of Cancer (WHOEORTC) classification in 2005 promoted the comparisons of primary cutaneous lymphoma (PCL) subtypes between different countries. The relative frequency of PCL varied according to geography. The study aimed to analyze the relative frequency and survival of PCLs in China and to compare the data with the published results from other countries. Methods We analyzed 98 patients with PCLs over a 6-year period and reclassified them according to the most recent WHO-EORTC classification (2005). Disease-specific survival rate and curves according to specific subtypes such as mycosis fungoides, lymphomatoid papulosis, and primary cutaneous peripheral T-cell lymphoma, unspecified was also calculated. Results The relative rate of PCL in China was distinct from those in Western countries. Our study showed a higher frequency of cutaneous T- and NK-cell lymphomas (CTCLs) (94%), and a lower frequency of cutaneous B-cell lymphomas (CBCLs) (6%). The 5-year survival rate of the total PCLs was 82%. There was no significant difference in the 5-year survival rate (P &gt;0.05 by Log-rank test) between CTCL (80%) and CBCL (100%). Conclusions The higher percentage of CTCL in China may provide a clue to further study the etiological factors of PCLs. Racial variations in factors such as HLA determinants may play a role in the development of CTCL.

Immunophenotypic shift of CD4 and CD8 antigen expression in primary cutaneous T‐cell lymphomas: a clinicopathologic study of three cases

Primary cutaneous T-cell lymphomas (CTCL) comprise a heterogeneous group of neoplasms with diverse clinical behavior. Mycosis fungoides (MF) is the most common type of CTCL. Immunophenotypical shift during progression of the disease is a rare event and its significance is unknown. We present three primary CTCL cases that showed an immunophenotypical shift and poor prognosis. Conventional hematoxylin/eosin and immunohistochemical-stained sections were examined in all the cases. Molecular analysis for rearrangement of the T-cell receptor (TCR) gene was performed in two cases. One case was classified as MF, while the other two lacked epidermotropism, and were considered primary cutaneous peripheral T-cell lymphoma (PTCL), NOS. Two cases were CD3+/CD4+ and one case was CD3+/CD8+ at diagnosis. The first two patients suffered many relapses and eventually, new CTCL lesions with a CD3+/CD8+ phenotype were observed. Both cases revealed identical clonal TCR rearrangements on the initial and late lesions, supporting the interpretation of a single clonal proliferation with different phenotypes. The third case progressed with skin recurrences and pulmonary lesions with a predominant CD3+/CD4+/CD8- phenotype. All cases manifested poor prognosis and two patients died of lymphoma. Immunophenotypical shift between CD4 and CD8 in CTCL seems to be a rare phenomenon that may be associated with disease progression.

CD8+ primary cutaneous peripheral T-cell lymphoma in an 18-year-old woman

The Journal of DermatologyVolume 40, Issue 7 p. 571-572 Letter to the Editor CD8+ primary cutaneous peripheral T-cell lymphoma in an 18-year-old woman Fumi Miyagawa, Corresponding Author Fumi Miyagawa Department of Dermatology, Nara Medical University School of Medicine, Nara, JapanCorrespondence: Fumi Miyagawa, M.D., Ph.D., Department of Dermatology, Nara Medical University School of Medicine, 840 Shijo, Kashihara, Nara 634-8522, Japan. Email: [email protected]Search for more papers by this authorTakaya Fukumoto, Takaya Fukumoto Department of Dermatology, Nara Medical University School of Medicine, Nara, JapanSearch for more papers by this authorSatoshi Yurugi, Satoshi Yurugi Department of Dermatology, Nara Medical University School of Medicine, Nara, JapanSearch for more papers by this authorHirokazu Nakamine, Hirokazu Nakamine Department of Pathology and Immunology Division, Kansai University for Health Sciences, Osaka, JapanSearch for more papers by this authorHideo Asada, Hideo Asada Department of Dermatology, Nara Medical University School of Medicine, Nara, JapanSearch for more papers by this author Fumi Miyagawa, Corresponding Author Fumi Miyagawa Department of Dermatology, Nara Medical University School of Medicine, Nara, JapanCorrespondence: Fumi Miyagawa, M.D., Ph.D., Department of Dermatology, Nara Medical University School of Medicine, 840 Shijo, Kashihara, Nara 634-8522, Japan. Email: [email protected]Search for more papers by this authorTakaya Fukumoto, Takaya Fukumoto Department of Dermatology, Nara Medical University School of Medicine, Nara, JapanSearch for more papers by this authorSatoshi Yurugi, Satoshi Yurugi Department of Dermatology, Nara Medical University School of Medicine, Nara, JapanSearch for more papers by this authorHirokazu Nakamine, Hirokazu Nakamine Department of Pathology and Immunology Division, Kansai University for Health Sciences, Osaka, JapanSearch for more papers by this authorHideo Asada, Hideo Asada Department of Dermatology, Nara Medical University School of Medicine, Nara, JapanSearch for more papers by this author First published: 17 April 2013 https://doi.org/10.1111/1346-8138.12151Citations: 1Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article.Citing Literature Volume40, Issue7July 2013Pages 571-572 RelatedInformation

Non–Mycosis Fungoides Cutaneous T-Cell Lymphomas

Primary cutaneous T-cell lymphomas (CTCL) excluding mycosis fungoides (MF) were discussed in 2 sessions of the 2011 Society for Hematopathology/ European Association of Haematopathology Workshop, Los Angeles, CA. Session 2 focused on primary cutaneous CD30+ T-cell lymphoproliferative disorders and their differential diagnosis, including systemic CD30+ T-cell lymphoma secondarily infiltrating the skin. Interesting features like special morphologic variants and atypical phenotypes were presented. In addition, the possibility of rare ALK+ primary cutaneous lymphomas was discussed. Session 3 examined other more uncommon non-MF CTCLs, including subcutaneous panniculitis-like T-cell lymphoma, extranodal NK/T-cell lymphoma, hydroa vacciniforme-like T-cell lymphoma, and rare subtypes of primary cutaneous peripheral T-cell lymphoma, not otherwise specified. In addition, systemic T-cell lymphomas involving the skin secondarily, such as angioimmunoblastic T-cell lymphoma, were included in this session. In this report, novel findings, areas of special interest, and diagnostic challenges emerging from the cases submitted to the workshop will be highlighted. The necessity to integrate histologic, immunophenotypical, genetic, and in particular, clinical data to arrive at the correct diagnosis, and subsequently provide adequate treatment, is emphasized.

Management of the Cutaneous Peripheral T-Cell Lymphomas

The cutaneous T-cell lymphomas are a heterogeneous collection of many biologically and clinically distinct cancers. The World Health Organization 2008 reclassification of cutaneous lymphomas delineated provisional entities that were previously classified simply as primary cutaneous peripheral T-cell lymphoma not otherwise specified. Because of the relative rarity of cases, standard treatment recommendations and algorithms are lacking. However, identification of these subtypes is important as they can have vastly different clinical outcomes. Furthermore, cytokine treatments effective in classic mycosis fungoides or Sezary syndrome not only are ineffective, but also may exacerbate certain subtypes. The aim of this review was to summarize the biological bases of the recently defined entities, reinforce the importance of acute clinical/pathologic diagnosis, and propose treatment strategies based on the available data.

Pilot Study of Denileukin Diftitox Alternate Dosing Regimen in Patients With Cutaneous Peripheral T-Cell Lymphomas

Better treatment and survival outcomes are needed for the rare primary cutaneous peripheral T-cell lymphomas.Five (62.5%) of 8 patients with peripheral T-cell lymphomas enrolled in a pilot study of denileukin diftitoxat 18 μg/kg per day for 5 days followed by once weekly for 24 weeks responded, including 2 complete responses, one of which is ongoing at 8 years. To evaluate the safety and efficacy of an alternate dosing regimen in rare primary cutaneous peripheral T-cell lymphoma variants. This is a prospective, single center, pilot study of denileukin diftitox (Dd) in patients with persistent or recurrent cutaneous peripheral T-cell lymphomas and mycosis fungoides (MF) variants, excluding Sézary syndrome (SS). Dd was administered at 18 μg/kg per day for 5 days and once weekly for 24 weeks, with response by modified skin weighed assessment tool. Eight patients, with a median age of 76 years (range, 44-88 years), were treated between December 2003 and July 2008. Five (62.5%) of 8 patients responded, including 3 patients with CD30(+) anaplastic large-cell lymphoma (ALCL) with 2 complete responses, one ongoing at 8 years. One patient with CD8(+) and 1 patient with natural killer T cell lymphoma (NK-T) had partial responses. Progressive disease occurred in 1 patient positive for human T-cell lymphotropic virus and 1 patient with ALCL. Vascular leak syndrome (VLS) occurred in 6 (75%) of 8 patients during or just after cycle 1. Three were grade 3, and 2 of these resulted in study withdrawal. Other adverse effects included nausea or vomiting (n = 3), fatigue (n = 1), back pain (n = 1), transaminase elevations (n = 3), and elevated creatinine (n = 1). Dd with an alternate dosing schedule was active in this small study of primary cutaneous T-cell lymphomas.

The Programmed Death-1 Protein Is Often Detected in Cutaneous CD4-Positive Small/Medium-Sized Pleomorphic T-cell Lymphoma

Primary cutaneous peripheral T-cell lymphomas (PTCL) include the provisional entity cutaneous CD4-positive small/medium-sized pleomorphic T-cell

Clinicopathological Characteristics and Clinical Course of CD8 Expressing Primary Cutaneous Peripheral T-Cell Lymphomas (CTCL) - Retrospective Case Study

Abstract 5213 Background:CD8-positive primary cutaneous T cell lymphomas (CTCL) are rare disorders and mainly include primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (AECTL) and CD8+ variant mycosis fungoides (MF). In contrast to primary cutaneous CD8+ AECTL, which frequently exhibits strikingly aggressive and unfavorable clinical behavior, CD8+ MF shows debatable clinical course, from an indolent to aggressive behavior. As previously reported, the indolent subtype CD8+ MF occur more frequently in pediatric group, while both clinical subtypes have been observed in adults. Albeit single case studies or small case series have been reported in the literature, it still lacks a large scale of study to enlighten the clinicopathological aspects of CD8+ primary CTCLs, in order to develop the appropriate therapeutic strategies. This study aims to retrospectively review these two entities to demonstrate their clinicopathologic characteristics and to correlate them with the clinical outcome. Design:The hematopathology files from H. Lee Moffitt Cancer Center & Research Institute (PATHNET) and Tianjian Cancer Research Institute were retrieved. The patients with a primary diagnosis of CD8 expressing primary CTCLs, diagnosed and treated between January 2004 and June 2011, were included. Cutaneous involvement by systemic peripheral T-cell lymphoma, primary cutanous gamma delta T-cell lymphoma, and subcutaneous panniculitis-like T-cell lymphoma were excluded. The corresponding patient demographics, laboratory datas, therapeutic strategies and the clinical outcomes were reviewed. All available histology slides, along with all of the ancillary study results were reviewed and correlated with the clinical outcome. Results:Total of 10 cases were included based on the confirmed histomorphological diagnosis. Cases were divided into two groups: 1) CD8+ MF (n=5) and 2) CD8+ non-MF (n=5) including 2 cases with definitive diagnosis of AECTL and 3 cases diagnosed as CD8-positive primary cutaneous T cell lymphoma, not further classifiable. Clinicopathological characteristics including patients' demographic data, diagnosis, site of involvement, treatment, duration of follow up and clinical outcomes are summarized in table 1. The overall survival time for CD8+CTCLs, non-MF type (excluding 1 patient with lost follow up) varied from 5 to 90 months (averaging 20.5 months) while it was shorter in CD8+ MF, 12.6 months (5 to 23 months). Of note, 1 patient with AECTL expired shortly after diagnosis, within 3 months, however; the other one received allogeneic hematopoietic stem cell transplant (allo-HSCT) and has been alive up to date. Conclusion:CD8-positive CTCLs remain a diagnostic challenge. CD8+ MF in adults exhibit dual growth patterns: localized or systemically disseminated disease. The latter could have a very short median overall survival regardless of the aggressive therapies. Allo-HSCT might be beneficial to those with AECTL. Larger series of CD8+ MF should be investigated for molecular gene profiling in order to establish genetic, molecular and phenotypic parameters not only to separate the indolent form from the aggressive subtype, but also to distinguish it from primary cutaneous CD8-positive AECTL.AgeGenderDxStageSites of InvolvementPhenotypeTreatmentFollow up (mon)Outcome153MCD8+ CTCL, not classifiableIRight frontoparietalCD3, CD5, CD8, CD43, TIA-1CHOP x619CR283MCD8+ CTCL, not classifiableIArms and trunkwk CD3, CD7, CD8, CD56, wk TIA,CHOP5AWD371MCD8+ CTCL, not classifiableIIBLeft eyelidCD3, CD8, TIAUV, CVP x 650CR425MAECTLIIIBRight toe, groin LNCD3, CD8, partial CD30RTX, ARA-C, cortisone, allo-HSCT8CR519FAECTLIVSkin, bone and lungCD3, CD5, CD8, TIACHOP X13LFU657MMF, CD8+IIBForeheadCD3, CD8, TIA-1UV, nitrogene mustard18AWD761FMF, CD8+IBLower ext and then diffuseCD3, CD4, CD5, CD8 and weak CD7.UV + Tragretin23CR859MMF, CD8+IBTrunkCD3, CD8, partial CD30NA5LFU919MMF, CD8+IVLeft palate and lungCD45RO, CD8CHOP X4 AND ESHAP X17DOD1032MMF, CD8+IVSkin, cervical LN, BMCD3, CD5, CD8CHOP X 2, HYPERCVAD X 2, VDCLP X 210DODNote: LUF: Lost follow up, AWD: alive with disease, DOD: died of disease, CR: complete remission, LN: lymph node and BM: bone marrow. Disclosures:No relevant conflicts of interest to declare.

XV. Primary cutaneous lymphomas

Primary cutaneous lymphomas (PCLs) are defined as non-Hodgkin lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. After the gastrointestinal lymphomas, PCL are the second most common group of extranodal non-Hodgkin lymphomas with an estimated annual incidence of 1/100 000. These PCLs must be distinguished from nodal or systemic malignant lymphomas involving the skin secondarily, which often have another clinical behavior, have a different prognosis and require a different therapeutic approach. In recent lymphoma classifications (WHO-EORTC; WHO 2008) the different types of primary cutaneous T-cell lymphoma (CTCL) and primary cutaneous B-cell lymphoma (CBCL) are therefore included as separate entities (see Table 1) [1, 2]. In the western world, CTCL constitutes 75%–80% of all PCLs and CBCL 20%–25%, but different distributions have been observed in other parts of the world. Within the group of CTCLs roughly three categories can be distinguished: (i) the group of classical CTCLs, including mycosis fungoides (MF), variants or subtypes of MF and Sezary syndrome (SS); (ii) the group of primary cutaneous CD30-positive lymphoproliferative disorders (CD30+ LPD); and (iii) a group of rare often aggressive cutaneous T/NK-cell lymphomas, including subcutaneous panniculitis-like T-cell lymphoma (SPTCL), extranodal NK/T-cell lymphoma, nasal type, and primary cutaneous peripheral T-cell lymphoma, not otherwise specified (PTCLNOS). Well-defined types of CBCL include primary cutaneous marginal zone lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL) and primary cutaneous diffuse large B-cell lymphoma, leg type (PCLBCL, LT). Herein, characteristic features of the main types of CTCL and CBCL are summarized and recent observations regarding diagnosis, prognostic factors and treatment are described.

Primär kutanes peripheres T-Zell-Lymphom (nicht anderweitig spezifiziert)

Peripheral cutaneous T-cell lymphoma which cannot be further classified due to high morphological and molecular variability are included into the group of "primary cutaneous peripheral T-cell lymphoma--not otherwise specified" (PCTL-NOS). PCTL-NOS represent a rare, heterogeneous group characterized by rapidly progressive nodules in the absence of typical mycosis fungoides plaques. Furthermore, therapeutic options are limited and prognosis is rather poor. We report on a 62-year-old patient presenting with asymptomatic papules in the popliteal and antecubital fossae. Based on histopathological criteria, PCTL-NOS was diagnosed. Based on recent gene expression studies, a positive reaction to tyrosine kinase and histone deacetylase inhibitors is hoped for.

Primary cutaneous peripheral T-cell lymphoma in a patient with X-linked agammaglobulinaemia

Primary cutaneous peripheral T-cell lymphoma in a patient with X-linked agammaglobulinaemia

Primary cutaneous peripheral T-cell lymphoma: Unspecified type

Primary cutaneous peripheral T-cell lymphoma: Unspecified type

Frequency of deletion of CDKN2A (9p21) gene in T-cell lymphomas in FISH analysis

ENWEndNote BIBJabRef, Mendeley RISPapers, Reference Manager, RefWorks, Zotero AMA Pastwińska A, Rygier J, Woroniecka R, et al. Frequency of deletion of CDKN2A (9p21) gene in T-cell lymphomas in FISH analysis. Contemporary Oncology/Współczesna Onkologia. 2011;15(3):131-136. doi:10.5114/wo.2011.23001. APA Pastwińska, A., Rygier, J., Woroniecka, R., Grygalewicz, B., Krawczyk, P., & Pieńkowska-Grela, B. et al. (2011). Frequency of deletion of CDKN2A (9p21) gene in T-cell lymphomas in FISH analysis. Contemporary Oncology/Współczesna Onkologia, 15(3), 131-136. https://doi.org/10.5114/wo.2011.23001 Chicago Pastwińska, Anna, Jolanta Rygier, Renata Woroniecka, Beata Grygalewicz, Paulina Krawczyk, Barbara Pieńkowska-Grela, and Grzegorz Rymkiewicz et al. 2011. "Frequency of deletion of CDKN2A (9p21) gene in T-cell lymphomas in FISH analysis". Contemporary Oncology/Współczesna Onkologia 15 (3): 131-136. doi:10.5114/wo.2011.23001. Harvard Pastwińska, A., Rygier, J., Woroniecka, R., Grygalewicz, B., Krawczyk, P., Pieńkowska-Grela, B., Rymkiewicz, G., and Feliksbrot-Bratosiewicz, M. (2011). Frequency of deletion of CDKN2A (9p21) gene in T-cell lymphomas in FISH analysis. Contemporary Oncology/Współczesna Onkologia, 15(3), pp.131-136. https://doi.org/10.5114/wo.2011.23001 MLA Pastwińska, Anna et al. "Frequency of deletion of CDKN2A (9p21) gene in T-cell lymphomas in FISH analysis." Contemporary Oncology/Współczesna Onkologia, vol. 15, no. 3, 2011, pp. 131-136. doi:10.5114/wo.2011.23001. Vancouver Pastwińska A, Rygier J, Woroniecka R, Grygalewicz B, Krawczyk P, Pieńkowska-Grela B et al. Frequency of deletion of CDKN2A (9p21) gene in T-cell lymphomas in FISH analysis. Contemporary Oncology/Współczesna Onkologia. 2011;15(3):131-136. doi:10.5114/wo.2011.23001.

Dermal infiltrates of cutaneous T‐cell lymphomas with epidermotropism but not other cutaneous lymphomas are abundant with langerin+ dendritic cells

The Langerhans cell (LC) hypothesis suggests that cutaneous T-cell lymphomas (CTCL) are diseases of chronic T-cell stimulation by LC-mediated antigen presentation. To investigate a broad panel of CTCL and cutaneous B-cell lymphomas (CBCL) for the spatial association of langerin(+) dendritic cells (DC) with T and B cells in the skin, respectively. Fifty-five specimens of CTCL and 10 of CBCL were double-stained with monoclonal antibodies against langerin and CD3 or CD20, respectively, and evaluated by confocal laser scan microscopy. Dermal infiltrates in mycosis fungoides (n = 38), primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma (n = 3) and primary cutaneous peripheral T-cell lymphoma, unspecified (n = 3) were characterized by a high frequency of dermal langerin(+) DCs. These cells were exclusively present in the malignant infiltrates. No direct co-localization of CD3 and langerin could be resolved. Dermal langerin(+) cells were detected only in one of six primary cutaneous anaplastic large cell lymphomas (C-ALCL), characterized by epidermotropism. In other C-ALCL cases (five of six), in lymphomatoid papulosis (n = 3), subcutaneous panniculitis-like T-cell lymphoma (n = 2), and all variants of CBCL no dermal langerin(+) DCs could be found. Langerin(+) DCs are abundant in the dermal infiltrates of T-cell lymphomas with specific involvement of the epidermis. This might indicate that immature LC and neoplastic T cells interact and gives rise to further studies to characterize the phenotype of the langerin(+) cell population described here and its role in the pathology of CTCL.

Clinicopathologic Analysis of Cutaneous Lymphoma in Taiwan: A High Frequency of Extranodal Natural Killer/T-Cell Lymphoma, Nasal Type, With an Extremely Poor Prognosis

Primary cutaneous lymphoma is an uncommon, extranodal lymphoma, and it is usually more indolent with a better prognosis than its histologically similar systemic counterpart is. To illustrate the clinicopathologic features of cutaneous lymphomas in Taiwan and to compare the relative frequencies of subtypes of cutaneous lymphoma among different geographic areas. A total of 56 patients with cutaneous lymphomas were retrospectively collected and were reclassified according to the 2005 World Health Organization and the European Organization for Research and Treatment of Cancer and the 2008 World Health Organization classifications. The data were compared with those from other studies for different geographic areas. Thirty-one (55%) tumors were primary cutaneous lymphomas, and twenty-five (45%) tumors were secondary or concurrent cutaneous lymphomas. Among primary cutaneous lymphomas, 23 cases (74%) were T-cell or natural killer-cell lymphomas, and 8 cases (26%) were B-cell lymphomas. The most common types were extranodal natural killer/T-cell lymphoma, nasal type, and primary cutaneous peripheral T-cell lymphoma, unspecified (5 cases each; 16%). In contrast with other primary cutaneous B-cell and T-cell lymphomas, either primary or secondary extranodal cutaneous natural killer/T-cell lymphomas, nasal type, had extremely poor prognoses (1-year overall survival, 0%). This study showed that the frequency of subtypes of primary cutaneous lymphoma varied in different geographic areas. Compared with the Western countries, there was a higher frequency of extranodal natural killer/T-cell lymphoma, nasal type, and a lower frequency of mycosis fungoides in Taiwan. Extranodal natural killer/T-cell lymphoma, nasal type, also had an extremely poor prognosis compared with other lymphomas.

P100 Primary cutaneous peripheral T-cell lymphoma, unspecified: a case report

Introduction Primary cutaneous T-cell lymphomas (PCTCLs) represent 76,9% of all primary cutaneous lymphomas. MF/Sezary and CD30+ T cell lymphoproliferative disorders are the most common, while the histological entity of PTCL unspecified (NOS) represents<10% of all CTCLs [1–4]. Case history A 44-year-old male patient presented to our department with several (up to 10) asymptomatic, isolated, sharply demarcated violet red nodules on trunk, extremities, as well as, on his face, and neck. The lesions were generally oval and ranged size from 1 to 4 cm in diameter. According to the patient, the skin lesions appeared on his face 9 months ago, and become multiple and more generalized. Initial skin biopsy was consistent with the diagnosis of pseudo-T-cell lymphoma. Three months later, a new skin biopsy was performed due to progression of the disease, and the diagnosis of peripheral T cell lymphoma unspecified was reached. Histological examination revealed diffuse infiltration of dermis with medium and large size lymphoid cells. Immunophenotypic analysis showed expression of the following markers: CD3+, CD4+, CD43+, while there was no expression of CD30. Ki67 was expressed on 60–70% at large lymphoid cells. Tissue clonality studied by PCR detected positive TCR rearrangement. Physical examination, complete blood cell counts, computed tomography of chest and abdomen and bone marrow aspirate failed to demonstrate extracutaneous disease. After all the adequate staging procedures the diagnosis of a primary cutaneous peripheral T cell lymphoma unspecified was established. The patient was treated with combined chemotherapy (cycloposphamide, Mitoxantron, Vincristine, and Prednisolone). Complete remission of skin lesions was achieved after the second course of therapy. Discussion Most patients with unspecified PTL presented with nodular or tumorous skin lesions, solitary, localized or multifocal [1]. The duration of skin lesions prior to diagnosis varied between 1 and 100 months (median 6 months) [1] Therapy consists in most cases of Doxorubicin-based chemotherapy (CHOP or CHOP-like), radiotherapy, or a combination of both. Complete remission can be achieved but are generally short-lived, requiring additional multiagent chemotherapy soon afterward. Prognosis is poor, with a 5-year survival rate of<20%. Conclusion PTCLs unspecified, presenting in the skin are rare and have an unfavorable prognosis. It is fair to conclude that it is as yet unknown how such patients with a PTL unspecified, can best be treated. Because of the inefficacy of current standard regimens, new therapies should be investigated.