Cutaneous Peripheral T-cell Lymphoma Research Articles

176 Indolent T-Lymphoblastic Proliferation Presenting Concurrently With a Cutaneous Peripheral T-Cell Lymphoma, a Case Study

176 Indolent T-Lymphoblastic Proliferation Presenting Concurrently With a Cutaneous Peripheral T-Cell Lymphoma, a Case Study

Primary Cutaneous Peripheral T-Cell Lymphoma in a Sporotrichoid Pattern: A Case Report

We present the extraordinary case of a 72-year-old man with a history of primary cutaneous peripheral T-cell lymphoma not otherwise specified (pcPTCL-NOS) previously controlled with topical agents who developed tumours in a sporotrichoid pattern. Culture of the tumours was negative, and histopathology showed findings consistent with recurrent pcPTCL. The tumours were successfully treated with localised radiation therapy. Sporotrichoid lesions are an extremely rare and atypical presentation of cutaneous lymphoma, with only 2 other cases reported in the literature. Our case reinforces the need to include cutaneous lymphoma in the differential diagnosis of nodules on the extremities spreading in a sporotrichoid pattern. Clinical recognition of this atypical presentation of cutaneous lymphoma allows for prompt, effective treatment, which might include localised radiation therapy.

The discovery and development of romidepsin for the treatment of T-cell lymphoma

ABSTRACTIntroduction: Romidepsin is a potent and selective inhibitor of histone deacetylases (HDCAi). It is also the only bicyclic inhibitor to undergo clinical assessment and is considered a promising drug for the treatment of T-cell lymphomas. The cellular action of romidepsin results in enhanced histone acetylation, as well as the acetylation of other nuclear or cytoplasmic proteins, influencing cell cycle, apoptosis, and angiogenesis. In phase II studies involving patients with relapsed or refractory of cutaneous T-cell lymphoma (CTCL) and peripheral T-cell lymphoma (PTCL), romidepsin produced overall response rates (ORR) of 34–35% and 25–38%, with complete response (CR) rates of 6% and 15–18%, respectively.Areas covered: This review summarizes the development of romidepsin, the mechanisms behind its antineoplastic action and its pharmacology. It also covers its pharmacokinetic and pharmacodynamic properties, as well as the preclinical and clinical data on its activity in T-cell lymphoma.Expert opinion: Since there are only few effective therapies available for T-cell lymphomas, romidepsin is a valuable option for relapsed/refractory patients with both CTCL and PTCL. It’s also generally well tolerated, and gives potentially durable responses for patients with advanced and symptomatic disease. Combinations of romidepsin with other antineoplastic agents may also further improve drug response and outcomes in T-cell lymphoma.

Primary cutaneous peripheral T-cell lymphoma mimicking pyoderma gangrenosum: A case report

Primary cutaneous peripheral T-cell lymphoma mimicking pyoderma gangrenosum: A case report

Hypomagnesemia and exposure to romidepsin: A Research on Adverse Drug events and Reports (RADAR) project.

e14072 Background: Romidepsin (R) is indicated for cutaneous T-cell lymphoma (CTCL) and peripheral T-cell lymphoma. Hypomagnesemia (HM) is common in these patients and is also listed in the Full Prescribing Information (FPI) for R. Moreover, since cardiac arrhythmias are associated with HM, patients exposed to R may be at higher risk. This study assessed whether HM was causally related to R exposure and to what extent magnesium (M) supplementation was undertaken in those with HM. Methods: We searched a large, U.S. patient data repository to detect all patients (aged 20-94 years) exposed to R (10/2010-01/2017). For these patients, serum M, as well as M supplementation data, was collected. Baseline M was assessed at initial R exposure, and HM was defined as either, a decline in M to < 1.8 mg/dL after R exposure or, in those with baseline M < 1.8 mg/dL, a decline in M by ≥0.1 mg/dL after R exposure. For each patient with HM after R exposure, the validated Naranjo Adverse Drug Reaction Probability Scale (NADRPS) was used to determine the probability that HM was caused by R. Results: Of 51 R-exposed CTCL (or other lymphoma) patients, 25 (49.0%) had HM. Of these 25, NADRPS scores yielded: 1 doubtful, 16 possible, 8 probable and 0 definite. Additionally, 24 of 25 patients with HM had documentation for presence or absence of M supplementation: 9 (37.5%) had supplementation with M agents considered to be bioavailable, 12 (50%) received M agents considered to have low bioavailability, and 3 (12.5%) had no M supplementation. Conclusions: In this study population, 25 of 51 (49.0%) patients had HM after R exposure, which appears to nearly double the percentage of patients described in the FPI. Moreover, 8 of 25 (32%) patients with HM had causality attributed to R exposure. In addition, 15 of 24 (63%) patients with HM received M supplementation with agents considered to have low bioavailability or received no M supplementation. These findings support the need for ongoing monitoring of R-exposed patients with low M, as well as the importance of repleting M with agents considered to be adequately bioavailable. Also, given that HM appears to be more frequent in this study population compared to pre-marketing data in the FPI, further studies are warranted.

Secondary cutaneous peripheral T‐cell lymphoma, unspecified, with generalized benign‐looking dermatitis: a possibly distinct peripheral T‐cell lymphoma

Cutaneous lymphomas are a heterogeneous group of tumors with various clinical manifestations, histological features, and prognoses. Among 425 cases of cutaneous lymphoma, we describe eight cases of peripheral T-cell lymphoma, unspecified, presenting with generalized benign-looking dermatitis. Our case series included eight cases of secondary cutaneous peripheral T-cell lymphoma, unspecified. The observed clinical morphology features included drug eruption-like generalized morbilliform maculopatches in two cases, generalized folliculitis-like papules in two cases, and generalized eczematous dermatitis-like papules and patches in four cases. Histopathological examination demonstrated tumor cell infiltrates of mainly atypical, small-to-medium lymphoid cells that were perivascularly or periadnexally distributed throughout the dermis. Immunohistochemical staining results for CD2, CD3, CD4, and CD8 revealed that the tumor cells were T-cells. This collection of cases and literature review emphasizes the need for clinical suspicion of peripheral T-cell lymphoma, unspecified, in the differential diagnosis of adult patients with refractory eczematous or benign-looking skin lesions.

Cutaneous CD8+ Cytotoxic T-Cell Lymphoma Infiltrates: Clinicopathological Correlation and Outcome of 35 Cases.

IntroductionCytotoxic CD8+ T-cell lymphomas are only rarely encountered and thus remain only poorly characterized. Our aim was to collect and correlate clinical and histological data of CD8+ skin lymphoma infiltrates to obtain a proper subtype assignment of CD8+ skin lymphoma infiltrates and to derive putative prognostic markers thereof.MethodsFormalin-fixed and paraffin-embedded (FFPE) tissue of 35 patients with CD8+ cytotoxic cutaneous T-cell lymphoma infiltrates was retrieved from the archives of the Institute of Pathology and the Department of Dermatology, University Hospital Wuerzburg, dating back from 1998 until 2015. Cytological, histological, immunohistochemical and molecular genetic features were assessed and correlated with respective clinical data.ResultsThe identified cases of CD8+ cytotoxic atypical lymphoproliferative infiltrates of the skin (n = 35) comprised 13 cases of mycosis fungoides (MF)/Sézary syndrome (SS), 4 cases of subcutaneous panniculitis-like T-cell lymphoma (SPTCL), 5 cases of primary cutaneous acral CD8+ lymphoma [formerly indolent CD8+ lymphoid proliferation (ILP)] and 1 case of aggressive epidermotropic primary cutaneous T-cell lymphoma (AECTCL). Moreover, nine cases were classified as primary cutaneous peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) and three cases as systemic PTCL-NOS. Multiple skin lesions, a high proliferative index and especially a final subtype attribution to AECTCL or systemic PTCL-NOS were associated with a worse survival. Coexpression of CD68 by tumor cells was exclusively observed in indolent acral CD8+ T-cell lymphoma and thus indicated an invariably benign clinical course. No further distinctive markers could be derived from our analysis.ConclusionCutaneous infiltrates of CD8+ cytotoxic T-cell lymphoma comprise clinically and histologically heterogeneous entities of either primary cutaneous T-cell lymphomas or secondary infiltrates of otherwise systemic peripheral T-cell lymphomas. A thorough clinicopathological correlation with respective staging examinations remains the mainstay for correct subtype assignment and proper prognostication as long as no better markers have been defined.

Cutaneous peripheral T-cell lymphoma, not otherwise specified: A single-center prognostic analysis

Peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) is a rare and aggressive disease either originating in or secondarily involving the skin. We sought to assess clinical, histopathologic, and prognostic features of patients with cutaneous PTCL-NOS. This was a retrospective chart review of patients with cutaneous PTCL-NOS between 1993 and 2013. Thirty patients with PTCL-NOS were included. Fourteen had skin-only disease and 15 had concurrent skin and systemic disease at presentation. In primary cutaneous PTCL-NOS, the overall survival rate at 5years was 61% (95% confidence interval, 37-100%; number still at risk, 2). The median overall survival was 5.6years. Patients were diagnosed a median of 2.4months from symptom onset. Patients with concurrent disease died a median of 2.1years after diagnosis. The estimated overall survival rate at 5years after diagnosis was 29% (95% confidence interval, 13-67%; number at risk, 3). The median overall survival was 3.9years. Patients were diagnosed a median of 6months from symptom onset, with a 53% increased risk of death for each year from symptom onset to diagnosis. This was a retrospective study with a limited number of cases. Age at diagnosis and B-symptoms predict poor survival in patients with cutaneous PTCL-NOS. In addition, poorer survival is observed in patients with multifocal lesions and concomitant skin and systemic PTCL-NOS.

A Clinicopathological Analysis of Primary Cutaneous Lymphomas: A 6-year Observational Study at a Tertiary Care Center of South India.

Background:Little data are available concerning clinical and pathological patterns of cutaneous lymphomas in India.Aim:To analyze the clinical and histopathological characteristics of cutaneous lymphomas in Indian patientsMaterials and Methods:This is a single-center, prospective, observational study carried out from January 1, 2010, to December 31, 2015. The patients underwent clinical examination, human T-cell lymphotropic virus-1 (HTLV-1) screening, skin biopsy with hematoxylin and eosin and immunohistochemistry staining.Results:Among 35 cases, 33 (94.3%) were T-cell, and 2 (5.7%) were B-cell lymphomas. The mean age was 52.66, and the male to female ratio was 2.5:1. The most common types of T-cell lymphomas included mycosis fungoides (MF) (57.1%) followed by adult T-cell lymphoma/leukemia (ATL) (17.1%). Primary cutaneous peripheral T-cell lymphoma not otherwise specified was diagnosed in 17.1% and anaplastic large cell lymphoma in 2.9%. The morphological types of MF included polymorphic, poikilodermatous, folliculotropic, hypopigmented, hyperpigmented, mixed, and purpuric. Skin manifestations of ATL included ulcerated plaques and erythroderma. Epidermotropism was very marked in ATL (83.3%) than in MF (70%). Larger Pautrier's microabscess was noted in ATL compared to smaller ones in MF. Markedly dense, diffuse infiltrate of atypical cells was noted in ATL in contrast to mild to moderate nodular or perivascular infiltrate in MF. ATL had an extremely poor prognosis.Limitations:Identification of DNA integration of HTLV-1 by Southern blot could not be analyzed, and the number of cases studied is limited.Conclusions:The study showed unique patterns of subtypes of cutaneous lymphomas in our country. Variations in the clinical pattern and histopathological analysis will help to differentiate T-cell lymphoma types which have prognostic implications.

Hematopoietic stem cell transplantation for cutaneous T-cell lymphoma: Summary of 11 cases from two facilities in Japan and Brazil.

Some patients with cutaneous T-cell lymphoma (CTCL) show a miserable clinical course and the only option that can induce long-term remission for advanced CTCL may be hematopoietic stem cell transplantation (HSCT). So far, studies on HSCT for CTCL patients have been limited. In this study, we summarized 11 cases with CTCL treated with HSCT, including nine cases in Japan and two cases in Brazil. The patients were five cases with mycosis fungoides (MF), two cases with Sézary syndrome (SS), three cases with anaplastic large cell lymphoma, and one case with primary cutaneous peripheral T-cell lymphoma, not otherwise specified (PTL-NOS). Currently, seven out of 11 cases are alive (at 13-108 months after transplantation) and four died at 15 days to 14 months after transplantation. When focusing on the eight patients who received allogeneic HSCT for MF/SS and PTL-NOS, all four patients at 45 years old or under are alive at present. One case showed relapse in the skin. On the other hand, one out of the other four patients at over 45 years old survived. Engraftment failure was seen in one case and all the other three cases experienced relapse. Although this is only a case series with a small number, our study has suggested that we should be careful about age when treating patients with MF/SS by allogeneic HSCT.

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.

Expression of T-follicular helper markers in sequential biopsies of progressive mycosis fungoides and other primary cutaneous T-cell lymphomas.

T-follicular helper (Tfh) lymphocytes represent the neoplastic cells of angioimmunoblastic T-cell lymphoma and have been observed also in several cutaneous T-cell lymphomas (CTCLs) and extracutaneous T-cell lymphomas, including peripheral T-cell lymphoma, not otherwise specified, mycosis fungoides (MF), cutaneous CD4 small/medium T-cell lymphoma (CD4SMTCL), and Sezary syndrome. We studied a large number of different types of primary CTCL for expression of Tfh markers, including 36 biopsies from 21 patients with MF (with sequential biopsies from patch stage and tumor stage of 15 patients), 13 patients with CD4SMTCL, 9 with lymphomatoid papulosis, 11 with cutaneous anaplastic large cell lymphoma (cALCL), 2 with cutaneous γ/δ T-cell lymphoma, 8 with subcutaneous panniculitis-like T-cell lymphoma, 3 with cutaneous aggressive epidermotropic CD8 cytotoxic T-cell lymphoma, 6 with cutaneous peripheral T-cell lymphoma, not otherwise specified, and 1 with Sezary syndrome. Expression of at least 3 of 5 markers (PD-1, CXCL-13, ICOS, Bcl-6, and CD10) in >10% of tumor cells was observed in 33 biopsies (MF = 20; CD4SMTCL = 11; 1 each cutaneous anaplastic large cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma, respectively). Our study shows that a Tfh phenotype is very common in MF and CD4SMTCL but can be observed rarely also in other types of CTCL. Expression of Tfh markers should not be used for classification of any entity of CTCL and may only integrate other immunohistochemical stainings for a more accurate characterization of these disorders. Precise distinction of Tfh-positive CTCLs from secondary skin manifestations of angioimmunoblastic T-cell lymphoma cannot rest on demonstration of a Tfh phenotype alone and should be achieved by a synthesis of clinical, histological, and phenotypic features.

Cutaneous lymphomas: new entities and rare variants

Primary cutaneous lymphomas are the second most common group of extranodal non-Hodgkin lymphomas. Recently several new variants and entities have been described but have not yet become part of the World Health Organization (WHO) classification. These forms include the granulomatous form of mycosis fungoides, which is associated with a poorer prognosis, as well as indolent CD8+ lymphoproliferations on the head and at acral localizations. Within the group of cutaneous CD30+ lymphoproliferative disorders, new histological types of lymphomatoid papulosis have been identified, such as type D (CD8+ epidermotropic) and typeE (angioinvasive) which simulate aggressive lymphomas. Cutaneous peripheral T-cell lymphomas are a prognostically heterogeneous group of cutaneous lymphomas. The cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma and cutaneous gamma/delta T-cell lymphoma are very aggressive neoplasms, whereas cutaneous CD4+ small to medium-sized T-cell lymphoma in its solitary or localized form represents an indolent lymphoproliferation: the terminology, histogenesis and differentiation from nodular T-cell pseudolymphoma are still a matter of debate. Among B-cell lymphomas, disorders associated with Epstein-Barr virus (EBV) are discussed focusing on EBV diffuse large B-cell lymphoma of the elderly and EBV-associated mucocutaneous ulcer. This review describes the clinical, histological and immunophenotypic features of new and rare entities and variants of cutaneous lymphomas and highlights the impact of the clinicopathological correlation in the diagnostic process.

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。

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.

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.

A case of coexistent cutaneous marginal zone B-cell lymphoma and peripheral T-cell lymphoma

A case of coexistent cutaneous marginal zone B-cell lymphoma and peripheral T-cell lymphoma

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