Pleomorphic T-cell Lymphoproliferative Disorder Research Articles

Decrease of 5-hydroxymethylcytosine in primary cutaneous CD4+ small/medium sized pleomorphic T-cell lymphoproliferative disorder

BackgroundPrimary cutaneous CD4+ small/medium-sized pleomorphic T-Cell lymphoproliferative disorder (PC-SMTLD) has been considered as a controversial dermatological disease that has been included in cutaneous T-cell lymphoma group, presenting most commonly as a solitary nodule and/or plaque with a specific and characteristic head and neck predilection. Due to the considerable overlap between PC-SMTLD and pseudolymphoma (PL), the differential diagnosis is often challenging. Methylation of DNA at position 5 of cytosine, and the subsequent reduction in intracellular 5-hydroxymethylcytosine (5-hmC) levels, is a key epigenetic event in several cancers, including systemic lymphomas. However, it has rarely been studied in cutaneous lymphomas. ObjectivesThe authors aimed to explore the role of differential 5-hmC immunostaining as a useful marker to distinguish PC-SMTLD from PL. MethodsRetrospective case series study with immunohistochemical and immunofluorescence analysis of 5-hmC was performed in PL and PC-SMTLD. ResultsSignificant decrease of 5-hmC nuclear staining was observed in PC-SMTLD when compared with PL (p < 0.0001). By semi-quantitative grade integration, there were statistical differences in the final 5-hmC scores in the two study groups. The IF co-staining of 5-hmC with CD4 revealed a decrease of 5-hmC in CD4+ lymphocytes of PC-SMTLD. Study limitationsThe small clinical sample size of the study. ConclusionsThe immunorreactivity of 5-hmC in CD4+ lymphocytes was highly suggestive of a benign process as PL. Furthermore, the decrease of 5-hmC nuclear staining in PC-SMTLD indicated its lymphoproliferative status and helped to make the differential diagnosis with PL.

Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder in a young woman.

Primary cutaneous CD4+ small/medium T-cell lymphoproliferative disorder (CD4+PCSM-LPD) is a low-grade cutaneous T cell disorder. There is no standardized approach to treatment of CD4+ PCSM-LPD due to its rarity. Herein, we discuss a 33-year-old woman with CD4+PCSM-LPD which resolved after a partial biopsy. We highlight that conservative and local treatment modalities should be considered prior to utilizing more aggressive and invasive treatment options.

PRIMARY CUTANEOUS CD4+ SMALL/MEDIUM CELL LYMPHOPROLIFERATIVE DISORDER IN THE FACE: CASE REPORT

Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (PCSMP-TLPD) is a rare provisional entity with uncertain malignant potential, with most cases presenting an indolent course and favorable prognosis. Clinically, PCSMP-TLPD presents as a solitary and asymptomatic lesion, usually in the head and neck region and trunk. An 86-year-old man was referred with a complaint of crusted lesion on the left side of the face for 1 year, with symptomatology in the last days. After incisional biopsy, microscopy showed a dermal infiltrate in a band‐like distribution of small/medium‐sized pleomorphic lymphocytes. Moreover, focal epidermotropism and folliculotropic patterns were observed. Immunohistochemistry showed positivity for CD3 and CD4 in the neoplastic cells. Disease staging showed local involvement. After 3 years of follow-up, the patient is healthy. PCSMP-TLPD presents an indolent course, such as shown in the current case, so its correct diagnosis is important due to the prognostic implications.

27737 The clinical spectrum of primary cutaneous CD4 + small/medium-sized pleomorphic T-cell lymphoproliferative disorder: An updated systematic literature review and case series

Background: Primary cutaneous CD4 + small/medium pleomorphic T-cell lymphoproliferative disorder (SMPLPD) is a provisional entity within the 2016 World Health Organization classification of primary cutaneous lymphomas. The condition is currently classified as a lymphoproliferative disorder to emphasize its benign course and discourage aggressive, systemic, treatment modalities.

248 The clinical spectrum of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder: An updated systematic literature review and case series

248 The clinical spectrum of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder: An updated systematic literature review and case series

Clinicopathological analysis of primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoproliferative disorder: a retrospective study of 22 patients.

Primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoproliferative disorder has been defined as a type of lymphoproliferative disorder with indolent clinical course and excellent prognosis, yet a precise diagnosis is still hard to reach. A retrospective analysis of 22 patients including 16 females and six males was performed. The age of patients ranged from 5 to 79years. The average age of all patients was 43.5, and the median age of all patients was 44.5. Two patients had multiple lesions, and others were presented with a solitary asymptomatic lesion. Besides general features, folliculotropism was observed in four cases. In addition to express CD3 and CD4, CD30 were positive to some extent. Some reactive cells could express CD8 and CD20. For follicular helper T-cell markers, although CXCL-13 was negative in the stained cases (18/18), the expression of PD-1 (12/17), BCL-6 (12/16) and CD10 (11/15) was observed in most cases. In addition, we performed T-cell receptor (TCR) rearrangement on five patients, and all of them showed monoclonality. Nearly all patients had excellent prognosis. Primary cutaneous CD4-positive small/medium pleomorphic T-cell lymphoproliferative disorder is complex. Some features like folliculotropism should also be noted. Besides, the expression of follicular helper T-cell markers is not invariable. Moreover, CD8 positivity, Ki-67 index, and lesion number were perhaps not absolute prognostic indicators. To reach a diagnosis of this rare entity, putting all the pieces together is important.

CD8+ Phenotype of Primary Cutaneous Small-Medium Pleomorphic T-Cell Lymphoproliferative Disorder

CD8+ Phenotype of Primary Cutaneous Small-Medium Pleomorphic T-Cell Lymphoproliferative Disorder

Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder in a woman successfully treated with intralesional interleukin injection and occlusive corticosteroid

Primary cutaneous CD4⁺ small/medium pleomorphic T-cell lymphoproliferative disorder in a woman successfully treated with intralesional interleukin injection and occlusive corticosteroid

Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder: Where do we stand? A systematic review.

Primary cutaneous CD4+ small/medium pleomorphic T-cell lymphoproliferative disorder (PCSMP-TLPD) is a provisional entity with uncertain malignant potential according to the latest revision of the WHO classification for lymphoid neoplasms. We conducted a systematic literature review of all previously reported cases of PCSMP-TLPD to highlight their typical and atypical features. The main features of PCSMP-TLPD and its possible clinicopathologic overlap with similar disorders are also discussed. It is hoped that this review will provide a useful outline of this condition and the most important differential diagnoses. Finally, we recommend a rigorous consensus among cutaneous lymphoma experts in drafting diagnostic criteria and the best case definition.

Treatment of primary cutaneous CD4+ small to medium pleomorphic T-cell lymphoproliferative disorder with the use of intralesional triamcinolone

Treatment of primary cutaneous CD4+ small to medium pleomorphic T-cell lymphoproliferative disorder with the use of intralesional triamcinolone

The development of primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder at the site of a melanoma excision scar

Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoproliferative disorder (PCSM-LPD) is a rare and low-grade form of cutaneous T-cell lymphoma (CTCL), representing 2% of all primary cutaneous lymphomas. Because of its rarity, the etiology or exact clinicopathology of PCSM-LPD remains unclear. We present the first case of PCSM-LPD, to our knowledge, arising at a past melanoma excision site. A 72-year-old woman with a past medical history significant for melanoma-in-situ excised 36 years ago presented to our clinic for evaluation of a single, erythematous plaque of the posterior arm within a melanoma excision scar. A biopsy was performed, revealing PCSM-LPD. Reports of the development of other T-cell lymphoproliferative disorders after prior skin trauma such as chemical burns, thermal injury, and mechanical trauma exist in the literature. Physicians should be aware of the possibility of the appearance of T-cell lymphoproliferative disorders at the site of scars or prior trauma with a time lag of months to years.

Incidence and ten-year follow-up of primary cutaneous lymphomas: a single-centre cohort study.

Primary cutaneous lymphomas (PCLs) are a rare group of extranodal non-Hodgkin lymphomas, and epidemiological data in Mediterranean countries are scarce. To investigate the incidence and characteristics of PCL in a single tertiary referral centre in Italy. A total of 141 PCL patients, seen over a 10-year follow-up period, were investigated. Incidence rate of PCL was 0.8 cases/100,000 person years. T-cell lymphoma represented 78.7% of all cases, the majority being early mycosis fungoides (MF) (64%; median age: 66 years), followed by lymphomatoid papulosis (LyP) (19%; median: age 48 years), and others (median age: 72 years), including eight cases of anaplastic large CD30+ T-cell lymphoma, four CD4+ small-medium pleomorphic T-cell lymphoproliferative disorder, four Sézary syndrome, one subcutaneous panniculitis-like T-cell lymphoma, one extranodal NK/T-cell lymphoma nasal-type, and one angioimmunoblastic T-cell lymphoma. B-cell lymphoma accounted for 21.3% of PCL, with 20 cases of cutaneous follicular centre B-cell (median age: 63 years), four primary cutaneous marginal zone, three primary cutaneous diffuse large B-cell, and three leg-type lymphoma. Complete remission within the first year after diagnosis occurred in 70.4% of MF, 61.9% of LyP, 78.9% of other T-cell lymphoma, and 93.1% of B-cell lymphoma cases. Based on a Cox proportional hazard regression model, age, gender, stage, and lactate dehydrogenase and β2-microglobulin blood levels did not predict clinical remission of MF or LyP. The incidence and characteristics of PCL in Italy are similar to those in other European countries. PCLs may be diagnosed at very early stages with good prognosis.