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The classification of primary cutaneous lymphomas and lymphoproliferative disorders (LPD) is continuously evolving by integrating novel clinical, pathological and molecular data. Recently two new classifications for haematological malignancies including entities of cutaneous lymphomas were proposed: the 5th edition of the WHO classification of haematolymphoid tumours and the International Consensus Classification (ICC) of mature lymphoid neoplasms. This article provides an overview of the changes introduced in these two classifications compared to the previous WHO classification. The main changes shared by both classifications include the downgrading of CD8+ acral T‐cell lymphoma to CD8+ acral T‐cell LPD, and the recognition of entities that were previously categorized as provisional and have now been designated as definite types including primary cutaneous small or medium CD4+ T‐cell LPD, primary cutaneous gamma/delta T‐cell lymphoma, primary cutaneous CD8+ aggressive epidermotropic cytotoxic T‐cell lymphoma, Epstein–Barr virus‐positive mucocutaneous ulcer. Both classifications consider primary cutaneous marginal zone B‐cell clonal neoplasm as an indolent disease but use a different terminology: primary cutaneous marginal zone lymphoma (WHO) and primary cutaneous marginal zone LPD (ICC). The 5th WHO classification further introduces and provides essential and desirable diagnostic criteria for each disease type and includes chapters on reactive B‐ or T‐cell rich lymphoid proliferations formerly referred as cutaneous pseudolymphomas, as well as histiocyte and CD8 T‐cell rich LPD in patients with inborn error of immunity. As already emphasized in previous lymphoma classifications, the importance of integrating clinical, histological, phenotypic and molecular features remains the crucial conceptual base for defining cutaneous (and extracutaneous) lymphomas. [ABSTRACT FROM AUTHOR]

Primary cutaneous T-cell lymphomas showing Gamma-Delta ( ) phenotype and predominantly Epidermotropic pattern are clinicopathologically distinct from classic primary cutaneous T-cell lymphomas. The last patient (n°4) experienced an ENT relapse (palate ulceration) after 42 months of remission under methotrexate. Cutaneous gamma delta T-cell lymphomas (CGDTCL) are rare lymphomas representing <1% of all cutaneous lymphomas, globally associated with a decreased overall survival.1,2 CGDTCL are a defined entity of the 2018 EORTC-WHO classification, most often presenting as nodules or infiltrated plaques, but mycosis fungoides-like3 and pagetoid reticulosis-like presentations have also been reported.4 The prognosis is generally poor.5,6 A few indolent cases have been reported.1,7-9 The clinical presentation of such indolent PCGDTCL has been poorly described. [Extracted from the article]

Background: Cutaneous peripheral T‐cell lymphoma, not otherwise specified (PTL NOS) is an aggressive, but poorly characterized neoplasm. Objectives: The European Organization for Research and Treatment of Cancer cutaneous lymphoma taskforce (EORTC CLTF) investigated 33 biopsies of 30 patients with primary cutaneous PTL NOS to analyse their clinical, histological, immunophenotypic features and outcome. Methods: Retrospective analysis of clinical data and histopathological features by an expert panel. Results: Cutaneous PTL NOS manifested clinically either with solitary or disseminated rapidly grown ulcerated tumours or disseminated papulo‐nodular lesions. Histologically, a mostly diffuse or nodular infiltrate in the dermis and often extending into the subcutis was found. Epidermotropism was rarely present and only mild and focal. Unusual phenotypes were frequent, e.g. CD3+/CD4−/CD8− and CD3+/CD4+/CD8+. Moreover, 18% of the cases exhibited an aberrant expression of the B‐cell marker CD20 by the tumour cells. All solitary tumours were located on the limbs and presented a high expression of GATA‐3 but this did not correlate with outcome and therefore could not serve as a prognostic factor. The prognosis was shown to be generally poor with 10 of 30 patients (33%) dying of lymphoma within the follow‐up of 36 months (mean value; range 3–144). The survival rates were 61% after 3 years (CI, 43–85%) and 54% after 5 years (CI, 36–81%). Small to medium‐sized morphology of tumour cells was associated with a better outcome than medium to large or large tumour cells. Age, gender, clinical stage, CD4/CD8 phenotype and GATA‐3 expression were not associated with prognosis. Chemotherapy was the most common treatment modality, but surgical excision and/or radiotherapy may represent an appropriate first‐line treatment for solitary lesions. Conclusions: Cutaneous PTL NOS shows an aggressive course in most patients independent of initial presentation, age and phenotype. Cytomorphology was identified as a prognostic factor. The data indicate a need for more effective treatment modalities in PTL NOS. [ABSTRACT FROM AUTHOR]

At diagnosis of ECTL, only one patient displayed an enlarged node > 1-5 cm (not biopsied), and the median number of Sézary cells was 1440 per mm SP 3 sp (range 396-4930). Dear Editor, The prognosis of erythrodermic cutaneous T-cell lymphomas (ECTL) depends on lymph node (N) and visceral (M) involvement, and blood stage (B). Pathological data at progression were reviewed for eight patients: skin biopsy (four patients), node biopsy (five patients) and cerebrospinal fluid (one patient). [Extracted from the article]

B Conclusion: b Within the heavily pre-treated MF population enrolled in TELLOMAK, Lacutamab shows clinical activity in KIR3DL2 expressing MF pts, with higher global ORR according to updated LN evaluation. MF pts are allocated to one of two cohorts: KIR3DL2 >=1% MF (Cohort 2) and KIR3DL2 <1% MF (Cohort 3). [Extracted from the article]

Summary: The erythrodermic ulcerated form of mycosis fungoides (MF) is exceptional, and treatment of refractory cases is challenging. Brentuximab vedotin (BV) is a monoclonal antibody combined with monomethyl auristatin E, recently approved for the treatment of refractory CD30+ cutaneous T‐cell lymphoma. We report a case of refractory MF in a 56‐year‐old man with a long history of large‐plaque parapsoriasis, as revealed by psoriasiform erythroderma, treated initially with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) polychemotherapy, inducing a 2‐year complete response. After relapse, interferon and gemcitabine were unsuccessful. Finally, treatment with BV was decided upon, despite the absence of CD30 expression. After three infusions of BV 1·8 mg kg−1, we achieved a complete and stable response, allowing an allogeneic stem cell transplant. The patient is still in complete remission, 19 months after the graft. This case illustrates the possibility of using BV in refractory CD30–MF as a salvage therapy. What's already known about this topic? The efficiency of brentuximab vedotin in refractory CD30+ cutaneous T‐cell lymphoma is well established.Erythrodermic forms of MF are challenging to treat, and allogeneic stem cell transplant is sometimes required. What does this study add? This psoriasiform, ulcerated and erythrodermic clinical presentation of MF is exceptional, and the response to brentuximab vedotin, despite the absence of CD30 expression on skin biopsy, allowed us to perform an allogeneic stem cell transplant, inducing long‐term complete remission. Linked Comment: Sun and Wang. Br J Dermatol 2019; 180:1300–1301. [ABSTRACT FROM AUTHOR]

Dear Editor, The treatment of advanced-stage cutaneous T-cell lymphomas (CTCL) is challenging and responses are usually short lived. Toxicities were observed in six of nine patients, including three severe adverse events: two patients developed severe acute renal failure (one of them had a single kidney), and one patient developed I Pneumocystis i pneumonia. Two of our patients with transformed SS had received four and six previous systemic treatment lines, respectively, and achieved a CR following B+B (22% of the whole group and 33% of patients with SS), allowing subsequent allogeneic HSCT and an ongoing CR 5 months and 1 year after transplant. [Extracted from the article]

Clinical presentation, therapeutic approach and outcome of primary cutaneous marginal zone B-cell lymphoma presenting as AL amyloidoma of the skin One patient (patient 3) with systemic lupus erythematosus and Sjögren syndrome developed PCMZBCL after abatacept treatment. Two patients received local treatment with very modest improvement: radiotherapy (24 Gy) (patient 3), topical clobetasol, cryotherapy and V-beam laser (patient 1). [Extracted from the article]

Dear Editor, The early diagnosis of cutaneous T-cell lymphoma (CTCL) may be challenging and based on a combination of clinical, biological, histological and molecular criteria.1 High-throughput sequencing (HTS) of the T-cell receptor genes allows identification and quantification of each T-cell clone through the recognition of CDR3 (complementarity-determining region 3) sequences in tissue samples.2 It can be used for the sensitive monitoring of CTCL minimal residual disease.3,4 It has also been shown that tumour clone frequency (TCF, defined as the frequency of the top T-cell clone as the percentage of all T cells in a skin sample) > 25% was an independent prognostic factor of progression-free and overall survival in early-stage mycosis fungoides (MF),5 and that it correlated with the degree of skin involvement in CTCL.6 Some studies suggested that HTS was more sensitive than standard polymerase chain reaction (PCR) for the identification of a dominant T-cell clone in early MF,2,7 whereas others found a similar sensitivity.8 However, the interpretation of HTS clonality data remains subjective, as internationally validated thresholds for the definition of a dominant T-cell clone by HTS have not been published. Diagnostic performance of high-throughput sequencing of the T-cell receptor beta gene for the diagnosis of cutaneous T-cell lymphoma Both techniques identified an identical T-cell clone in all blood-skin couples of samples, except in one blood sample in which a dominant T-cell clone was identified by HTS I TRB i , identical to the skin, but no dominant T-cell clone was identified by PCR. [Extracted from the article]

Cutaneous γ/δ T-cell lymphoma (CGD-TCL) is a recent entity described in the newly revised World Health Organization-European Organization for Research and Treatment of Cancer classification of cutaneous lymphomas, and is characterized by the γ/δ T-cell receptor expression on atypical lymphocytes. Only a few cases of primary CGD-TCL have been reported, with an extremely aggressive course (median survival time of 15 months). We describe 2 atypical cases of CGD-TCL. The first case was initially misdiagnosed as an inflammatory panniculitis due to the granulomatous infiltrate on the skin biopsy specimen. Diagnosis was confirmed using δ PCR that revealed γ/δ T-cell clonal expansion. The evolution was marked by predominant γ/δ T-cell infiltrate with diffuse body fat involvement as seen on positron emission tomography-computed tomography. The second case is the first described Epstein-Barr virus (EBV)-associated CGD-TCL with a rapidly fatal evolution. CGD-TCL is also a heterogeneous entity and δ PCR and EBV-encoded RNA probe to detect an EBV latent infection may help diagnose and characterize these cutaneous lymphomas. Copyright © 2011 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Dear Editor, Brentuximab vedotin (BV) is an antibody-drug conjugate, composed of a humanized anti-CD30 IgG1 and the antimitotic monomethyl auristatine E.[1] The interest of BV in the management of CD30 SP + sp mycosis fungoides (MF) and primary cutaneous anaplastic large-cell lymphoma (ALCL) has recently been demonstrated compared with methotrexate or bexarotene.[2] The expression of the CD30 receptor (tumour necrosis factor receptors family) by these T-cell lymphomas has been known since 1985.[3] It is not constant in MF and is more frequent in transformed disease.[1] BV may also be efficient in MF without any expression of CD30[4] and has been used in combination with bendamustine in transformed Sézary syndrome.[5] Finally, it can be used as a bridge to allogeneic stem-cell transplantation (ASCT) in relapsed or refractory non-Hodgkin lymphoma.[6] Can BV be resumed after ASCT? It is used in the consolidation treatment of Hodgkin lymphoma,[7] and has been studied in patients with CD30 SP + sp Hodgkin lymphoma or systemic ALCL who relapsed after achieving complete or partial remission with initial BV therapy,[8] but there is a lack of available data in primary cutaneous T-cell lymphomas. Brentuximab vedotin as a bridge to allogeneic stem-cell transplantation for refractory or relapsing patients with CD30 positive anaplastic or T-cell non-Hodgkin lymphomas: a study on behalf of the SFGM-TC. [Extracted from the article]

Challenges in the diagnosis of primary cutaneous CD30+ anaplastic large-cell lymphoma Dear Editor, Primary cutaneous anaplastic large-cell lymphoma (pcALCL) is a cutaneous T-cell lymphoma (CTCL) generally associated with a good prognosis. Peripheral T-cell lymphoma with progression to a clonally related, Epstein Barr virus+, cytotoxic aggressive T-cell lymphoma: evidence for secondary EBV infection of an established malignant T-cell clone. [Extracted from the article]