The importance of assessing blood tumour burden in cutaneous T-cell lymphoma

Mycosis fungoides (MF) and S\ue9zary syndrome (SS) are the best-studied subtypes of cutaneous T-cell lymphoma. The level of blood tumour burden in patients is important for diagnosis, disease staging, prognosis and management, as well as assessing treatment response. Until recently, the assessment of blood involvement was made using manual counts of morphologically atypical T cells (S\ue9zary cells), but this approach may be subjective, and is affected by interobserver variability. Objective and consistent approaches to accurately quantifying blood involvement are required to ensure appropriate stage-related management of patients and to improve our understanding of the prognostic implications of blood tumour burden in these diseases. While assessment of blood involvement is common in SS and advanced-stage MF, an improved understanding of the implications of blood involvement at early disease stages could help identify patients more likely to progress to late-stage disease, and hence guide treatment decisions and frequency of follow-up assessment, ultimately improving patient outcomes. This concise review discusses the development of flow cytometry-based classifications for assessing blood involvement in MF and SS, and summarizes current recommendations for blood classification and assessment of blood response to treatment

The importance of assessing blood tumour burden in cutaneous T-cell lymphoma

Mycosis fungoides (MF) and Sezary syndrome (SS) are the best-studied subtypes of cutaneous T-cell lymphoma. The level of blood tumour burden in patients is important for diagnosis, disease staging, prognosis and management, as well as assessing treatment response. Until recently, the assessment of blood involvement was made using manual counts of morphologically atypical T cells (Sezary cells), but this approach may be subjective, and is affected by interobserver variability. Objective and consistent approaches to accurately quantifying blood involvement are required to ensure appropriate stage-related management of patients and to improve our understanding of the prognostic implications of blood tumour burden in these diseases. While assessment of blood involvement is common in SS and advanced-stage MF, an improved understanding of the implications of blood involvement at early disease stages could help identify patients more likely to progress to late-stage disease, and hence guide treatment decisions and frequency of follow-up assessment, ultimately improving patient outcomes. This concise review discusses the development of flow cytometry-based classifications for assessing blood involvement in MF and SS, and summarizes current recommendations for blood classification and assessment of blood response to treatment.Dermatology-oncolog

Prognostic factors, prognostic indices and staging in mycosis fungoides and Sezary syndrome: where are we now?

Dermatology-oncolog

Evaluating Response Trends of Chlormethine/Mechlorethamine Gel in Patients With Stage I-IIA Mycosis Fungoides: Analysis of Individual Patient Data From a Randomized Controlled Phase II Study to Facilitate Optimal Treatment Experiences.

Chlormethine (CL) gel was approved for treatment of mycosis fungoides based on the pivotal 201 trial (NCT00168064). Data visualization from individual patients is a powerful tool for discovery of hidden treatment trends. Here, we present a post hoc analysis of individual patient data from the pivotal trial to provide a more granular depiction of treatment and response changes over time, with an emphasis on end of treatment status. Individual patient response data were plotted over a 12-month treatment period to visualize patient experiences while using CL gel. Responder status was assigned according to end-of-treatment Composite Assessment of Index Lesion Severity (CAILS) score, and patients were classified as early (≤4 months) or late responders based on timing of response. Baseline and active treatment characteristics were compared between early and late responders, and baseline body surface area (BSA) was compared between responders and patients with stable or progressive disease. Data from 123 patients with baseline and postbaseline results were included. At the end of treatment, 64.2%/55.3% were responders, 30.9%/34.1% had stable disease, and 4.9%/10.6% had progressive disease by CAILS and mSWAT, respectively. Among patients who responded to treatment, 64.6% and 35.4% were early and late responders, respectively. Response pattern analysis also identified patients with an intermittent response or initial progressive disease. Baseline BSA was not associated with responder status. Late responders had longer treatment duration and higher postbaseline plaque elevation, while early responders had a higher frequency of dermatitis. Results presented here can facilitate optimal treatment experiences for patients starting CL gel

Cutaneous T cell lymphoma

Primary cutaneous T cell lymphomas (CTCLs) are a heterogeneous group of lymphomas that present in the skin with no evidence of extracutaneous disease at the time of diagnosis. CTCL subtypes demonstrate a variety of clinical, histological, and molecular features, and can follow an indolent or a very aggressive course. The underlying pathogenetic mechanisms are not yet entirely understood. The pathophysiology of CTCL is complex and a single initiating factor has not yet been identified. Diagnosis is based on clinicopathological correlation and requires an interdisciplinary team. Treatment decision is made based on short-term and long-term goals. Therapy options comprise skin-directed therapies, such as topical steroids or phototherapy, and systemic therapies, such as monoclonal antibodies or chemotherapy. So far, the only curative treatment approach is allogeneic haematopoietic stem cell transplantation. Novel therapies, such as chimeric antigen receptor T cells, monoclonal antibodies or small molecules, are being investigated in clinical trials. Patients with CTCL have reduced quality of life and a lack of effective treatment options. Further research is needed to better identify the underlying mechanisms of CTCL development and course as well as to better tailor treatment strategies to individual patients.Cutaneous T cell lymphoma is a group of lymphomas that initially manifest in the skin. This Primer summarizes the pathophysiology, epidemiology, diagnosis and management of these disorders.Dermatology-oncolog

Classifications of cutaneous lymphomas and lymphoproliferative disorders: An update from the EORTC cutaneous lymphoma histopathology group.

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

Developments in the understanding of blood involvement and stage in mycosis fungoides/Sezary syndrome

Dermatology-oncolog