Abstract

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is part of a spectrum of cutaneous CD30+ lymphoproliferative disease that also includes lymphomatoid papulosis. It often occurs in elderly patients, presenting at a median age of 60 years, although it may occur at any age. It is a CD30+ T-cell neoplasm composed of large cells with anaplastic, pleomorphic, or immunoblastic morphology, with exclusively cutaneous onset and localization. The clinical course of pcALCL is predominantly indolent. Most elderly patients with lymphoma tend to have a sedentary lifestyle, which has a negative effect on their quality of life (QoL) and survival. Several studies indicate that exercise has a positive impact on QoL because it reduces peak oxygen consumption, improves physical capacity, increases self-esteem, reduces accumulated stress, and promotes relaxation. Therefore, particularly in indolent lymphomas, it is necessary to indicate a program of physical activity to be practiced systematically. Complete surgical excision and local radiotherapy are the first line gold standard in pcALCL with a solitary lesion.

Highlights

  • Primary cutaneous anaplastic large cell lymphoma is a CD30+ T-cell neoplasm composed of large cells with anaplastic, pleomorphic, or immunoblastic morphology, with exclusively cutaneous onset and localization [1]

  • The morphology and immunophenotypic characteristics of Lymphomatoid papulosis (LyP) and Primary cutaneous anaplastic large cell lymphoma (pcALCL) overlap significantly and no biomarker has to date been able to reliably distinguish these two entities, so it is essential to correlate the pathological results with the clinical history that represents the only distinctive elements

  • PcALCL is a lymphoma with a good prognosis and low mortality, which mainly concerns the elderly

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Summary

Introduction

Primary cutaneous anaplastic large cell lymphoma (pcALCL) is a CD30+ T-cell neoplasm composed of large cells with anaplastic, pleomorphic, or immunoblastic morphology, with exclusively cutaneous onset and localization [1]. The clinical course of pcALCL is predominantly indolent, completely different from that of the systemic forms of anaplastic large cell lymphomas (ALCL) [2]. Neoplastic cells defined as “hallmark cells” are morphologically distinguishable as large pleomorphic cells with abundant cytoplasm and eccentric kidney-shaped nuclei These are common aspects of all kinds of ALCL, but each type differs in clinical presentation, prognosis, and molecular features. About 25% of patients have the DUSP22-IR4 locus at onset, while TP63 rearrangements are rare Unlike systemic forms, these chromosomal aberrations do not appear to be related to a worse prognosis [5]. The main objective of this review will be to describe all new diagnostic and therapeutic features of primary cutaneous ALCL regarding elderly patients and to evaluate the importance of physical activity

Clinical Presentation
Histological and Molecular Features
Differential Diagnosis
Therapies
Findings
Discussion
Conclusions
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