Molecular cytogenetics of primary cutaneous t-cell lymphomas: From cytogenetics to the identification of specific gene-level aberrations
Year: 2004
Type: dissertation
Abstract: Cutaneous T-cell lymphomas (CTCL) are a heterogenous group of non-Hodgkin-lymphomas (NHL), belonging to primary cutaneous lymphomas and show a growing incidence.The most common form of CTCL is mycosis fungoides (MF), and its leukaemic form with erythrodermia and affected lymph nodes is called Sézary syndrome (SS).Sézary syndrome may evolve from M F, or start direc tly as SS.Clinically, MF usually develops slowly over years from ec zema like patches resulting in tumours or erythroderma.In the skin lesion of MF malignant CD4-positive, atypical lymphocytes infiltrate towards the epidermis, surrounded by reactive (e.g.cytotoxic) T lymphocytes.At later stages, the malignant cells may be observed in the lymphoreticular system, blood, kidneys, lungs and brain.The cause, anatomical site and timing of malignant transformation are unknown.Early-stage MF is difficult to diagnose clinically and histologically, because it resembles benign, inflammatory eruptions e.g.alopecia mucinosa or large-plaq ue parap soriasis (LP P).A con troversy prev ails, as to whether L PP is a pr emalignant o r a fully malignant condition.T-cell gene rearrangemen t analysis has not provided decisive help beacuse of its relative lack of specificity, but has indicated early systemic spread of the disease.None of the treatments used is curative, but at early stages of the disease, long term remissions can be achieved.Acquired , recurrent chr omoso mal aberra tions in cance r associate with m alignant transformation and disease progression.Such aberrations may reveal microscopically observable amplification of oncogenes, deletions of tumour suppressor genes or transloca tions producing functional fusion genes or disrupting tumour suppressor gene s.Patients with C TCL c ommon ly show a wide variety of clonal or non-clonal chromosomal aberrations in their blood or skin.The conventional cytogenetic studies of CTC L are difficult, as the malignant ce lls respond p oorly to mito gens need ed for indu cing visible, ana lysable mitotic chromosomes for G-banding staining.Before the molecular cytogenetic studies presented here, no recurrent specific
Source: Työväentutkimus Vuosikirja
Author: Leena Karenko
Language: English
Subfield: Dermatology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W1165496488