T-cell chronic lymphocytic leukaemia: the spectrum of mature T-cell disorders.
Mature (post-thymic) T-cell malignancies comprise a heterogeneous group of diseases with distinct clinical, morphological and immunophenotypic features. As some of these features overlap in the various disorders, it is essential to use a number of diagnostic criteria to define the clinicopathological entities, namely: membrane markers, morphology by light and electron microscopy, clinical features, seroepidemiology of HTLV-I, histopathology, cytogenetic studies and DNA analysis. The latter, which is necessary to document clonality and confirm T-lineage, examines the rearrangement of the T-cell receptor beta, gamma and delta genes. By means of this multiparameter approach, it is possible to distinguish within the post-thymic leukaemias 4 disease entities: T-chronic lymphocytic leukaemia or large granular lymphocyte leukaemia, T-prolymphocytic leukaemia, adult T-cell leukaemia/lymphoma and Sézary syndrome.
Matutes
E
E
MRC Leukaemia Unit, Royal Postgraduate Medical School, London, UK.
Brito-Babapulle
V
V
Worner
I
I
Sainati
L
L
Foroni
L
L
Catovsky
D
D
D002454:Cell Differentiation; D002471:Cell Transformation, Neoplastic / Q000276:immunology / Q000473:pathology / Q000648:ultrastructure*; D006801:Humans; D015463:Leukemia, Prolymphocytic / Q000276:immunology / Q000473:pathology; D015461:Leukemia, Prolymphocytic, T-Cell / Q000276:immunology / Q000473:pathology*; D015459:Leukemia-Lymphoma, Adult T-Cell / Q000276:immunology / Q000473:pathology; D010641:Phenotype; D012751:Sezary Syndrome / Q000276:immunology / Q000473:pathology; D012878:Skin Neoplasms / Q000276:immunology / Q000648:ultrastructure; D013601:T-Lymphocytes / Q000145:classification / Q000473:pathology / Q000648:ultrastructure*