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Williams Hematology, 8e
| Part XI. Neoplastic Lymphoid Diseases > |
T-Cell Prolymphocytic Leukemia
Sections: Definition and
History, Etiology and
Pathogenesis, Genetics, Ataxia-Telangiectasia
Mutated783 Gene, T-Cell Leukemia
1 and Related Genes, Clinical Features, Laboratory Features, Differential
Diagnosis, Polyclonal T-Cell
Lymphocytosis, Large Granular
Lymphocytic Leukemia, Adult T-Cell
Leukemia/Lymphoma, Mycosis Fungoides
and SéZary Syndrome, T-Cell CLL, Therapy, Course and Prognosis.
Topics Discussed: adult t-cell lymphoma/leukemia; ataxia-telangiectasia; atm gene; chronic lymphocytic leukemia; htlv-i infections; leukemia, t-cell, chronic; lymphocyte, large granular; lymphocytosis; mtcp1 gene; mycosis fungoides; sezary syndrome; tcl1a protein, human.
Excerpt:"In 1989, the French-American-British (FAB) Cooperative Group
distinguished five subgroups of T-cell leukemia, namely: T-cell
CLL; T-cell PLL; human T-lymphotropic virus type I-positive (HTLV-I+)
adult T-cell leukemia/lymphoma; and Sézary syndrome.865 When
a new entity called large granular lymphocytic leukemia was defined
(see Chap. 96), the existence of T-cell CLL
as a distinct entity became a topic of debate.866–869 Because
of this the World Health Organization commissioned a panel of experts
to draft a new classification of the hematologic malignancies.870 At
a meeting in November 1997, this panel proposed a categorization
of peripheral T-cell malignancies that largely was based on the
REAL classification (see Chap. 92).871 However,
because of its aggressive clinical behavior, T-cell CLL was reclassified
under the heading of T-cell PLL, without regard to subtle differences
in morphology.872 Even together they account for
less than 5 percent of all chronic lymphoid leukemias.Use of comparative genomic hybridization to detect chromosomal
imbalances in T-cell PLL of patients in Europe found that the chromosomal regions
most often overrepresented were 8q (75%), 5p (62%),..."
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