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T-Cell Prolymphocytic Leukemia

Porwit, Anna LU (2024) p.3-662
Abstract

This chapter summarizes current knowledge and diagnostic criteria for T-cell prolymphocytic leukemia (T-PLL). T-PLL is an aggressive disease characterized by a proliferation of small to medium-sized lymphocytes with a postthymic phenotype usually involving the blood, bone marrow, lymph nodes, spleen, and skin. The leukemic cells are usually positive for CD2, CD3 CD5, CD7, CD43, CD52, and CD26, but CD4 and CD8 are variably expressed. T-PLL is diagnosed by the presence of three major criteria (>5 × 109/L cells of T-PLL phenotype in peripheral blood or bone marrow, T-cell clonality, abnormalities of 14q32 or Xq28 OR expression of TCL1A/B or MTCP1) or two major criteria and one of the minor criteria: abnormalities involving chromosome 11... (More)

This chapter summarizes current knowledge and diagnostic criteria for T-cell prolymphocytic leukemia (T-PLL). T-PLL is an aggressive disease characterized by a proliferation of small to medium-sized lymphocytes with a postthymic phenotype usually involving the blood, bone marrow, lymph nodes, spleen, and skin. The leukemic cells are usually positive for CD2, CD3 CD5, CD7, CD43, CD52, and CD26, but CD4 and CD8 are variably expressed. T-PLL is diagnosed by the presence of three major criteria (>5 × 109/L cells of T-PLL phenotype in peripheral blood or bone marrow, T-cell clonality, abnormalities of 14q32 or Xq28 OR expression of TCL1A/B or MTCP1) or two major criteria and one of the minor criteria: abnormalities involving chromosome 11 (11q22.3, ATM); abnormalities in chromosome 8, including idic(8)(p11), t(8;8), and trisomy 8q; abnormalities in chromosomes 5, 12, 13, or 22 or in complex karyotype; or involvement of T-PLL-specific site (e.g., splenomegaly, effusions).

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author
organization
publishing date
type
Chapter in Book/Report/Conference proceeding
publication status
published
subject
keywords
bone marrow biopsy, cytology, immunophenotyping, leukemia, prolymphocytic, T-cell
host publication
Hematopathology, 3rd Edition
pages
3 - 662
publisher
Elsevier
external identifiers
  • scopus:105011420998
ISBN
9780323831659
9780323831666
DOI
10.1016/B978-0-323-83165-9.00031-5
language
English
LU publication?
yes
additional info
Publisher Copyright: © 2025 Elsevier Inc. All rights reserved.
id
810a3cf0-ef88-414a-a7c3-b1e7e624e088
date added to LUP
2026-02-05 14:43:44
date last changed
2026-06-26 08:46:19
@inbook{810a3cf0-ef88-414a-a7c3-b1e7e624e088,
  abstract     = {{<p>This chapter summarizes current knowledge and diagnostic criteria for T-cell prolymphocytic leukemia (T-PLL). T-PLL is an aggressive disease characterized by a proliferation of small to medium-sized lymphocytes with a postthymic phenotype usually involving the blood, bone marrow, lymph nodes, spleen, and skin. The leukemic cells are usually positive for CD2, CD3 CD5, CD7, CD43, CD52, and CD26, but CD4 and CD8 are variably expressed. T-PLL is diagnosed by the presence of three major criteria (&gt;5 × 109/L cells of T-PLL phenotype in peripheral blood or bone marrow, T-cell clonality, abnormalities of 14q32 or Xq28 OR expression of TCL1A/B or MTCP1) or two major criteria and one of the minor criteria: abnormalities involving chromosome 11 (11q22.3, ATM); abnormalities in chromosome 8, including idic(8)(p11), t(8;8), and trisomy 8q; abnormalities in chromosomes 5, 12, 13, or 22 or in complex karyotype; or involvement of T-PLL-specific site (e.g., splenomegaly, effusions).</p>}},
  author       = {{Porwit, Anna}},
  booktitle    = {{Hematopathology, 3rd Edition}},
  isbn         = {{9780323831659}},
  keywords     = {{bone marrow biopsy; cytology; immunophenotyping; leukemia; prolymphocytic; T-cell}},
  language     = {{eng}},
  month        = {{01}},
  pages        = {{3--662}},
  publisher    = {{Elsevier}},
  title        = {{T-Cell Prolymphocytic Leukemia}},
  url          = {{http://dx.doi.org/10.1016/B978-0-323-83165-9.00031-5}},
  doi          = {{10.1016/B978-0-323-83165-9.00031-5}},
  year         = {{2024}},
}