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T-cell prolymphocytic leukemia (TPLL)
Etiology:
- occurs in younger patients with ataxia telangiectasia
Epidemiology:
- occurs both in adults as a sporadic disease & in younger patients with ataxia telangiectasia
Pathology:
- aggressive T cell leukemia
- small to medium sized prolymphocytes with mature post-thymic T cell phenotype
Classification: REAL classification:
- T-cell chronic lymphocytic leukemia/prolymphocytic leukemia
Immunophenotype:
- Tdt -, CD1a -
- CD2 +, CD3 +, CD7 +
- CD4 + & CD8 - in ~60%
- CD4 + & CD8 + in ~25%
- CD4 - & CD8 + in ~15%
Genetics:
1) T cell receptor gamma & beta chains clonally rearranged
2) rearrangements of chromosome 14q32.1 region involving TCL1B
3) MTCP1 overexpressed with t(X;14) translocation
4) associated with defects in ATM gene
Clinical manifestations:
- marked splenomegaly
- lymphadenopathy
- skin lesions
- serous effusion Laboratgory:
- high white blood cell count, predominance of prolymphocytes
Management:
- poor response to chemotherapy
- short survival time
Interactions
disease interactions
General
chronic lymphocytic leukemia (CLL)
peripheral T-cell lymphoid neoplasm
prolymphocytic leukemia
small lymphocytic lymphoma
References
- WHO Classification Tumours of Haematopoietic and Lymphoid
Tissues. IARC Press 2001
- Shi M, Jevremovic D
Lymphoma and plasma cell neoplasms
T / NK cell disorders. T cell prolymphocytic leukemia
http://www.pathologyoutlines.com/topic/lymphomanonBTcellpro.html