T-cell prolymphocytic leukemia (TPLL)
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Classification
REAL classification:
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
Immunophenotype
Genetics
- T cell receptor gamma & beta chains clonally rearranged
- rearrangements of chromosome 14q32.1 region involving TCL1B
- MTCP1 overexpressed with t(X;14) translocation
- 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
More general terms
- peripheral T-cell lymphoid neoplasm
- prolymphocytic leukemia
- small lymphocytic lymphoma
- chronic lymphocytic leukemia (CLL)
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