PMID- 9684923
OWN - NLM
STAT- MEDLINE
DCOM- 19980922
LR  - 20220310
IS  - 1042-8194 (Print)
IS  - 1026-8022 (Linking)
VI  - 29
IP  - 3-4
DP  - 1998 Apr
TI  - The t(2;5) in human lymphomas.
PG  - 249-56
AB  - A recurrent, reciprocal balanced translocation, t(2;5) (p23;q35), has been 
      recognized in CD30+ anaplastic large-cell lymphomas (ALCL), a newly recognized 
      subtype comprising approximately 5% of all non-Hodgkin's lymphoma (NHL). This 
      translocation creates a novel fusion protein, NPM-ALK, which has transforming 
      properties in vitro and can cause large-cell lymphoma in vivo when transfected 
      into murine bone marrow. Multiple techniques including reverse 
      transcriptase-polymerase chain reaction (RT-PCR) amplification of NPM-ALK fusion 
      transcripts, genomic DNA-PCR, RNA in situ hybridization, and fluorescence in situ 
      hybridization (FISH) of metaphase chromosomes and interphase nuclei, and 
      immunohistochemical detection of the 80 kilodalton protein (p80) derived from the 
      NPM-ALK fusion have enabled surveys of normal and lymphoma tissues for evidence 
      of the translocation. These studies suggest that expression of ALK protein, a 
      novel orphan receptor tyrosine kinase, is normally confined to the nervous 
      system. In lymphoma, NPM-ALK expression is most often seen in young patients with 
      the monomorphic or small-cell variant of ALCL who present with advanced stage 
      disease and have tumors with a CD30+, T- or null-cell phenotype. It is less 
      frequently detected in older patients and in ALCL of pleomorphic histology. In 
      addition, expression of NPM-ALK has been found in occasional CD30 negative B-cell 
      lymphomas with diffuse large cell or immunoblastic histology. NPM-ALK is rarely, 
      if ever, detected in Hodgkin's disease or secondary ALCL. Although initially 
      found in primary nodal ALCL, recent studies suggest that NPM-ALK expression may 
      occur in lymphoma at extranodal sites, including the skin; it remains 
      controversial, however, whether CD30+ primary cutaneous lymphoma and its benign 
      counterpart, lymphomatoid papulosis (LyP), express NPM-ALK in some cases. A 
      retrospective study has suggested that expression of NPM-ALK is associated with a 
      better overall 5-year survival; these results must be confirmed in prospective 
      studies of patients with uniform staging and therapy to more fully understand the 
      clinical significance of the t(2;5) and its novel chimeric protein, NPM-ALK.
FAU - Kadin, M E
AU  - Kadin ME
AD  - Department of Pathology, Beth Israel Hospital and Harvard Medical School, Boston, 
      MA 02115, USA. mkadin@bidmc.harvard.edu
FAU - Morris, S W
AU  - Morris SW
LA  - eng
GR  - CA 01702/CA/NCI NIH HHS/United States
GR  - CA 69129/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PT  - Review
PL  - United States
TA  - Leuk Lymphoma
JT  - Leukemia & lymphoma
JID - 9007422
RN  - 0 (Neoplasm Proteins)
RN  - EC 2.7.1.- (p80(NPM-ALK) protein)
RN  - EC 2.7.10.1 (ALK protein, human)
RN  - EC 2.7.10.1 (Anaplastic Lymphoma Kinase)
RN  - EC 2.7.10.1 (Protein-Tyrosine Kinases)
RN  - EC 2.7.10.1 (Receptor Protein-Tyrosine Kinases)
SB  - IM
MH  - Anaplastic Lymphoma Kinase
MH  - Chromosomes, Human, Pair 2/*genetics
MH  - Chromosomes, Human, Pair 5/*genetics
MH  - Humans
MH  - Lymphoma, Large-Cell, Anaplastic/*genetics/metabolism
MH  - Neoplasm Proteins/*genetics
MH  - Protein-Tyrosine Kinases/*genetics/metabolism
MH  - Receptor Protein-Tyrosine Kinases
MH  - Skin Neoplasms/genetics/metabolism
MH  - Translocation, Genetic/*genetics
RF  - 52
EDAT- 1998/07/31 00:00
MHDA- 1998/07/31 00:01
CRDT- 1998/07/31 00:00
PHST- 1998/07/31 00:00 [pubmed]
PHST- 1998/07/31 00:01 [medline]
PHST- 1998/07/31 00:00 [entrez]
AID - 10.3109/10428199809068562 [doi]
PST - ppublish
SO  - Leuk Lymphoma. 1998 Apr;29(3-4):249-56. doi: 10.3109/10428199809068562.