PMID- 10552961
OWN - NLM
STAT- MEDLINE
DCOM- 19991130
LR  - 20220311
IS  - 0006-4971 (Print)
IS  - 0006-4971 (Linking)
VI  - 94
IP  - 10
DP  - 1999 Nov 15
TI  - Lymphomas expressing ALK fusion protein(s) other than NPM-ALK.
PG  - 3509-15
AB  - The tumor cells in ALK-positive lymphoma ("ALKoma") usually express the product 
      of the NPM-ALK chimeric gene, generated by the t(2;5) chromosomal translocation. 
      However, 10% to 20% of ALK-positive lymphomas express ALK fusion protein(s) other 
      than NPM-ALK, and in this report, we describe the immunohistologic and 
      clinicopathologic features of 15 such cases. The absence of the NPM-ALK fusion 
      gene was confirmed by reverse transcriptase-polymerase chain reaction (RT-PCR) in 
      8 cases and by fluorescence in situ hybridization (FISH) analysis in a further 2 
      cases. In each case, ALK staining was restricted to the cytoplasm and the 
      N-terminus of NPM to the nucleus (contrasting with lymphomas expressing NPM-ALK 
      in which cytoplasmic as well as nuclear labeling is seen). However, in the course 
      of screening 53 ALK-positive lymphomas, 2 biopsies were found that had a 
      "cytoplasm-only" ALK staining pattern but that nevertheless were shown to carry 
      the (2;5) (by NPM staining and RT-PCR). The 15 cases resembled typical 
      NPM-ALK-positive lymphomas in that all were of T or null phenotype, usually 
      occurred in young male patients, and frequently presented with advanced disease 
      associated with systemic symptoms and extranodal involvement. Moreover, their 
      prognosis was excellent and indistinguishable from that of classical 
      t(2;5)-positive tumors, but was clearly different from that of ALK-negative 
      anaplastic large-cell lymphomas. These results suggest that lymphomas carrying 
      variants of the NPM-ALK fusion protein can be detected by immunostaining for ALK 
      and NPM and also that they can be grouped with classical t(2;5)-positive tumors 
      as a single entity (ALK-positive lymphoma or "ALKoma") that shows a better 
      prognosis than ALK-negative anaplastic large-cell lymphoma.
FAU - Falini, B
AU  - Falini B
AD  - Institute of Hematology, University of Perugia, Perugia, Italy.
FAU - Pulford, K
AU  - Pulford K
FAU - Pucciarini, A
AU  - Pucciarini A
FAU - Carbone, A
AU  - Carbone A
FAU - De Wolf-Peeters, C
AU  - De Wolf-Peeters C
FAU - Cordell, J
AU  - Cordell J
FAU - Fizzotti, M
AU  - Fizzotti M
FAU - Santucci, A
AU  - Santucci A
FAU - Pelicci, P G
AU  - Pelicci PG
FAU - Pileri, S
AU  - Pileri S
FAU - Campo, E
AU  - Campo E
FAU - Ott, G
AU  - Ott G
FAU - Delsol, G
AU  - Delsol G
FAU - Mason, D Y
AU  - Mason DY
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - United States
TA  - Blood
JT  - Blood
JID - 7603509
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  - Adolescent
MH  - Anaplastic Lymphoma Kinase
MH  - Humans
MH  - In Situ Hybridization, Fluorescence
MH  - Lymphoma/*enzymology/genetics/metabolism
MH  - Male
MH  - Protein-Tyrosine Kinases/*biosynthesis/genetics/*metabolism
MH  - Receptor Protein-Tyrosine Kinases
MH  - Reverse Transcriptase Polymerase Chain Reaction
MH  - Translocation, Genetic
EDAT- 1999/11/24 00:00
MHDA- 1999/11/24 00:01
CRDT- 1999/11/24 00:00
PHST- 1999/11/24 00:00 [pubmed]
PHST- 1999/11/24 00:01 [medline]
PHST- 1999/11/24 00:00 [entrez]
AID - S0006-4971(20)71022-0 [pii]
PST - ppublish
SO  - Blood. 1999 Nov 15;94(10):3509-15.