PMID- 8656674 OWN - NLM STAT- MEDLINE DCOM- 19960726 LR - 20151119 IS - 0887-6924 (Print) IS - 0887-6924 (Linking) VI - 10 IP - 5 DP - 1996 May TI - Lineage involvement by BCR/ABL in Ph+ lymphoblastic leukemias: chronic myelogenous leukemia presenting in lymphoid blast vs Ph+ acute lymphoblastic leukemia. PG - 795-802 AB - Chronic myelogenous leukemia (CML) can sometimes present in lymphoid blast phase (L-BP), and can be difficult to distinguish from Philadelphia chromosome-positive (Ph+) acute lymphoblastic leukemia (ALL). Some have suggested that the determination of cell lineages involved by the Ph chromosome may be used for distinguishing CML presenting in L-BP (presumably multilineage disease) from Ph+ ALL (presumably lymphoid-restricted), although others have suggested the term 'stem cell ALL' for the multilineage process. Because it has been difficult to perform lineage studies of the Ph chromosome, we investigated the use of fluorescence in situ hybridization (FISH) with probes for BCR (on chromosome 22) and ABL (on chromosome 9) to study lineage involvement in Ph+ lymphoblastic malignancies. We analyzed routine blood and marrow specimens from eight patients who presented with Ph+ lymphoblastic leukemia and found that FISH recognized the 9;22 translocation, distinguished between the two common molecular variants, and readily identified multilineage vs lymphoblast-restricted disease. In our series, four patients had multilineage and four had lymphoblast-restricted disease. Multilineage disease was associated with morphologic features of CML at diagnosis and/or reversion to chronic phase CML after treatment leading us to consider it as CML presenting in L-BP. Patients with lymphoid-restricted disease lacked such findings. The survival of three of our four patients with multilineage disease was prolonged, at 25, 28+, and 126+ months, and when data from our entire series are added to those of 18 previously reported cases that were studied for lineage involvement (reviewed in Leukemia 1993; 7: 147), the difference in overall survival between patients with multilineage and lymphoblast-restricted disease is significant (median overall survival of 47 months vs 8 months, respectively; P=0.013, log rank). Our findings illustrate that FISH analysis can be used to recognize lineage involvement in patients presenting with Ph+ lymphoblastic malignancies, and they provide further support to the notion that multilineage and lymphoblast-restricted disease are distinct clinically as well as biologically. FAU - Anastasi, J AU - Anastasi J AD - Department of Pathology, University of Chicago, IL, USA. FAU - Feng, J AU - Feng J FAU - Dickstein, J I AU - Dickstein JI FAU - Le Beau, M M AU - Le Beau MM FAU - Rubin, C M AU - Rubin CM FAU - Larson, R A AU - Larson RA FAU - Rowley, J D AU - Rowley JD FAU - Vardiman, J W AU - Vardiman JW LA - eng GR - CA42557/CA/NCI NIH HHS/United States PT - Comparative Study PT - Journal Article PT - Research Support, Non-U.S. Gov't PT - Research Support, U.S. Gov't, Non-P.H.S. PT - Research Support, U.S. Gov't, P.H.S. PL - England TA - Leukemia JT - Leukemia JID - 8704895 RN - 0 (Biomarkers, Tumor) RN - 0 (Neoplasm Proteins) RN - EC 2.7.10.2 (Fusion Proteins, bcr-abl) SB - IM MH - Adolescent MH - Adult MH - Biomarkers, Tumor/*analysis MH - Blast Crisis/genetics/*pathology MH - Cell Lineage MH - Child MH - Child, Preschool MH - Female MH - Fusion Proteins, bcr-abl/*analysis MH - Humans MH - In Situ Hybridization, Fluorescence MH - Leukemia, Myelogenous, Chronic, BCR-ABL Positive/genetics/*pathology MH - Male MH - Neoplasm Proteins/*analysis MH - Neoplastic Stem Cells/metabolism/*pathology MH - *Philadelphia Chromosome MH - Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics/*pathology EDAT- 1996/05/01 00:00 MHDA- 1996/05/01 00:01 CRDT- 1996/05/01 00:00 PHST- 1996/05/01 00:00 [pubmed] PHST- 1996/05/01 00:01 [medline] PHST- 1996/05/01 00:00 [entrez] PST - ppublish SO - Leukemia. 1996 May;10(5):795-802.