| Disease | ALL, L1/L2 type; exceptionally found in L3-like ALL, T-ALL, LMNH, or ANLL |
| Phenotype / cell stem origin | 'pre B' (cIg+) ALL; may be cIg- or sIg+ |
| Epidemiology | 5% of ALL, or 20% of pre B ALL; found in children and young adults (1-60 yrs, median: 10 yrs --> one of the most frequent ALL in childhood); 3 male/4 female patients |
| Clinics | moderate organomegaly; frequent CNS involvement; blood data: high WBC (median 20 X 109/l); high LDH |
| Cytology | CD19+, CD10+, and also CD9+ |
| Prognosis | chromosome anomaly associated with adverse prognostic features; CR in most cases; median event free survival: 2 yrs; no age (?) or blood data prognostic significance; according to some -but not other- authors, the unbalanced form is of better prognosis (5 yr survival = 70%); median survival: 4 yrs in children, 6 mths in adults in one study, > 3yrs in adults in another |
| Cytogenetics Morphological | breakpoint is in 19p13.3; two different forms (with different prognoses?) (see diagrams above): - the balanced t(1;19), one fourth of cases, with a der(1) and a der(19); - the unbalanced form, found in 3/4 cases, with 2 normal chromosomes 1, a der(19), and 1 normal chromosome 19: --> partial trisomy for 1q23-1qter and monosomy for 19p13.3-pter; the 2 forms can be in mosaic; note: 19p13 and 19q13 may be confused (e.g. literature reports) |
| Additional anomalies | in half cases; partial dup (1q), +6, del(6q), +8, i(9q), +17, i(17q), +21 t(17;19)(q22;p13) is not stricto sensu a variant, but, so far, an equivalent, with HLF (hepatic heukemia factor), on 17q22, involved in the translocation. |
| Prognostic significance of the balanced t(1;19) and unbalanced der(19)t(1;19) translocations in acute lymphoblastic leukemia. |
| Secker-Walker LM, Berger R, Fenaux P, Lai JL, Nelken B, Garson M, Michael PM, Hagemeijer A, Harrison CJ, Kaneko Y |
| Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K. 1992 ; 6 (5) : 363-369. |
| PMID 1593901 |
| |
| Chromosomal translocations involving the E2A gene in acute lymphoblastic leukemia: clinical features and molecular pathogenesis. |
| Hunger SP |
| Blood. 1996 ; 87 (4) : 1211-1224. |
| PMID 8608207 |
| |
| Immunologic, cytogenetic, and clinical characterization of childhood acute lymphoblastic leukemia with the t(1;19) (q23; p13) or its derivative. |
| Pui CH, Raimondi SC, Hancock ML, Rivera GK, Ribeiro RC, Mahmoud HH, Sandlund JT, Crist WM, Behm FG |
| Journal of clinical oncology : official journal of the American Society of Clinical Oncology. 1994 ; 12 (12) : 2601-2606. |
| PMID 7989935 |
| |
| Heterogeneity of t(1;19)(q23;p13) acute leukaemias. French Haematological Cytology Group. |
| Troussard X, Rimokh R, Valensi F, Leboeuf D, Fenneteau O, Guitard AM, Manel AM, Schillinger F, Leglise C, Brizard A |
| British journal of haematology. 1995 ; 89 (3) : 516-526. |
| PMID 7734349 |
| |
| Fusion with E2A converts the Pbx1 homeodomain protein into a constitutive transcriptional activator in human leukemias carrying the t(1;19) translocation. |
| Lu Q, Wright DD, Kamps MP |
| Molecular and cellular biology. 1994 ; 14 (6) : 3938-3948. |
| PMID 7910944 |
| |
| PBX2 and PBX3, new homeobox genes with extensive homology to the human proto-oncogene PBX1. |
| Monica K, Galili N, Nourse J, Saltman D, Cleary ML |
| Molecular and cellular biology. 1991 ; 11 (12) : 6149-6157. |
| PMID 1682799 |
| |
| Chromosomal translocation t(1;19) results in synthesis of a homeobox fusion mRNA that codes for a potential chimeric transcription factor. |
| Nourse J, Mellentin JD, Galili N, Wilkinson J, Stanbridge E, Smith SD, Cleary ML |
| Cell. 1990 ; 60 (4) : 535-545. |
| PMID 1967982 |
| |
| A new homeobox gene contributes the DNA binding domain of the t(1;19) translocation protein in pre-B ALL. |
| Kamps MP, Murre C, Sun XH, Baltimore D |
| Cell. 1990 ; 60 (4) : 547-555. |
| PMID 1967983 |
| |
| Molecular analysis of the t(1;19) breakpoint cluster region in pre-B cell acute lymphoblastic leukemias. |
| Mellentin JD, Nourse J, Hunger SP, Smith SD, Cleary ML |
| Genes, chromosomes & cancer. 1990 ; 2 (3) : 239-247. |
| PMID 2078515 |
| |
