|
|
Previous Article | Table of Contents | Next Article 
Clinical and molecular characterization of a rare syndrome of acute
promyelocytic leukemia associated with translocation (11;17)
JD Licht, C Chomienne, A Goy, A Chen, AA Scott, DR Head, JL Michaux, Y Wu, A DeBlasio and WH Miller
Division of Molecular Medicine, Mount Sinai Medical Center, New York, NY
10029.
Analysis of a variant translocation t(11;17) in a case of acute
promyelocytic leukemia (APL) led to discovery of a novel zinc finger gene,
PLZF, fused to the retinoic acid receptor-alpha (RAR alpha) gene. We
reviewed the clinical and molecular features of five additional patients
with t(11;17)-associated APL. The clinical course of three patients was
characterized by early death and three experienced disseminated
intravascular coagulation. Morphologically all of the patients fell in a
unusual morphologic spectrum of APL, with features intermediate between M2
and M3 AML. All six patients had PLZF-RAR alpha gene fusion as detected by
reverse transcription/polymerase chain reaction assay, Southern blotting,
or pulsed-field gel electrophoresis. Five of the six patients failed to
achieve complete remission after initial chemotherapy or differentiation
therapy with all-trans retinoic acid (ATRA). A sixth patient responded to
initial chemotherapy, but on relapse failed to respond to ATRA. When tested
in vitro, cultured cells from three of the patients failed to differentiate
in response to ATRA. APL associated with t(11;17) and fusion of the PLZF
and RAR alpha genes is a discrete clinico-pathologic syndrome with a
distinctly worse prognosis than t(15;17) APL.
Volume 85,
Issue 4,
pp. 1083-1094,
02/15/1995
Copyright © 1995 by The American Society of Hematology

CiteULike Connotea Del.icio.us Digg Reddit Technorati What's this?
This article has been cited by other articles:

|
 |

|
 |
 
H.-Y. Wang, J. Ding, M. A. Vasef, and K. S. Wilson
A bcr3/Short Form PML-RAR{alpha} Transcript in an Acute Promyelocytic Leukemia Resulted From a Derivative Chromosome 17 Due to Submicroscopic Insertion of the PML Gene Into the RAR{alpha} Locus
Am J Clin Pathol,
January 1, 2009;
131(1):
64 - 71.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
Z.-Y. Wang and Z. Chen
Acute promyelocytic leukemia: from highly fatal to highly curable
Blood,
March 1, 2008;
111(5):
2505 - 2515.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
F. Guidez, S. Parks, H. Wong, J. V. Jovanovic, A. Mays, A. F. Gilkes, K. I. Mills, M.-C. Guillemin, R. M. Hobbs, P. P. Pandolfi, et al.
RAR{alpha}-PLZF overcomes PLZF-mediated repression of CRABPI, contributing to retinoid resistance in t(11;17) acute promyelocytic leukemia
PNAS,
November 20, 2007;
104(47):
18694 - 18699.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. Heuser, B. Argiropoulos, F. Kuchenbauer, E. Yung, J. Piper, S. Fung, R. F. Schlenk, K. Dohner, T. Hinrichsen, C. Rudolph, et al.
MN1 overexpression induces acute myeloid leukemia in mice and predicts ATRA resistance in patients with AML
Blood,
September 1, 2007;
110(5):
1639 - 1647.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
X. Zheng, A. Seshire, B. Ruster, G. Bug, T. Beissert, E. Puccetti, D. Hoelzer, R. Henschler, and M. Ruthardt
Arsenic but not all-trans retinoic acid overcomes the aberrant stem cell capacity of PML/RAR{alpha}-positive leukemic stem cells
Haematologica,
March 1, 2007;
92(3):
323 - 331.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
S. S. Farag, A. S. Ruppert, K. Mrozek, R. J. Mayer, R. M. Stone, A. J. Carroll, B. L. Powell, J. O. Moore, M. J. Pettenati, P. R.K. Koduru, et al.
Outcome of Induction and Postremission Therapy in Younger Adults With Acute Myeloid Leukemia With Normal Karyotype: A Cancer and Leukemia Group B Study
J. Clin. Oncol.,
January 20, 2005;
23(3):
482 - 493.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
Y. Jing, L. Xia, and S. Waxman
Targeted removal of PML-RARalpha protein is required prior to inhibition of histone deacetylase for overcoming all-trans retinoic acid differentiation resistance in acute promyelocytic leukemia
Blood,
July 18, 2002;
100(3):
1008 - 1013.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. C. Petti, F. Fazi, M. Gentile, D. Diverio, P. De Fabritiis, M. S. De Propris, R. Fiorini, M. A. A. Spiriti, F. Padula, P. G. Pelicci, et al.
Complete remission through blast cell differentiation in PLZF/RARalpha -positive acute promyelocytic leukemia: in vitro and in vivo studies
Blood,
July 18, 2002;
100(3):
1065 - 1067.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
V. Bertagnolo, M. Marchisio, S. Pierpaoli, M. L. Colamussi, F. Brugnoli, G. Visani, G. Zauli, and S. Capitani
Selective up-regulation of phospholipase C-{beta}2 during granulocytic differentiation of normal and leukemic hematopoietic progenitors
J. Leukoc. Biol.,
June 1, 2002;
71(6):
957 - 965.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. Melnick, G. Carlile, K. F. Ahmad, C.-L. Kiang, C. Corcoran, V. Bardwell, G. G. Prive, and J. D. Licht
Critical Residues within the BTB Domain of PLZF and Bcl-6 Modulate Interaction with Corepressors
Mol. Cell. Biol.,
March 15, 2002;
22(6):
1804 - 1818.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
B. Falini and D. Y. Mason
Proteins encoded by genes involved in chromosomal alterations in lymphoma and leukemia: clinical value of their detection by immunocytochemistry
Blood,
January 15, 2002;
99(2):
409 - 426.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
P. P. Pandolfi
Oncogenes and tumor suppressors in the molecular pathogenesis of acute promyelocytic leukemia
Hum. Mol. Genet.,
April 1, 2001;
10(7):
769 - 775.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. A Johnson
Chromatin modification and disease
J. Med. Genet.,
December 1, 2000;
37(12):
905 - 915.
[Full Text]
|
 |
|

|
 |

|
 |
 
A. Melnick, G. W. Carlile, M. J. McConnell, A. Polinger, S. W. Hiebert, and J. D. Licht
AML-1/ETO fusion protein is a dominant negative inhibitor of transcriptional repression by the promyelocytic leukemia zinc finger protein
Blood,
December 1, 2000;
96(12):
3939 - 3947.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
E. M. Rego, L.-Z. He, R. P. Warrell Jr., Z.-G. Wang, and P. P. Pandolfi
Retinoic acid (RA) and As2O3 treatment in transgenic models of acute promyelocytic leukemia (APL) unravel the distinct nature of the leukemogenic process induced by the PML-RARalpha and PLZF-RARalpha oncoproteins
PNAS,
August 17, 2000;
(2000)
180290497.
[Abstract]
[Full Text]
|
 |
|

|
 |

|
 |
 
D. Sainty, V. Liso, A. Cantu-Rajnoldi, D. Head, M.-J. Mozziconacci, C. Arnoulet, L. Benattar, S. Fenu, M. Mancini, E. Duchayne, et al.
A new morphologic classification system for acute promyelocytic leukemia distinguishes cases with underlying PLZF/RARA gene rearrangements
Blood,
August 15, 2000;
96(4):
1287 - 1296.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
D. Grimwade, A. Biondi, M.-J. Mozziconacci, A. Hagemeijer, R. Berger, M. Neat, K. Howe, N. Dastugue, J. Jansen, I. Radford-Weiss, et al.
Characterization of acute promyelocytic leukemia cases lacking the classic t(15;17): results of the European Working Party
Blood,
August 15, 2000;
96(4):
1297 - 1308.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
L. A. Hansen, C. C. Sigman, F. Andreola, S. A. Ross, G. J. Kelloff, and L. M. De Luca
Retinoids in chemoprevention and differentiation therapy
Carcinogenesis,
July 1, 2000;
21(7):
1271 - 1279.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. L. Redner, J. D. Chen, E. A. Rush, H. Li, and S. L. Pollock
The t(5;17) acute promyelocytic leukemia fusion protein NPM-RAR interacts with co-repressor and co-activator proteins and exhibits both positive and negative transcriptional properties
Blood,
April 15, 2000;
95(8):
2683 - 2690.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. M. Melnick, J. J. Westendorf, A. Polinger, G. W. Carlile, S. Arai, H. J. Ball, B. Lutterbach, S. W. Hiebert, and J. D. Licht
The ETO Protein Disrupted in t(8;21)-Associated Acute Myeloid Leukemia Is a Corepressor for the Promyelocytic Leukemia Zinc Finger Protein
Mol. Cell. Biol.,
March 15, 2000;
20(6):
2075 - 2086.
[Abstract]
[Full Text]
|
 |
|

|
 |

|
 |
 
S. C. Kogan, S.-h. Hong, D. B. Shultz, M. L. Privalsky, and J. M. Bishop
Leukemia initiated by PMLRARalpha : the PML domain plays a critical role while retinoic acid-mediated transactivation is dispensable
Blood,
March 1, 2000;
95(5):
1541 - 1550.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. E. Hoatlin, Y. Zhi, H. Ball, K. Silvey, A. Melnick, S. Stone, S. Arai, N. Hawe, G. Owen, A. Zelent, et al.
A Novel BTB/POZ Transcriptional Repressor Protein Interacts With the Fanconi Anemia Group C Protein and PLZF
Blood,
December 1, 1999;
94(11):
3737 - 3747.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
X. Li, H. Peng, D. C. Schultz, J. M. Lopez-Guisa, F. J. Rauscher III, and R. Marmorstein
Structure-Function Studies of the BTB/POZ Transcriptional Repression Domain from the Promyelocytic Leukemia Zinc Finger Oncoprotein
Cancer Res.,
October 1, 1999;
59(20):
5275 - 5282.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
T. Zhang, H. Xiong, L.-X. Kan, C.-K. Zhang, X.-F. Jiao, G. Fu, Q.-H. Zhang, L. Lu, J.-H. Tong, B.-W. Gu, et al.
Genomic sequence, structural organization, molecular evolution, and aberrant rearrangement of promyelocytic leukemia zinc finger gene
PNAS,
September 28, 1999;
96(20):
11422 - 11427.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. L. Redner, J. Wang, and J. M. Liu
Chromatin Remodeling and Leukemia: New Therapeutic Paradigms
Blood,
July 15, 1999;
94(2):
417 - 428.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
T. Sternsdorf, E. Puccetti, K. Jensen, D. Hoelzer, H. Will, O. G. Ottmann, and M. Ruthardt
PIC-1/SUMO-1-Modified PML-Retinoic Acid Receptor alpha Mediates Arsenic Trioxide-Induced Apoptosis in Acute Promyelocytic Leukemia
Mol. Cell. Biol.,
July 1, 1999;
19(7):
5170 - 5178.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
F. L. Coco, D. Diverio, B. Falini, A. Biondi, C. Nervi, and P. G. Pelicci
Genetic Diagnosis and Molecular Monitoring in the Management of Acute Promyelocytic Leukemia
Blood,
July 1, 1999;
94(1):
12 - 22.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J.H. Jansen, M.C. de Ridder, W.M.C. Geertsma, C.A.J. Erpelinck, K. van Lom, E.M.E. Smit, R. Slater, B.A. v. Reijden, G.E. de Greef, P. Sonneveld, et al.
Complete Remission of t(11;17) Positive Acute Promyelocytic Leukemia Induced by All-trans Retinoic Acid and Granulocyte Colony-Stimulating Factor
Blood,
July 1, 1999;
94(1):
39 - 45.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
A. Melnick and J. D. Licht
Deconstructing a Disease: RAR{alpha}, Its Fusion Partners, and Their Roles in the Pathogenesis of Acute Promyelocytic Leukemia
Blood,
May 15, 1999;
93(10):
3167 - 3215.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R. Shaknovich, P. L. Yeyati, S. Ivins, A. Melnick, C. Lempert, S. Waxman, A. Zelent, and J. D. Licht
The Promyelocytic Leukemia Zinc Finger Protein Affects Myeloid Cell Growth, Differentiation, and Apoptosis
Mol. Cell. Biol.,
September 1, 1998;
18(9):
5533 - 5545.
[Abstract]
[Full Text]
|
 |
|

|
 |

|
 |
 
N. Pallisgaard, P. Hokland, D. C. Riishoj, B. Pedersen, and P. Jorgensen
Multiplex Reverse Transcription-Polymerase Chain Reaction for Simultaneous Screening of 29 Translocations and Chromosomal Aberrations in Acute Leukemia
Blood,
July 15, 1998;
92(2):
574 - 588.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J. L. Slack and M. Yu
Constitutive Expression of the Promyelocytic Leukemia-Associated Oncogene PML-RARalpha in TF1 Cells: Isoform-Specific and Retinoic Acid-Dependent Effects on Growth, bcl-2 Expression, and Apoptosis
Blood,
May 1, 1998;
91(9):
3347 - 3356.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
F. Guidez, S. Ivins, J. Zhu, M. Soderstrom, S. Waxman, and A. Zelent
Reduced Retinoic Acid-Sensitivities of Nuclear Receptor Corepressor Binding to PML- and PLZF-RARalpha Underlie Molecular Pathogenesis and Treatment of Acute Promyelocytic Leukemia
Blood,
April 15, 1998;
91(8):
2634 - 2642.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
R.J. LIN, H.-Y. KAO, P. ORDENTLICH, and R.M. EVANS
The Transcriptional Basis of Steroid Physiology
Cold Spring Harb Symp Quant Biol,
January 1, 1998;
63(0):
577 - 586.
[Abstract]
[PDF]
|
 |
|

|
 |

|
 |
 
D. Grimwade, P. Gorman, E. Duprez, K. Howe, S. Langabeer, F. Oliver, H. Walker, D. Culligan, J. Waters, M. Pomfret, et al.
Characterization of Cryptic Rearrangements and Variant Translocations in Acute Promyelocytic Leukemia
Blood,
December 15, 1997;
90(12):
4876 - 4885.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
K. Mrozek, K. Heinonen, D. Lawrence, A. J. Carroll, P. R.K. Koduru, K. W. Rao, M. P. Strout, R. E. Hutchison, J. O. Moore, R. J. Mayer, et al.
Adult Patients With De Novo Acute Myeloid Leukemia and t(9; 11)(p22; q23) Have a Superior Outcome to Patients With Other Translocations Involving Band 11q23: A Cancer and Leukemia Group B Study
Blood,
December 1, 1997;
90(11):
4532 - 4538.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
X. Li, J. M. Lopez-Guisa, N. Ninan, E. J. Weiner, F. J. Rauscher III, and R. Marmorstein
Overexpression, Purification, Characterization, and Crystallization of the BTB/POZ Domain from the PLZF Oncoprotein
J. Biol. Chem.,
October 24, 1997;
272(43):
27324 - 27329.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
C. D. Jennings and K. A. Foon
Recent Advances in Flow Cytometry: Application to the Diagnosis of Hematologic Malignancy
Blood,
October 15, 1997;
90(8):
2863 - 2892.
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
M. H. M. Koken, A. Reid, F. Quignon, M. K. Chelbi-Alix, J. M. Davies, J. H. S. Kabarowski, J. Zhu, S. Dong, S.-J. Chen, Z. Chen, et al.
Leukemia-associated retinoic acid receptor alpha fusion partners, PML and PLZF, heterodimerize and colocalize to nuclear bodies
PNAS,
September 16, 1997;
94(19):
10255 - 10260.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
J.-Y. Li, M. A. English, H. J. Ball, P. L. Yeyati, S. Waxman, and J. D. Licht
Sequence-specific DNA Binding and Transcriptional Regulation by the Promyelocytic Leukemia Zinc Finger Protein
J. Biol. Chem.,
September 5, 1997;
272(36):
22447 - 22455.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
F. Mandelli, D. Diverio, G. Avvisati, A. Luciano, T. Barbui, C. Bernasconi, G. Broccia, R. Cerri, M. Falda, G. Fioritoni, et al.
Molecular Remission in PML/RARalpha -Positive Acute Promyelocytic Leukemia by Combined All-trans Retinoic Acid and Idarubicin (AIDA) Therapy
Blood,
August 1, 1997;
90(3):
1014 - 1021.
[Abstract]
[Full Text]
[PDF]
|
 |
|

|
 |

|
 |
 
L. Benedetti, A. A. Levin, B. M. Scicchitano, F. Grignani, G. | |