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Cited by 85Myeloproliferative Neoplasms: Diagnosis and TreatmentAcute Myeloid Leukemia ResearchHematological disorders and diagnostics

Leukemic transformation of polycythemia vera: A single center study of 23 patients

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Passamonti, Francesco, Rumi, Elisa, Arcaini, Luca, Castagnola, Carlo, Lunghi, Monia, Bernasconi, Paolo, Giovanni Della Porta, Matteo, Columbo, Nora +3 more·2005·Cancer
DOI 10.1002/cncr.21297
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Abstract

BACKGROUND: Acute leukemia (AL) may occur as rare and late event of polycythemia vera (PV). METHODS: The current study included 23 patients who developed acute leukemia in a cohort of 414 consecutive PV patients with long‐term observation (3208 person years of follow‐up). Kaplan–Meier Product‐Limit method was used to estimate the cumulative probability of survival; Gehan–Wilcoxon test was applied to compare survival in different groups of patients. RESULTS: Median age was 68 years, and 18 patients (78%) were > 60 years of age. At diagnosis of AL, most patients had a white blood count > 10 × 10 9/L (n = 17; 74%), Hgb < 10 g/dL (n = 13; 57%), and platelet count > 50 × 10 9/L (n = 17; 74%). Of 14 patients in whom cytogenetic analysis was available at leukemic transformation, 12 showed high‐risk abnormalities including complex karyotype (n = 10), del (7)(q22) sole (n = 1) and del (X)(q26) sole (n = 1), whereas 2 had a normal karyotype. In patients whose karyotype was available at diagnosis of PV, cytogenetic evolution was documented at progression to AL. Treatment consisted of supportive care and/or low‐dose chemotherapy (n = 15), or induction chemotherapy (n = 8). This included idarubicin plus cytarabine (n = 3), high‐dose cytarabine (n = 4), and fludarabine‐based regimen (n = 1). Allogenic stem cell transplantation was offered to a single patient, who is alive at Day + 70. The outcome of patients was poor, with a median survival of 2.9 months (range, 0.6–20.1 mos), with no significant differences between palliation and intensive treatments. CONCLUSIONS: AL following PV has distinct clinical and biologic features. Outcome of patients is poor irrespective of the treatment employed. Cancer 2005. © 2005 American Cancer Society.

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  • TP53 multihit HSPCs clustered separately from TP53-WT pre-LSCs in two distinct populations with enrichment of LSC and erythroid-associated transcription, respectively, and a differentiation trajectory toward the erythroid-biased population.

    “Integration of single-cell transcriptomes and diffusion map analysis of HSPCs from TP53-sAML patients showed that TP53 multihit HSPCs clustered separately from TP53-WT pre-LSCs in two distinct populations with enrichment of LSC and erythroid-associated transcription, respectively (Fig. 2a and Supplementary Table 3), and a differentiation trajectory toward the erythroid-biased population (Fig. 2b),”

    From Single-cell multi-omics identifies chronic inflammation as a driver of TP53-mutant leukemic evolution (2023) · cites this paper as [23]

    The Cytogenetic Findings and Discussion passages (page numbers unavailable) report karyotypes and clinical outcomes in patients with leukemia following polycythemia vera. Searches for TP53 multihit HSPCs, single-cell transcriptomes, diffusion maps, and an erythroid-biased differentiation trajectory found no passage addressing the claimed populations or trajectory. (LLM, conf=0.76)

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Research works printed but not linked: 28

  1. Vardiman JW, Harris NL, Brunning RD. The World Health Organization (WHO) classification of the myeloid neo- plasms. Blood. 2002;100:2292-2302.
  2. Cortes J, Kantarjian H. Beyond chronic myelogenous leuke- mia: potential role for imatinib in Philadelphia-negative myeloproliferative disorders. Cancer. 2004;100:2064 -2078.
  3. Tefferi A, Solberg LA, Silverstein MN. A clinical update in polycythemia vera and essential thrombocythemia. Am J Med. 2000;109:141-149.
  4. Passamonti F, Rumi E, Pungolino E, et al. Life expectancy and prognostic factors for survival in patients with polycy- themia vera and essential thrombocythemia. Am J Med. 2004;117:755-761.
  5. Passamonti F, Malabarba L, Orlandi E, et al. Polycythemia vera in young patients: a study on the long-term risk of thrombosis, myelofibrosis and leukemia. Haematologica. 2003;88:13-18.
  6. Finazzi G, Caruso V, Marchioli R, et al. Acute leukemia in polycythemia vera. An analysis of 1638 patients enrolled in a prospective observational study. Blood. 2005;105:2664 - 2670.
  7. Mesa RA, Li CY, Ketterling RP, et al. Leukemic transforma- tion in myelofibrosis with myeloid metaplasia: a single- institution experience with 91 cases. Blood. 2005;105:973- 977.
  8. Najean Y, Deschamps A, Dresch C, et al. Acute leukemia and myelodysplasia in polycythemia vera. A clinical study with long-term follow-up. Cancer. 1988;61:89 -95.
  9. Cervantes F, Tassies D, Salgado C, et al. Acute transforma- tion in nonleukemic chronic myeloproliferative disorders: actuarial probability and main characteristics in a series of 218 patients. Acta Haematol. 1991;85:124 -127.
  10. Merlat A, Lai JL, Sterkers Y, et al. Therapy-related myelodys- plastic syndrome and acute myeloid leukemia with 17p deletion. A report on 25 cases. Leukemia. 1999;13:250 -257.
  11. Weinfeld A, Westin J, Ridell B, Swolin B. Polycythaemia vera terminating in acute leukaemia. A clinical, cytogenetic and morphologic study in 8 patients treated with alkylating agents. Scand J Haematol. 1977;19:255-272.
  12. Berlin NI. Diagnosis and classification of the polycythemias. Semin Hematol. 1975;12:339 -351.
  13. Berk PD, Goldberg JD, Donovan PB, et al. Therapeutic rec- ommendations in polycythemia vera based on Polycythe- mia Vera Study Group protocols. Semin Hematol. 1986;23: 132-143.
  14. Pearson TC, Messinezy M. The diagnostic criteria of poly- cythaemia rubra vera. Leuk Lymphoma. 1996;22(Suppl 1): 87-93.
  15. Bennett JM, Catovsky D, Daniel MT, et al. Proposed revised criteria for the classification of acute myeloid leukemia. A report of the French-American-British Cooperative Group. Ann Intern Med. 1985;103:620 -625.
  16. Cheson BD, Cassileth PA, Head DR, et al. Report of the National Cancer Institute-sponsored workshop on defini- tions of diagnosis and response in acute myeloid leukemia. J Clin Oncol. 1990;8:813-819.
  17. Grimwade D, Walker H, Oliver F, et al. The importance of diagnostic cytogenetics on outcome in AML: analysis of 1612 patients entered into the MRC AML 10 trial. The Med- ical Research Council Adult and Children's Leukaemia Working Parties. Blood. 1998;92:2322-2333.
  18. Camos M, Cervantes F, Montoto S, et al. Acute lymphoid leukemia following polycythemia vera. Leuk Lymphoma. 1999;32:395-398.
  19. Andersen MK, Christiansen DH, Jensen BA, et al. Therapy- related acute lymphoblastic leukaemia with MLL rearrange- ments following DNA topoisomerase II inhibitors, an in- creasing problem: report on two new cases and review of the literature since 1992. Br J Haematol. 2001;114:539 -543.
  20. Lowenberg B, Downing JR, Burnett A. Acute myeloid leuke- mia. N Engl J Med. 1999;341:1051-1062.
  21. Leith CP, Kopecky KJ, Godwin J, et al. Acute myeloid leuke- mia in the elderly: assessment of multidrug resistance (MDR1) and cytogenetics distinguishes biologic subgroups with remarkably distinct responses to standard chemother- apy. A Southwest Oncology Group study. Blood. 1997;89: 3323-3329.
  22. Cytogenetics of acutely transformed chronic myeloprolifer- ative syndromes without a Philadelphia chromosome. A multicenter study of 55 patients. Groupe Francais de Cyto- genetique Hematologique. Cancer Genet Cytogenet. 1988;32: 157-168.
  23. Shamdas GJ, Spier CM, List AF. Myelodysplastic transforma- tion of polycythemia vera: case report and review of the literature. Am J Hematol. 1991;37:45-48.
  24. Pedersen-Bjergaard J, Andersen MK, Christiansen DH, Ner- lov C. Genetic pathways in therapy-related myelodysplasia and acute myeloid leukemia. Blood. 2002;99:1909 -1912.
  25. Sterkers Y, Preudhomme C, Lai JL, et al. Acute myeloid leukemia and myelodysplastic syndromes following essen- tial thrombocythemia treated with hydroxyurea: high pro- portion of cases with 17p deletion. Blood. 1998;91:616 -622.
  26. Song WJ, Sullivan MG, Legare RD, et al. Haploinsufficiency of CBFA2 causes familial thrombocytopenia with propensity to develop acute myelogenous leukaemia. Nat Genet. 1999; 23:166 -175.
  27. Jurado M, Deeg H, Gooley T, et al. Haemopoietic stem cell transplantation for advanced polycythaemia vera or essen- tial thrombocythaemia. Br J Haematol. 2001;112:392-396.
  28. Rondelli D, Barosi G, Bacigalupo A, et al. Allogeneic he- matopoietic stem-cell transplantation with reduced-in- tensity conditioning in intermediate-or high-risk patients with myelofibrosis with myeloid metaplasia. Blood. 2005; 105:4115-4119.

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Authors
Passamonti, F., Rumi, E., Arcaini, L., Castagnola, C., Lunghi, M., Bernasconi, P., Giovanni Della Porta, M., Columbo, N., Pascutto, C., Cazzola, M., & Lazzarino, M.
Affiliations
University of Pavia (IT)
Corresponding author
Passamonti, Francesco
ORCID
Venue
Cancer
Publisher
Wiley
Published
2005-07-26
Volume
104
Issue
5
Pages
1032-1036
ISSN
  • 0008-543X (ISSN-L)
  • 1097-0142
DOI
10.1002/cncr.21297
OpenAlex
W1603401075
Cited by
85
References
0
Citations by year
2026: 2 · 2025: 1 · 2024: 3 · 2023: 4 · 2022: 4 · 2020: 9 · 2019: 3 · 2018: 7 · 2017: 5 · 2016: 3 · 2015: 9 · 2013: 3 · 2012: 7
FWCI
0.89
Type
journal article
Language
en
Open access
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Cancer (publishedVersion)
  • PubMed (publishedVersion)
  • IrInSubria (University of Insubria) (submittedVersion)
Topics
  • Health Sciences › Medicine › Genetics › Myeloproliferative Neoplasms: Diagnosis and Treatment
  • Health Sciences › Medicine › Hematology › Acute Myeloid Leukemia Research
  • Health Sciences › Medicine › Emergency Medicine › Hematological disorders and diagnostics
Keywords
polycythemia vera, leukemic transformation, acute leukemia, complex karyotype, cytogenetic evolution, survival, induction chemotherapy, allogeneic stem cell transplantation
MeSH terms
Acute Disease, Aged, Chromosome Aberrations, Humans, Karyotyping, Leukemia, Middle Aged, Polycythemia Vera

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