Digitala Vetenskapliga Arkivet

Change search
CiteExportLink to record
Permanent link

Direct link
Cite
Citation style
  • apa
  • ieee
  • modern-language-association-8th-edition
  • vancouver
  • Other style
More styles
Language
  • de-DE
  • en-GB
  • en-US
  • fi-FI
  • nn-NO
  • nn-NB
  • sv-SE
  • Other locale
More languages
Output format
  • html
  • text
  • asciidoc
  • rtf
Survival in adult acute lymphoblastic leukaemia (ALL): A report from the Swedish ALL Registry
Uppsala University, Disciplinary Domain of Medicine and Pharmacy, Faculty of Medicine, Department of Medical Sciences, Haematology.ORCID iD: 0000-0002-1737-5568
Skane Univ Hosp, Dept Haematol Oncol & Radiophys, Lund, Sweden.
Univ Hosp Linkoping, Dept Haematol, Linkoping, Sweden.
Sahlgrens Univ Hosp, Sect Haematol & Coagulat, Dept Med, Gothenburg, Sweden.
Show others and affiliations
2019 (English)In: European Journal of Haematology, ISSN 0902-4441, E-ISSN 1600-0609, Vol. 103, no 2, p. 88-98Article in journal (Refereed) Published
Abstract [en]

Objectives: As new, effective therapies emerge for acute lymphoblastic leukaemia (ALL), the results of clinical trials need to relate to standard of care.

Methods: We used the population-based Swedish ALL Registry to evaluate characteristics, treatment and long-term outcome in 933 patients with diagnosis between 1997 and 2015.

Results: The median age was 53 years. The frequency of Philadelphia (Ph)-positive leukaemia was 34% of examined B-ALL with a peak incidence at 50-59 years. Five-year overall survival (OS) improved between 1997-2006 and 2007-2015; in patients 18-45 years from 50% (95% CI 43-57) to 65% (95% CI 58-72), 46-65 years from 25% (95% CI 18-32) to 46% (95% CI 37-55) and >65 years from 7% (95% CI 2.6-11) to 11% (95% CI 5.9-16) (P < 0.05). Men with Ph-neg B-ALL 46-65 years had inferior OS compared with women (P < 0.01). Standardised mortality ratio was 5.7 (95% CI 5.0-6.3) for patients who survived 5 years from diagnosis. In multivariable analysis, Ph-positive disease was not associated with impaired prognosis but with lower risk of death in 2007-2015.

Conclusions: In a population-based cohort, OS has improved in adult ALL, especially for Ph-positive disease but for middle-aged men with Ph-negative B-ALL outcome was poor. Cure without late toxicity or relapse is still desired.

Place, publisher, year, edition, pages
WILEY , 2019. Vol. 103, no 2, p. 88-98
Keywords [en]
acute lymphoblastic leukaemia, adult, Philadelphia-positive
National Category
Hematology
Identifiers
URN: urn:nbn:se:uu:diva-390495DOI: 10.1111/ejh.13247ISI: 000475475200003PubMedID: 31074910OAI: oai:DiVA.org:uu-390495DiVA, id: diva2:1341943
Available from: 2019-08-12 Created: 2019-08-12 Last updated: 2020-01-15Bibliographically approved
In thesis
1. Registry-Based Studies in Adult Acute Lymphoblastic Leukemia in Sweden: Survival and Quality of Life
Open this publication in new window or tab >>Registry-Based Studies in Adult Acute Lymphoblastic Leukemia in Sweden: Survival and Quality of Life
2020 (English)Doctoral thesis, comprehensive summary (Other academic)
Abstract [en]

Acute lymphoblastic leukemia (ALL), a common child malignancy, also constitutes a minor fraction of adult cancer with approximately 50 new cases per year in Sweden. While the five-year overall survival (OS) in pediatric ALL is more than 90%, the prognosis in adults is dismal. Using the Swedish ALL quality registry, this thesis investigates treatment and outcome of adult ALL according to national guidelines. In addition, the introduction of patient-reported outcome in the ALL and Acute Myeloid Leukemia registries is evaluated. 

In Paper I, measurement of minimal residual disease by flow cytometry was found to be feasible but not consistently applied in the 35 patients with Philadelphia (Ph)-negative B-ALL investigated. In Paper II, treatment, toxicity and outcome of 155 patients, 55-85 years (y) with ALL diagnosis between 2005 and 2012 were studied in detail by patient charts review. An age-adopted protocol recommended from 2009 did not result in better outcome. In Paper III, disease recurrence in the same cohort as Paper II was studied. The median overall survival (OS) after ALL relapse was 3.6 months. In Paper IV, the whole ALL registry was studied and OS was estimated in 930 adult patients diagnosed in the periods 1997-2006 and 2007-2015. Five year OS improved in patients 18-45y from 50% to 65%, in patients 46-65y from 25% to 46%, and in patients >65y from 7% to 11%. This demonstrates that young patients have the best prognosis, in part due to the introduction of a dose-intense “pediatric-like” chemotherapy protocol. Compared to women, middle-aged men were found to have a worse outcome.

Historically, Philadelphia-positive (Ph-pos) ALL has a poor prognosis compared to Ph-negative ALL. In this material, the frequency of Ph-pos ALL was 34% of examined B-ALL. Analysis of the whole registry revealed that in 2007-2015, i.e. after the introduction of the tyrosine kinase inhibitor imatinib, Ph-pos ALL was no longer associated with inferior OS. In Paper V, ALL and Acute Myeloid Leukemia patients, six months after diagnosis, completed a web or paper questionnaire regarding quality of life, symptoms and experience with care. The response rate was 64%. Depression symptoms were frequent (18%), especially in young women who reported worrying about fertility.

In summary, although OS in adult ALL has improved, more effective and less toxic therapies in upfront treatment are highly warranted. Collection of patient-reported outcome in a national quality registry is feasible and can add important aspects of cancer care that are not usually addressed.

Place, publisher, year, edition, pages
Uppsala: Acta Universitatis Upsaliensis, 2020. p. 69
Series
Digital Comprehensive Summaries of Uppsala Dissertations from the Faculty of Medicine, ISSN 1651-6206 ; 1630
Keywords
Acute Lymphoblastic Leukemia, Philadelphia Chromosome, Overall Survival, Population-based, Quality Registry, Relapse, Patient-Reported Outcome, Health-Related Quality of Life, Depression
National Category
Hematology
Identifiers
urn:nbn:se:uu:diva-401431 (URN)978-91-513-0853-1 (ISBN)
Public defence
2020-03-06, H:son-Holmdahlsalen, Akademiska sjukhuset, ing 100, Uppsala, 09:00 (English)
Opponent
Supervisors
Available from: 2020-02-13 Created: 2020-01-15 Last updated: 2020-02-13

Open Access in DiVA

fulltext(667 kB)134 downloads
File information
File name FULLTEXT01.pdfFile size 667 kBChecksum SHA-512
e4731a8c0fafd88a1bc7f6ae8023c2146c42f63bf2da42e2d6b33d0260f105694d93118c3cf1fcdd07ef8276528238602ff4de37f0241b467b8c60bab5cca7c8
Type fulltextMimetype application/pdf

Other links

Publisher's full textPubMed

Search in DiVA

By author/editor
Lennmyr, EmmaJoelsson, JoelHallböök, Helene
By organisation
Haematology
In the same journal
European Journal of Haematology
Hematology

Search outside of DiVA

GoogleGoogle Scholar
Total: 134 downloads
The number of downloads is the sum of all downloads of full texts. It may include eg previous versions that are now no longer available

doi
pubmed
urn-nbn

Altmetric score

doi
pubmed
urn-nbn
Total: 117 hits
CiteExportLink to record
Permanent link

Direct link
Cite
Citation style
  • apa
  • ieee
  • modern-language-association-8th-edition
  • vancouver
  • Other style
More styles
Language
  • de-DE
  • en-GB
  • en-US
  • fi-FI
  • nn-NO
  • nn-NB
  • sv-SE
  • Other locale
More languages
Output format
  • html
  • text
  • asciidoc
  • rtf