Review Article

ANCA-Associated Vasculitides and Hematologic Malignancies: Lessons from the Past and Future Perspectives

Table 1

Studies investigating the incidence of leukemia and all hematopoietic tumours in AAVs.

Knight et al. 2002 [62]Faurschou et al. 2008 [63]Holle et al. 2011 [64]Heijl et al. 2011 [65]Zycinska et al. 2013 [66]Faurschou et al. 2015 [67]Rahmattulla et al. 2015 [68]van Daalen et al. 2017 [33]Sriskandarajah et al. 2017 [69]Heaf et al. 2018 [70]Yoo et al. 2018 [46]

SIR of leukemia (95% CI)5.7 (2.3–12)5.9 (1.2-17)Not statistically increased3.2 (0.4-11.7)4.3 (2.1-11.2)13.3 (3.6-34)Not Statistically increasedNot statistically increasedNot statistically increased
Observed minus expected leukemia cases (%) if significant11
SIR of all hematopoietic tumours (95% CI)3.8 (2.1–6.3)1.9 (0.5–5.0)Not statistically increasedNot statistically increased3.52 (1.32–9.37)Not statistically increased
Study period1969-19941973-19991966-20021995-20021990-20081973-19991991-20132000-20141988-20121985-2015
Patient number1065293290535117293138323419278150
Patient compositionGPA onlyGPA onlyGPA onlyAAVAAVGPA onlyAAVAAVAAVAAVAAV
CountrySwedenDenmarkGermanyEurope, MexicoPolandDenmarkNetherlandsEuropeNorwayDenmarkKorea
Data collectionNational studyNational studySingle-center studyMulticenter studySingle-center studyNational studySingle-center studySingle-center studyNational studyNational studySingle-center study