CLINICAL IMPACT OF AGE AND COMORBIDITY IN PNH PATIENTS
(Abstract release date: 05/18/17)
EHA Library. Colado varela E. 05/18/17; 182472; PB1758
Enrique Colado varela
Contributions
Contributions
Abstract
Abstract: PB1758
Type: Publication Only
Background
PNH is an ultra-rare disorder affecting mainly young adults, but can be diagnosed in geriatric population. Comorbitidy is more prevalent in general geriatric population and can either hamper diagnostic evaluation or increase the complexity of PNH patient care.
Aims
To identify geriatric-age PNH in Spanish PNH registry
To study the clinical characteristics at diagnosis and evolution of geriatric-age PNH and compare them to non-geriatric PNH population
To analyse the impact of both age and prognosis in the PNH setting
To evaluate the use of eculizumab in geriatric age patients.
Methods
In a multicentric retrospective study, Cumulative Illness Rating Scale for Geriatrics (CIRS-G) and clinical and biological variables have been collected from a Spanish PNH Group patient cohort. Statistical analysis was performed using GraphPad Prism v5 (La Jolla, CA)
Results
44 patients from 11 centres in Spain have been included up to date. 8 patients (17,8%) were diagnosed in geriatric age (equal or older than 65 years) (Age range for the complete cohort: 17-83 years) and 9 patients presented with high comorbidity, arbitrary defined as CIRS-G score >10. (Range for the complete cohort: 3-13) Age and comorbidity were poorly correlated (p= 0.0187, R-square 0.15) No differences in clinical presentation (Classic, PNH in the setting of another bone marrow failure syndrome or Subclinical PHN or high disease activity) when stratifying by age or comorbidity were observed. 4 patients had a concomitant myeloid clonal disorder (3 myelodisplastic syndrome and 1 myeloproliferative neoplasm), 3 of them (75%) in geriatric age.
Median follow up was 7,2 years. Both age equal or older than 65 years and CIRS-G >10 were associated to poorer overall survival (HR: 0.0134 and 0.045 & p= 0.0015 and 0.0103 respectively)
Regarding PNH with high disease activity, 18 patients were identified, 4 of them in geriatric age. In 2 of them (50%), Eculizumab was used, which contrasts with eculizumab use in younger patients (78,6% in the same indication) Regarding comorbitidy impact on eculizumab therapy outcome, 2 patients had CIRS-G score >10 and had similar overall survival as patients with lower comorbidity in this cohort.
Conclusion
Age and comorbidity are associated with poorer overall survival in PNH. Older age and comorbidity may not preclude the use of effective treatment in PNH patients, including those with high disease activity. Prospective evaluation of comorbidity in PNH patients, regardless of age is warranted.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): PNH, Elderly, Comorbidities
Abstract: PB1758
Type: Publication Only
Background
PNH is an ultra-rare disorder affecting mainly young adults, but can be diagnosed in geriatric population. Comorbitidy is more prevalent in general geriatric population and can either hamper diagnostic evaluation or increase the complexity of PNH patient care.
Aims
To identify geriatric-age PNH in Spanish PNH registry
To study the clinical characteristics at diagnosis and evolution of geriatric-age PNH and compare them to non-geriatric PNH population
To analyse the impact of both age and prognosis in the PNH setting
To evaluate the use of eculizumab in geriatric age patients.
Methods
In a multicentric retrospective study, Cumulative Illness Rating Scale for Geriatrics (CIRS-G) and clinical and biological variables have been collected from a Spanish PNH Group patient cohort. Statistical analysis was performed using GraphPad Prism v5 (La Jolla, CA)
Results
44 patients from 11 centres in Spain have been included up to date. 8 patients (17,8%) were diagnosed in geriatric age (equal or older than 65 years) (Age range for the complete cohort: 17-83 years) and 9 patients presented with high comorbidity, arbitrary defined as CIRS-G score >10. (Range for the complete cohort: 3-13) Age and comorbidity were poorly correlated (p= 0.0187, R-square 0.15) No differences in clinical presentation (Classic, PNH in the setting of another bone marrow failure syndrome or Subclinical PHN or high disease activity) when stratifying by age or comorbidity were observed. 4 patients had a concomitant myeloid clonal disorder (3 myelodisplastic syndrome and 1 myeloproliferative neoplasm), 3 of them (75%) in geriatric age.
Median follow up was 7,2 years. Both age equal or older than 65 years and CIRS-G >10 were associated to poorer overall survival (HR: 0.0134 and 0.045 & p= 0.0015 and 0.0103 respectively)
Regarding PNH with high disease activity, 18 patients were identified, 4 of them in geriatric age. In 2 of them (50%), Eculizumab was used, which contrasts with eculizumab use in younger patients (78,6% in the same indication) Regarding comorbitidy impact on eculizumab therapy outcome, 2 patients had CIRS-G score >10 and had similar overall survival as patients with lower comorbidity in this cohort.
Conclusion
Age and comorbidity are associated with poorer overall survival in PNH. Older age and comorbidity may not preclude the use of effective treatment in PNH patients, including those with high disease activity. Prospective evaluation of comorbidity in PNH patients, regardless of age is warranted.
Session topic: 12. Bone marrow failure syndromes incl. PNH - Clinical
Keyword(s): PNH, Elderly, Comorbidities
{{ help_message }}
{{filter}}