Survival in Adult Patients with Chronic Primary and Secondary Immune Thrombocytopenia: A Population-based Study

Overview

Journal Transfusion

Specialty Hematology

Date 2023 Jan 5

PMID 36601709

Authors

Nikolaj Mannering

Dennis Lund Hansen

Anton Pottegard

Henrik Frederiksen

Affiliations

Soon will be listed here.

Abstract

Background: Few studies have investigated long-term survival in patients with primary immune thrombocytopenia (pITP). Further, changes in prognosis over the past decades and prognosis of secondary immune thrombocytopenia (sITP) are largely unstudied. Our objectives were to study comorbidity-adjusted prognostic changes and causes of death in chronic pITP and sITP patients.

Study Design/methods: Using nationwide Danish health registries 1980-2016, we identified 1762 patients with chronic pITP (median age 58 (IQR, 37-73) years) and 128 with chronic sITP (median age 59 (IQR, 40-73) years). Patients were age-sex-matched to 74,781 general population comparators. Comorbidity was assessed using Charlson Comorbidity Index (CCI).

Results: Overall median survival was reduced by 5.1 years (95% CI, 0.7-9.4) (p < .001) for pITP and 11.1 years (95% CI, 5.8-16.4) (p < .001) for sITP. 5-year survival increased from 69% (95% CI, 59-78) in 1980-89 to 80% (95% CI, 75-83) in 2010-16 for pITP, and decreased from 100% (95% CI, 89-98) to 64% (95% CI, 87-91) for sITP. However, numbers were small for sITP. 5-year survival for pITP with high CCI was 41% (95% CI, 32-49), and 85% (95% CI, 83-87) for low CCI. Bleeding, infection and hematological cancer were relatively frequent causes of death with adjusted subhazard ratios of 3.25 (95% CI, 2.33-4.52), 1.53 (95% CI, 1.08-2.16) and 2.16 (95% CI, 1.12-4.16) in pITP respectively, and 10.52 (95% CI, 1.43-77.36) for hematological cancer in sITP.

Conclusions: Long-term survival is reduced in chronic ITP but seems to be improving. Comorbidity and sITP are associated with a poor prognosis.

Citing Articles

Mental health and use of psychotropic prescription drugs in adult patients with primary immune thrombocytopenia: a nationwide population-based cohort study.

Mannering N, Hansen D, Pottegard A, Andersen K, Frederiksen H Haematologica. 2024; 109(9):2944-2954.

PMID: 38721747 PMC: 11367223. DOI: 10.3324/haematol.2024.285364.

Survival in adult patients with chronic primary and secondary immune thrombocytopenia: A population-based study.

Mannering N, Hansen D, Pottegard A, Frederiksen H Transfusion. 2023; 63(2):415-426.

PMID: 36601709 PMC: 10108240. DOI: 10.1111/trf.17212.

References

Hansen D, Moller S, Andersen K, Gaist D, Frederiksen H . Evans syndrome in adults - incidence, prevalence, and survival in a nationwide cohort. Am J Hematol. 2019; 94(10):1081-1090. DOI: 10.1002/ajh.25574. View

Sandvad M, Pedersen E, Frederiksen H, Mannering N . Risk of infection in adult patients with primary immune thrombocytopenia (ITP): a systematic review. Expert Rev Hematol. 2021; 14(10):961-974. DOI: 10.1080/17474086.2021.1976635. View

Pamuk G, Pamuk O, Baslar Z, Ongoren S, Soysal T, Ferhanoglu B . Overview of 321 patients with idiopathic thrombocytopenic purpura. Retrospective analysis of the clinical features and response to therapy. Ann Hematol. 2002; 81(8):436-40. DOI: 10.1007/s00277-002-0488-x. View

Khodadi E, Asnafi A, Shahrabi S, Shahjahani M, Saki N . Bone marrow niche in immune thrombocytopenia: a focus on megakaryopoiesis. Ann Hematol. 2016; 95(11):1765-76. DOI: 10.1007/s00277-016-2703-1. View

Ekstrand C, Bahmanyar S, Cherif H, Kieler H, Linder M . Cancer risk in patients with primary immune thrombocytopenia - A Swedish nationwide register study. Cancer Epidemiol. 2020; 69:101806. DOI: 10.1016/j.canep.2020.101806. View

Norgaard M, Jensen A, Engebjerg M, Farkas D, Thomsen R, Cha S . Long-term clinical outcomes of patients with primary chronic immune thrombocytopenia: a Danish population-based cohort study. Blood. 2011; 117(13):3514-20. DOI: 10.1182/blood-2010-10-312819. View

Portielje J, Westendorp R, Kluin-Nelemans H, Brand A . Morbidity and mortality in adults with idiopathic thrombocytopenic purpura. Blood. 2001; 97(9):2549-54. DOI: 10.1182/blood.v97.9.2549. View

Frederiksen H, Christiansen C, Norgaard M . Risk and prognosis of adult primary immune thrombocytopenia. Expert Rev Hematol. 2012; 5(2):219-28. DOI: 10.1586/ehm.12.7. View

Chandan J, Thomas T, Lee S, Marshall T, Willis B, Nirantharakumar K . The association between idiopathic thrombocytopenic purpura and cardiovascular disease: a retrospective cohort study. J Thromb Haemost. 2018; 16(3):474-480. DOI: 10.1111/jth.13940. View

10.

Newton J, Reese J, Watson S, Vesely S, Bolton-Maggs P, George J . Fatigue in adult patients with primary immune thrombocytopenia. Eur J Haematol. 2011; 86(5):420-9. DOI: 10.1111/j.1600-0609.2011.01587.x. View

11.

Sestol H, Trangbaek S, Bussel J, Frederiksen H . Health-related quality of life in adult primary immune thrombocytopenia. Expert Rev Hematol. 2018; 11(12):975-985. DOI: 10.1080/17474086.2018.1548930. View

12.

Doobaree I, Nandigam R, Bennett D, Newland A, Provan D . Thromboembolism in adults with primary immune thrombocytopenia: a systematic literature review and meta-analysis. Eur J Haematol. 2016; 97(4):321-30. DOI: 10.1111/ejh.12777. View

13.

Krauth M, Puthenparambil J, Lechner K . Paraneoplastic autoimmune thrombocytopenia in solid tumors. Crit Rev Oncol Hematol. 2011; 81(1):75-81. DOI: 10.1016/j.critrevonc.2011.02.004. View

14.

Thomsen R, Schoonen W, Farkas D, Riis A, Jacobsen J, Fryzek J . Risk for hospital contact with infection in patients with splenectomy: a population-based cohort study. Ann Intern Med. 2009; 151(8):546-55. DOI: 10.7326/0003-4819-151-8-200910200-00008. View

15.

Guidry J, George J, Vesely S, Kennison S, Terrell D . Corticosteroid side-effects and risk for bleeding in immune thrombocytopenic purpura: patient and hematologist perspectives. Eur J Haematol. 2009; 83(3):175-82. DOI: 10.1111/j.1600-0609.2009.01265.x. View

16.

Kashiwagi H, Tomiyama Y . Pathophysiology and management of primary immune thrombocytopenia. Int J Hematol. 2013; 98(1):24-33. DOI: 10.1007/s12185-013-1370-4. View

17.

Palandri F, Polverelli N, Sollazzo D, Romano M, Catani L, Cavo M . Have splenectomy rate and main outcomes of ITP changed after the introduction of new treatments? A monocentric study in the outpatient setting during 35 years. Am J Hematol. 2016; 91(4):E267-72. DOI: 10.1002/ajh.24310. View

18.

Vontela N, Kovesdy C, Latif Z, Lane R, Weir A . Prevalence and Incidence of Immune Thrombocytopenia in Patients With Prostate Cancer. Am J Med Sci. 2018; 356(5):499-500. DOI: 10.1016/j.amjms.2018.05.003. View

19.

Heden K, Jensen A, Farkas D, Norgaard M . Validity of a procedure to identify patients with chronic idiopathic thrombocytopenic purpura in the Danish National Registry of Patients. Clin Epidemiol. 2010; 1:7-10. PMC: 2943173. DOI: 10.2147/clep.s4832. View

20.

Quan H, Li B, Couris C, Fushimi K, Graham P, Hider P . Updating and validating the Charlson comorbidity index and score for risk adjustment in hospital discharge abstracts using data from 6 countries. Am J Epidemiol. 2011; 173(6):676-82. DOI: 10.1093/aje/kwq433. View