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Impact of intermediate‐dose prophylaxis on progression of haemarthropathy in patients with severe haemophilia A: A 10‐year, single‐centre experience in Korea
Author(s) -
Kim Ju Y.,
Lee Da J.,
Chun Tong J.,
You Chur W.
Publication year - 2018
Publication title -
haemophilia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.213
H-Index - 92
eISSN - 1365-2516
pISSN - 1351-8216
DOI - 10.1111/hae.13610
Subject(s) - medicine , haemophilia , haemophilia a , quality of life (healthcare) , age groups , surgery , pediatrics , demography , nursing , sociology
Abstract Aim To determine the impact of 10‐year intermediate‐dose prophylaxis on haemarthropathy progression in patients with severe haemophilia A (SHA). Methods Prophylactic treatment with intermediate dose was given maximally for 10 years to 42 patients with SHA in a haemophilia treatment centre in Korea. Patients were divided into three groups based on prophylactic treatment started age: 1‐10 (group A’), 11‐20 (group B’), and ≥21 (group C’). Average annual increase of Pettersson score (P‐score) was compared between the treatment groups. Results Average ages and P‐scores at initiation of prophylaxis were 4.65±3.43 years and 2.09±3.25, 16.13±1.73 years and 7.37±4.38, and 28.33±7.25 years and 12.33±6.50 for groups A’, B’, and C’. Average annual increase of P‐score in groups A’, B’, and C’ was 0.039±0.11, 0.063±0.123, and 0.078±0.124. Assuming that intermediate‐dose prophylaxis started at the average age, P‐score and annual increase of P‐score would be the average values of each group; it would thus take 210 and 46.5 years to reach –2SD of the average critical level of haemarthropathy (The level of haemarthropathy (P‐score 13.0 ± 2.7) above which there is a significant impact on quality of life in Korean) in groups A’ and B’. However, it would take 55 and 15.75 years if the annual P‐score increase were +2SD of the average value in groups A’ and B’. Conclusion Intermediate‐dose prophylaxis for patients with SHA in Korea would maintain arthropathy below the critical level for most of the patients’ lifetime when started before adolescence. However, this would not be achieved in some adolescent patients with rapid progression of arthropathy and in most adult patients.