Various regimens for prophylactic treatment of patients with haemophilia
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Various regimens for prophylactic treatment of patients with haemophilia. / Schwarz, Rudolf; Ljung, Rolf; Tedgård, Ulf.
In: EUR J HAEMATOL, Vol. 94 , 02.2015, p. 11-6.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Various regimens for prophylactic treatment of patients with haemophilia
AU - Schwarz, Rudolf
AU - Ljung, Rolf
AU - Tedgård, Ulf
N1 - © 2015 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
PY - 2015/2
Y1 - 2015/2
N2 - Haemophilia prophylaxis is superior to on-demand treatment to prevent joint damage. 'High-dose prophylaxis' as used in Sweden is more effective in preventing arthropathy than an 'intermediate-dose regimen' (the Netherlands) and the Canadian tailored primary prophylaxis. Prophylaxis may reduce the risk of developing inhibitors. There is no difference in inhibitor risk between plasma derived and recombinant factor VIII (rFVIII) products but the Rodin study showed increased risk with second-generation rFVIII products. MRI is a new and very sensitive tool to detect the symptoms of early arthropathy but some results (soft tissue changes in 'bleed-free joints') still need to be investigated. Ultrasound is a very helpful method to aid diagnosis especially during the acute phase of a bleed. The risk of infection with central venous access remains a matter of debate. A fully implanted central venous access device (CVAD) has a significant lower risk of infection compared to external CVADs. Patient's age under 6 yr and inhibitor presence are additional risk factors for infections. The role of arteriovenous fistulae needs to be investigated because significant complications have been reported. Disease-specific quality of life instruments are complementary to generic instruments evaluating QoL in patients with haemophilia and have become important health outcome measures.
AB - Haemophilia prophylaxis is superior to on-demand treatment to prevent joint damage. 'High-dose prophylaxis' as used in Sweden is more effective in preventing arthropathy than an 'intermediate-dose regimen' (the Netherlands) and the Canadian tailored primary prophylaxis. Prophylaxis may reduce the risk of developing inhibitors. There is no difference in inhibitor risk between plasma derived and recombinant factor VIII (rFVIII) products but the Rodin study showed increased risk with second-generation rFVIII products. MRI is a new and very sensitive tool to detect the symptoms of early arthropathy but some results (soft tissue changes in 'bleed-free joints') still need to be investigated. Ultrasound is a very helpful method to aid diagnosis especially during the acute phase of a bleed. The risk of infection with central venous access remains a matter of debate. A fully implanted central venous access device (CVAD) has a significant lower risk of infection compared to external CVADs. Patient's age under 6 yr and inhibitor presence are additional risk factors for infections. The role of arteriovenous fistulae needs to be investigated because significant complications have been reported. Disease-specific quality of life instruments are complementary to generic instruments evaluating QoL in patients with haemophilia and have become important health outcome measures.
KW - Adult
KW - Age Factors
KW - Antibodies
KW - Arthritis
KW - Catheterization, Central Venous
KW - Child, Preschool
KW - Drug Administration Schedule
KW - Factor VIII
KW - Hemarthrosis
KW - Hemophilia A
KW - Humans
KW - Infant
KW - Joints
KW - Magnetic Resonance Imaging
KW - Quality of Life
KW - Risk Factors
U2 - 10.1111/ejh.12496
DO - 10.1111/ejh.12496
M3 - SCORING: Journal article
C2 - 25560789
VL - 94
SP - 11
EP - 16
JO - EUR J HAEMATOL
JF - EUR J HAEMATOL
SN - 0902-4441
ER -
