Priority 9 from the Bleeding Disorder PSP
| UNCERTAINTY: What is the best way to prevent or treat bleeds in people with bleeding disorders who have developed an inhibitor? (JLA PSP Priority 9) | |
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| Overall ranking | 9 |
| JLA question ID | 0078/9 |
| Explanatory note | At present we have a small number of agents available to treat bleeding episodes in patients with inhibitors. However none of them is reliably effective and excessive use runs the risk of inducing thrombosis. New approaches to haemophilia treatment may be able to reduce bleeding in these patients but again are not completely effective, are designed for prophylaxis rather than acute bleeding and may interact dangerously with existing bypass therapies. All of which makes it important to determine the optimum doses and combinations to maximise benefit and minimise risk. |
| Evidence | Systematic reviews are outdated Wight J, Paisley S, Knight C. Immune tolerance induction in patients with haemophilia A with inhibitors: a systematic review. Haemophilia. 2003;9(4):436-63. Chai-Adisaksopha C, Nevitt SJ, Simpson ML, Janbain M, Konkle BA. Bypassing agent prophylaxis in people with hemophilia A or B with inhibitors. Cochrane Database Syst Rev. 2017;9:CD011441.Zhou ZY, Hay JW. Efficacy of bypassing agents in patients with hemophilia and inhibitors: a systematic review and meta-analysis. Clin Ther. 2012;34(2):434-45 |
| Health Research Classification System category | Blood |
| Extra information provided by this PSP | |
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| Original uncertainty examples | How to cure inhibitors ~ How to prevent inhibitors occurring ~ do treatments course inhibitors |
| Submitted by | Not available |
| PSP information | |
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| PSP unique ID | 0078 |
| PSP name | Bleeding Disorders |
| Total number of uncertainties identified by this PSP. | 66 (To see a full list of all uncertainties identified, please see the detailed spreadsheet held on the JLA website) |
| Date of priority setting workshop | 7 July 2018 |