Guidelines for the management of haemophilia in Egypt
, , , , , , , , , , and | Jan 18, 2019
About this article
Article Category: Clinical Practice
Published Online: Jan 18, 2019
Page range: 83 - 92
DOI: https://doi.org/10.17225/jhp00118
Keywords
© 2018 Galila Mokhtar, Amal El-Beshlawy, Mohssen El Alfy, Magdy El Ekiaby, Naglaa Omar, Khaled Abd El-Azim Eid, Hoda Hassab, Osama El Safy, Ahmed Mansour, Rasha El Ashry, Naglaa Shaheen, Azza Abdel Gawad, published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.
Figure 1
Definitions of factor replacement therapy protocols [1]
| PROTOCOL | DEFINITION |
|---|---|
| On-demand treatment (episodic) | Treatment given at the time of clinically evident bleeding |
| Primary prophylaxis | Regular continuous Continuous is defined as the intent of treating for 52 weeks per year and receiving a minimum of an a priori defined frequency of infusions for at least 45 weeks (85%) of the year under consideration Large joints: ankles, knees, hips, elbows and shoulders |
| Secondary prophylaxis | Regular continuous Continuous is defined as the intent of treating for 52 weeks per year and receiving a minimum of an a priori defined frequency of infusions for at least 45 weeks (85%) of the year under consideration Large joints: ankles, knees, hips, elbows and shoulders |
| Tertiary prophylaxis | Regular continuous Continuous is defined as the intent of treating for 52 weeks per year and receiving a minimum of an a priori defined frequency of infusions for at least 45 weeks (85%) of the year under consideration |
| Intermittent prophylaxis (periodic) | Treatment given to prevent bleeding for periods not exceeding 45 weeks in a year |
Relationship of bleeding severity to clotting factor level [1]
| SEVERITY | CLOTTING FACTOR LEVEL | BLEEDING EPISODES |
|---|---|---|
| Severe | <1 IU/dl (<0.01 IU/ml) or <1% of normal | Spontaneous bleeding into joints or muscles, predominantly in the absence of identifiable haemostatic challenge |
| Moderate | 1–5 IU/dl (0.01–0.05 IU/ml) or 1–5% of normal | Occasional spontaneous bleeding; prolonged bleeding with minor trauma or surgery |
| Mild | 5–40 IU/dl (0.05–0.40 IU/ml) or 5- <40% of normal | Severe bleeding with major trauma or surgery. Spontaneous bleeding is rare |
Clotting factor concentrate replacement before and after surgery
| HAEMOPHILIA A | HAEMOPHILIA B | |||||
|---|---|---|---|---|---|---|
| TYPE OF | DESIRED | DURATION | INTERVAL | DESIRED | DURATION | INTERVAL |
| Major surgery | ||||||
| Pre-op | 80-100 | 60-80 | ||||
| Post-op | 60-80 | 1-3 | 8-12 | 40-60 | 1-3 | 12 |
| 40-60 | 4-6 | 8-12 | 30-50 | 4-6 | 12 | |
| 30 -50 | 7-14 | 12 | 20-40 | 7-14 | 24 | |
| Minor surgery | ||||||
| Pre-op | > 50 | > 50 | ||||
| Post-op | 1-5 depending | 12 | 1-5 depending | 24 | ||
| FVIII/FIX should be monitored peri-operatively-, immediately postoperative and at least once daily in the hospitalised period | ||||||