Main causes of death and therapeutic regimens in 5 patients with RBD.
| Cases | Sex | Age (yr) | Factor levels | Cause of death | The last therapeutic regimen | Treatment/province |
|---|---|---|---|---|---|---|
| FVD-1 | F | 23 | 1% | Post-hemorrhage | FFP | On-demand/Khorasan-Razavi |
| FVD-2 | F | 2 | 4% | Internal bleeding and hepatomegaly | FFP | On-demand/Khorasan-Razavi |
| FVD-3 | F | 2a) | 1% | CNS bleeding | FFP | On-demand/Hamedan |
| FXIIIDb) | M | 23 | <5% | CNS bleeding post HCV eradication | Fibro+Cryo | Pro & On-demand/Hamedan |
| F58D | F | 18 | 7%c), 10%d) | Anaphylaxis shock post FF | FFP+FVIIIC | On-demand/Hamedan |
a)Months, b)case infected with hepatitis C virus (HCV), c)factor V level in plasma, d)factor VIII in plasma.
Abbreviations: CNS, central nervous system; Cryo, cryoprecipitate; F58D, factor V and VIII deficiency; FFP, fresh frozen plasma; Fibro, fibrogammin; FXIIID, factor XIII deficiency; FVD, factor V deficiency; FVIIIC, factor VIII concentrate; Pro, prophylaxis; RBD, rare bleeding disorder.
Blood Res 2020;55:213~216 https://doi.org/10.5045/br.2020.2020023
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