Table 5.
Treatment and outcomes for patients with HAE-nC1-INH
| Patient # | Gene | cDNA change | Clinical symptoms | Treatment | Outcomes | Follow-up treatment plans |
|---|---|---|---|---|---|---|
| 1 | MYOF | c.5923G > A | Persistent swelling | No treatment (refused by the patient) | Persistent swelling | Icatibant and lanadelumab |
| 2 | MYOF | c.6001 C > G | Recurrent edema with spontaneous resolution | Icatibant | Shorten duration of edema | Lanadelumab 300 mg q2w |
| Lanadelumab | Decreased frequency and shorten duration of edema | |||||
| 3 | MYOF | c.3965 C > G | Recurrent edema with spontaneous resolution | Allergen avoidance | Decreased frequency of edema | Follow-up |
| 4 | MYOF | c.1097G > T | Recurrent edema with spontaneous resolution | No treatment (refused by the patient) | Frequent attack of edema | Icatibant and lanadelumab |
| 5 | HS3ST6 | c.497G > A | Persistent swelling | Icatibant and lanadelumab for 6 injections | No improvement in symptoms | Follow-up |
| 6 | FXII | c.303_304del | Persistent swelling | Icatibant | No improvement in symptoms | Lanadelumab 300 mg q4w |
| Lanadelumab for 10 injections | Fully remission | |||||
| 7 | KNG1 | c.1690 C > T | Recurrent edema with spontaneous resolution | No treatment (refused by the patient) | No episodes within 1 year of follow-up | Follow-up |
| 8 | KNG1 | c.1143G > C | Recurrent edema and bullae with spontaneous resolution | Allergen avoidance | Decreased frequency of edema | Icatibant and lanadelumab |