Hereditary C1 inhibitor deficiency
ICD-10 D84.1 ICD-11 4A00.14

First-Line Acute Attack Management in Hereditary C1 Inhibitor Deficiency

Acute attacks in hereditary C1 inhibitor deficiency require prompt intervention. Identifying the right treatment option depends on what is available and clinically safe for the individual patient at the time of the attack.

First-line management of acute attacks involves use of a specific plasma-derived blood product — the full protocol specifies the precise conditions under which this option applies and what the complete decision algorithm entails.

Full details, including selection criteria and the complete treatment pathway, are in the structured protocol below.

References

DOI: 10.1111/all.15214

If SDP is not available, attacks should be treated with fresh frozen plasma (FFP), where safe supply is available.

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