Hemophilia A / B: Management

Hemophilia A / B: overview

This algorithm summarizes management of hemophilia A and B, branching on the deficient factor and on the presence of inhibitors.

For hemophilia A without an inhibitor, prophylaxis with emicizumab (HAVEN) is shown alongside replacement with factor VIII concentrate, available in standard and extended half-life formulations. Hemophilia B is managed with factor IX concentrate given as prophylaxis. For patients with inhibitors, the chart lists three complementary strategies. In hemophilia A, emicizumab remains effective despite inhibitors and serves as prophylaxis. Acute bleeding is treated with bypassing agents, either activated prothrombin complex concentrate (aPCC) or recombinant factor VIIa (rFVIIa). Eradication of the inhibitor is attempted with immune tolerance induction using high-dose factor. Inhibitor status is therefore the key branch that shifts management away from simple factor replacement.

Trials behind this algorithm

HAVEN 3 trial, emicizumab, no inhibitor ≥12y/o Mahlangu J et al, NEJM 2018, PMID: 30157389
Joint Outcome Study Manco-Johnson et al, NEJM, 2007, PMID:17687129
HAVEN 1 trial, emicizumab, with inhibitor ≥12y/o Oldenburg J et al, NEJM 2017, PMID: 28691557

Background reading: Hemophilia board review notes.

Hemophilia A / B: the algorithm in words

Starting point: Hemophilia A or B (by factor). Full background: Hemophilia notes.

Hemophilia A: no inhibitor (first-line): Prophylaxis: Emicizumab (HAVEN 3); Replacement: Factor VIII concentrate (prophylaxis · Joint Outcome Study).
Hemophilia B (first-line): Replacement: Factor IX concentrate (prophylaxis).
With inhibitors (second-line): Hem A: Emicizumab (HAVEN 1); Bleeds: Bypassing agents (aPCC / rFVIIa) (acute bleeding); Eradicate: Immune tolerance induction (high-dose factor).