We also advanced AG-236 into an operationally seamless Phase II/III program in polycythemia vera, adding another potential growth driver within hematology. Turning to our second quarter highlights on the next slide, we delivered a quarter marked by strong commercial performance, meaningful pipeline progress, and continued portfolio discipline. First, we delivered sustained commercial momentum with $44.7 million in total net revenue, including $40.9 million in the U.S. Second, we further diversified our pipeline through the in-licensing of cevidoplenib, a next-generation, highly selective oral Syk inhibitor for ITP, progressing toward phase III and strengthening our rare hematology pipeline.
Finally, we ended the quarter with approximately $1 billion in cash equivalents, and marketable securities, providing financial flexibility to support both commercial growth and pipeline progression. Overall, we entered the second half of 2026 with strong commercial delivery, a more diversified pipeline, an important near-term regulatory catalyst, and the capital position to execute on our strategy. Turning to our second quarter financial results, total mitapivat net revenue was $44.7 million, including $40.9 million in the U.S. Selling, general, and administrative expense was $61.5 million, compared to $45.9 million in the prior year's period, reflecting an increase in commercial-related activities as we executed a launch of AQVESME in February.
Turning to our outlook for 2026, we continue to expect approximately $45 million-$50 million from PK deficiency revenues in the U.S. Our priorities for the remainder of the year remain clear: driving the AQVESME launch, preparing for a potential sickle cell disease approval, advancing our pipeline, and maintaining financial discipline. In the U.S., performance reflected continued growth in thalassemia demand and solid commercial execution. Net revenue in the quarter reflected approximately $5 million of one-time benefits related to stocking in thalassemia, along with modest gross-to-net favorability.
| Metric | Period | Current guidance |
|---|---|---|
| U.S. PK deficiency revenue | FY2026 | ~$45M-$50M (reaffirmed) |
| Full-year operating expenses | FY2026 | approximately flat vs 2025 (excl. $25M cevidoplenib upfront) |
| Gross-to-net | ongoing | 10%-20% (reaffirmed, with quarter-over-quarter variability) |
| Metric | YoY | Note |
|---|---|---|
| Total mitapivat net revenue | — | Q2 2026 total net revenue of $44.7M; no prior-year comparator disclosed on the call |
| R&D expense | up ~$8.9M ($100.8M vs $91.9M) | Increase in process R&D of ~$15M driven by the $25M upfront payment tied to the Oscotec/cevidoplenib agreement |
| SG&A expense | up ~$15.6M ($61.5M vs $45.9M) | Higher commercial-related activities following the February AQVESME launch |
| Net loss | improved ~$11.3M ($100.7M vs $112M) | Revenue growth partially offsetting higher operating expenses |
| Topic | Previous mention | Current period | Trend |
|---|---|---|---|
| Thalassemia launch metric | Reporting cumulative REMS-certified prescriptions as demand indicator | Will discontinue prescription reporting after Q3 and transition to revenue as primary performance measure | — |
| Patient mix | Early adoption skewed to highly motivated transfusion-dependent (TDT) patients | Growing proportion of non-transfusion-dependent (NTDT) patients, the larger commercial opportunity | — |
| Time to treatment initiation | Faster than the anticipated 10-12-week range due to motivated early patients | Trending toward and expected to stabilize within the 10-12-week range as NTDT adoption broadens | — |
| Sickle cell disease | sNDA under FDA review | Accepted with priority review, Nov 1 PDUFA; first patient dosed in REIGNITE confirmatory trial; commercial launch prep underway | — |
| Pipeline breadth | Anchored by mitapivat | Expanded into ITP (cevidoplenib), polycythemia vera (AG-236), and PKU (AG-181) | — |