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New Data in Myelodysplastic Syndromes Presented at the 56th American Society of Hematology (ASH) Annual Meeting and Exposition

BIOGY

Celgene Corporation (NASDAQ:CELG) and Acceleron Pharma Inc. (NASDAQ:XLRN) today announced preliminary data from ongoing phase 2 clinical trials in patients with lower risk myelodysplastic syndromes (MDS) at the 56th American Society of Hematology (ASH) Annual Meeting and Exposition. In his oral presentation, Dr. Uwe Platzbecker showed that patients with lower risk MDS treated with luspatercept achieved increased hemoglobin levels and transfusion independence. In a separate poster presentation, Dr. Rami Komrokji showed that lower risk MDS patients who were treated with sotatercept also achieved increased hemoglobin levels and transfusion independence. Celgene and Acceleron are jointly developing luspatercept and sotatercept.

“These results in lower risk MDS patients are very exciting,” said Uwe Platzbecker, M.D., Professor of Hematology and Head of the MDS program at the University Hospital in Dresden, Germany and coordinating principal investigator of the luspatercept PACE-MDS study. “Sotatercept and luspatercept may be useful early in the treatment of lower risk MDS patients, either as the initial treatment for anemia or in patients who do not respond or become refractory to treatment with ESAs. These investigational therapeutics have been very well-tolerated and therefore have the potential to benefit many MDS patients.”

Luspatercept Data Presented at ASH

In this study, luspatercept was evaluated in patients with low- or intermediate-1 risk MDS.

A total of 26 patients were treated in this dose-finding stage of the study in which luspatercept was administered subcutaneously once every 3 weeks for up to 5 doses (16 weeks) at doses of 0.125 (n=3), 0.25 (n=3), 0.5 (n=3), 0.75 (n=6), 1.0 (n=3) 1.33 (n=6), or 1.75 (n=2) mg/kg. Of these 26 patients, 19 had a high transfusion burden (≥4 units RBC/8 weeks) and 7 had a low transfusion burden (<4 units RBC/8 weeks). 54% of patients had been treated previously with erythropoiesis stimulating agents (ESA) and 19% of patients had previously been treated with lenalidomide.

Low Transfusion Burden (LTB) Patients:

  • 4 of 5 (80%) LTB patients treated with doses of 0.75-1.75 mg/kg of luspatercept achieved the primary endpoint of hemoglobin increase ≥1.5 g/dL for ≥2 weeks in this 16 week study
  • Additionally, 2 of 5 (40%) of LTB patients achieved the International Working Group (IWG) Hematologic Improvement Erythroid (HI-E) response criteria of a hemoglobin increase ≥1.5 g/dL for ≥8 weeks
  • The mean maximum change for patients treated with luspatercept doses of 0.75. and 1.75 mg/kg was 2.2 and 3.5 g/dL, respectively
  • All 5 LTB patients treated with luspatercept doses of 0.75-1.75 mg/kg had received prior ESA

High Transfusion Burden (HTB) Patients:

  • 5 of 12 (42%) HTB patients treated with luspatercept doses of 0.75-1.75 mg/kg of achieved IWG HI-E criteria of a reduction of ≥4 units RBC over 8 weeks
  • 3 of 12 (25%) HTB patients treated with luspatercept doses of 0.75-1.75 mg/kg achieved transfusion independence for ≥8 weeks

Emerging markers of response:

  • As published earlier this year in Nature Medicine, the murine analog of luspatercept, RAP-536, can correct ineffective erythropoiesis in a mouse model of MDS
  • Splicing factor 3B1 (SF3B1) mutations are seen commonly in MDS patients with ring sideroblasts and are associated with ineffective erythropoiesis
  • Erythroid response (HI-E, IWG) was achieved in 41% of patients treated at ≥0.75 mg/kg. Erythroid response (HI-E, IWG) was achieved in 67% of patients with ring sideroblasts and SF3B1 mutations

The most common adverse events were diarrhea, muscle spasms, bone pain, fatigue, myalgia and nasopharyngitis. There were no drug-related serious adverse events. There was one possibly related grade 3 adverse event of blast cell count increase.

Sotatercept Data Presented at ASH

A second phase 2 study evaluated sotatercept in patients with low- or intermediate-1 risk MDS.

A total of 54 patients were treated in this dose-finding study in which sotatercept was administered subcutaneously once every 3 weeks at doses of 0.1 (n=7), 0.3 (n=6), 0.5 (n=21), and 1.0 (n=20) mg/kg. Of these 54 patients, 46 (85%) had a high transfusion burden (≥4 units RBC/8 weeks) and 8 (15%) had a low transfusion burden (<4 units RBC/8 weeks). 96% of patients had prior ESA, 57% had a prior hypomethylating agent, and 48% had prior lenalidomide.

Low Transfusion Burden (LTB) Patients:

  • 5 (63%) patients achieved a mean hemoglobin increase ≥1.5 g/dL and transfusion independence sustained for ≥ 8 weeks
  • Duration of transfusion independence ranged from 76 to 233 days
  • Maximum mean hemoglobin increases ranged from 1.9 to 4.4 g/dL

High Transfusion Burden (HTB) Patients:

  • 19 of 45 HTB patients (42%) achieved IWG HI-E criteria of a reduction ≥4 RBC units/8 weeks
  • 5 HTB patients (11%) achieved transfusion independence
  • Duration of transfusion independence ranged from 59 to 345+ days

The most common adverse events were fatigue/asthenia, headache, decreased appetite, nausea and dyspnea. 3 of 54 (6%) patients discontinued due to treatment emergent adverse events considered related to sotatercept. 1 patient with grade 2 hemolytic anemia; 1 patient with grade 3 hypertension; and 1 patient with grade 2 muscle weakness in the 0.3, 0.5, and 1.0 mg/kg dose groups, respectively.

About Luspatercept

Luspatercept is a modified activin receptor type IIB fusion protein that acts as a ligand trap for members in the Transforming Growth Factor-Beta (TGF-β) superfamily involved in the late stages of erythropoiesis (red blood cell production). Luspatercept regulates late-stage erythrocyte (red blood cell) precursor cell differentiation and maturation. This mechanism of action is distinct from that of erythropoietin (EPO), which stimulates the proliferation of early-stage erythrocyte precursor cells. Acceleron and Celgene are jointly developing luspatercept as part of a global collaboration. Luspatercept is currently in phase 2 clinical trials in patients with beta-thalassemia and in patients with myelodysplastic syndromes. For more information, please visit www.clinicaltrials.gov.

About Sotatercept

Sotatercept is an activin receptor type IIA fusion protein that acts as a ligand trap for members in the Transforming Growth Factor-Beta (TGF-β) superfamily involved in the late stages of erythropoiesis (red blood cell production). Sotatercept regulates late-stage erythrocyte (red blood cell) precursor cell differentiation and maturation. This mechanism of action is distinct from that of erythropoietin (EPO), which stimulates the proliferation of early-stage erythrocyte precursor cells. Acceleron and Celgene are jointly developing sotatercept as part of a global collaboration. Sotatercept is currently in multiple phase 2 clinical trials. For more information, please visit www.clinicaltrials.gov.

About Acceleron

Acceleron is a clinical stage biopharmaceutical company focused on the discovery, development and commercialization of novel protein therapeutics for cancer and rare diseases. The company is a leader in understanding the biology of the Transforming Growth Factor-Beta (TGF-β) protein superfamily, a large and diverse group of molecules that are key regulators in the growth and repair of tissues throughout the human body, and in targeting these pathways to develop important new medicines. Acceleron has built a highly productive R&D platform that has generated innovative clinical and preclinical protein therapeutic candidates with novel mechanisms of action. These protein therapeutic candidates have the potential to significantly improve clinical outcomes for patients with cancer and rare diseases.

About Celgene

Celgene Corporation, headquartered in Summit, New Jersey, is an integrated global pharmaceutical company engaged primarily in the discovery, development and commercialization of innovative therapies for the treatment of cancer and inflammatory diseases through gene and protein regulation. For more information, please visit the Company's website at www.celgene.com. Follow us on Twitter @Celgene as well.

Forward-Looking Statements

Acceleron:
Cautionary Note on Forward-Looking Statements

This press release includes forward-looking statements about the Company's strategy, future plans and prospects, including statements regarding the development of the Company's compounds, including luspatercept and sotatercept, and the Company's TGF-β superfamily program generally, the timeline for clinical development and regulatory approval of the Company's compounds, the expected timing for the reporting of data from ongoing trials, and the structure of the Company's planned or pending clinical trials. The words "anticipate," "appear," "believe," "estimate," "expect," "intend," "may," "plan," "predict," "project," "target," "potential," "will," "would," "could," "should," "continue," and similar expressions are intended to identify forward-looking statements, although not all forward-looking statements contain these identifying words. Each forward-looking statement is subject to risks and uncertainties that could cause actual results to differ materially from those expressed or implied in such statement. Applicable risks and uncertainties include the risks that the Company's cash position will be insufficient to fund operations into the second half of 2017, that preclinical testing of the Company's compounds and preliminary data from clinical trials may not be predictive of the results or success of ongoing or later clinical trials, that data may not be available when we expect it to be, that the Company or its collaboration partner, Celgene, will be unable to successfully complete the clinical development of its compounds, that the development of the Company's compounds will take longer or cost more than planned, that the Company may be delayed in initiating or completing any clinical trials, and that the Company's compounds will not receive regulatory approval or become commercially successful products. Other risks and uncertainties include those identified under the heading "Risk Factors" included in the Company's Annual Report on Form 10-K which was filed with the Securities and Exchange Commission (SEC) on March 17, 2014, and other filings that the Company may make with the SEC in the future. The forward-looking statements contained in this press release reflect the Company's current views with respect to future events, and the Company does not undertake and specifically disclaims any obligation to update any forward-looking statements.

Celgene:

This press release contains forward-looking statements, which are generally statements that are not historical facts. Forward-looking statements can be identified by the words "expects," "anticipates," "believes," "intends," "estimates," "plans," "will," “outlook” and similar expressions. Forward-looking statements are based on management’s current plans, estimates, assumptions and projections, and speak only as of the date they are made. We undertake no obligation to update any forward-looking statement in light of new information or future events, except as otherwise required by law. Forward-looking statements involve inherent risks and uncertainties, most of which are difficult to predict and are generally beyond our control. Actual results or outcomes may differ materially from those implied by the forward-looking statements as a result of the impact of a number of factors, many of which are discussed in more detail in our Annual Report on Form 10-K and our other reports filed with the Securities and Exchange Commission.

For Celgene:
Investors: 908-673-9628
investors@celgene.com
or
Media: 908-673-2275
media@celgene.com
or
For Acceleron
Investors:
Acceleron Pharma
Steven Ertel, 617-649-9234
Chief Business Officer
or
Media:
Suda Communications LLC
Maureen L. Suda, 585-387-9248