Jazz Pharmaceuticals Completes U.S. FDA Supplemental Biologics License Application for Rylaze™ (asparaginase erwinia chrysanthemi (recombinant)-rywn) Monday/Wednesday/Friday Dosing Schedule

DUBLIN – Jazz Pharmaceuticals plc (Nasdaq: JAZZ) today announced that the Company has completed the submission of a Supplemental Biologics License Application (sBLA) to the U.S. Food and Drug Administration (FDA) seeking approval for a Monday/Wednesday/Friday (M/W/F) intramuscular (IM) dosing schedule for Rylaze™ (asparaginase erwinia chrysanthemi (recombinant)-rywn), for use as a component of a multi-agent chemotherapeutic regimen for the treatment of acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) in adult and pediatric patients one month and older who have developed hypersensitivity to E. coli-derived asparaginase. The submission will be reviewed under the Real-Time Oncology Review (RTOR) program, an initiative of FDA’s Oncology Center of Excellence designed for efficient review of safe and effective cancer treatments, and follows Rylaze’s initial approval under the RTOR program in June 2021.

“We were pleased Rylaze, a much-needed therapeutic option, was approved under the RTOR program while the clinical trial was ongoing. Our science-led and patient-focused development program has enabled us to deliver a clinically significant advancement for patients,” said Rob Iannone, M.D., M.S.C.E., executive vice president, global head of research and development of Jazz Pharmaceuticals. “With a dosing schedule of Rylaze administered 25/25/50 mg/m2 on Monday/Wednesday/Friday, patients maintain a clinically meaningful level of nadir serum asparaginase activity through the entire duration of treatment. We look forward to submitting two additional regulatory applications this year to ensure as many patients as possible can have access to a reliable and high-quality supply of this important therapy, including another regulatory application to FDA to support the intravenous route of administration and an additional application in Europe later this year.”

The sBLA submitted by Jazz is supported by data from the three-cohort intramuscular administration part of the Phase 2/3 trial of Rylaze in adult and pediatric patients with ALL and LBL who have developed hypersensitivity to an E. coli-derived asparaginase. The trial studied three dosing regimens of Rylaze, with cohort 1a receiving 25 mg/m2 administered M/W/F, cohort 1b receiving 37.5 mg/m2 administered M/W/F and cohort 1c receiving 25 mg/m2 administered Monday and Wednesday and 50 mg/m2 administered on Friday. Initial results showed that in cohort 1c, a dosing regimen of Rylaze administered 25 mg/m2 on Monday and Wednesday and 50 mg/m2 on Friday demonstrated a positive benefit-to-risk profile, showing that Rylaze maintains a clinically meaningful level of nadir serum asparaginase activity (NSAA) ≥0.1 IU/mL at both 48 and 72 hours (from Friday to Monday). In addition, the safety profile of Rylaze was consistent with the reported safety information for patients with ALL/LBL receiving asparaginase with combination chemotherapy. Initial results from the trial were presented at the 63rd American Society of Hematology (ASH) Annual Meeting in December 2021. [1]

The sBLA follows FDA approval of Rylaze in June 2021 under the RTOR program. [2] Rylaze was also granted orphan drug designation for the treatment of ALL/LBL in June 2021 and was added to the National Comprehensive Cancer Network® Clinical Practice Guidelines in Oncology (NCCN Guidelines®) in July 2021. [3]

About Rylaze™ (asparaginase erwinia chrysanthemi (recombinant)-rywn)
Rylaze, also known as JZP458, is approved in the U.S. for use as a component of a multi-agent chemotherapeutic regimen for the treatment of acute lymphoblastic leukemia (ALL) and lymphoblastic lymphoma (LBL) in adult and pediatric patients one month or older who have developed hypersensitivity to E. coli-derived asparaginase. Rylaze has orphan drug designation for the treatment of ALL/LBL in the United States. Rylaze is a recombinant erwinia asparaginase that uses a novel Pseudomonas fluorescens expression platform. JZP458 was granted Fast Track designation by the U.S. Food and Drug Administration (FDA) in October 2019 for the treatment of this patient population. Rylaze was approved as part of the Real-Time Oncology Review program, an initiative of the FDA’s Oncology Center of Excellence designed for efficient delivery of safe and effective cancer treatments to patients.

The full U.S. Prescribing Information for Rylaze is available at: https://pp.jazzpharma.com/pi/rylaze.en.USPI.pdf

Important Safety Information

RYLAZE should not be given to people who have had:

  • Serious allergic reactions to RYLAZE
  • Serious swelling of the pancreas (stomach pain), serious blood clots, or serious bleeding during previous asparaginase treatment

RYLAZE may cause serious side effects, including:

  • Allergic reactions (a feeling of tightness in your throat, unusual swelling/redness in your throat and/or tongue, or trouble breathing), some of which may be life-threatening
  • Swelling of the pancreas (stomach pain)
  • Blood clots (may have a headache or pain in leg, arm, or chest)
  • Bleeding
  • Liver problems

Contact your doctor immediately if any of these side effects occur.

Some of the most common side effects with RYLAZE include: liver problems, nausea, bone and muscle pain, tiredness, infection, headache, fever, allergic reactions, fever with low white blood cell count, decreased appetite, mouth swelling (sometimes with sores), bleeding, and too much sugar in the blood.

RYLAZE can harm your unborn baby. Inform your doctor if you are pregnant, planning to become pregnant, or nursing. Females of reproductive potential should use effective contraception (other than oral contraceptives) during treatment and for 3 months following the final dose. Do not breastfeed while receiving RYLAZE and for 1 week after the final dose.

Tell your healthcare provider if there are any side effects that are bothersome or that do not go away.

These are not all the possible side effects of RYLAZE. For more information, ask your healthcare provider.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.fda.gov/medwatch, or call 1-800-FDA-1088 (1-800-332-1088).

About Acute Lymphoblastic Leukemia (ALL)
ALL is a cancer of the blood and bone marrow that can progress quickly if not treated. [4] Leukemia is the most common cancer in children, and about three out of four of these cases are ALL. [5] Although it is one of the most common cancers in children, ALL is among the most curable of the pediatric malignancies due to recent advancements in treatment. [6,7] Adults can also develop ALL, and about four of every 10 cases of ALL diagnosed are in adults. [5] The American Cancer Society estimates that almost 6,000 new cases of ALL will be diagnosed in the United States in 2021. [5] Asparaginase is a core component of multi-agent chemotherapeutic regimens in ALL. [8] However, asparaginase treatments derived from E. coli are associated with the potential for development of hypersensitivity reactions. [9]

About Lymphoblastic Lymphoma (LBL)
LBL is a rare, fast-growing, aggressive subtype of Non-Hodgkin’s lymphoma, most often seen in teenagers and young adults. [10] LBL is a very aggressive lymphoma – also called high-grade lymphoma – which means the lymphoma grows quickly with early spread to different parts of the body. [11,12]

About Jazz Pharmaceuticals plc
Jazz Pharmaceuticals plc (NASDAQ: JAZZ) is a global biopharmaceutical company whose purpose is to innovate to transform the lives of patients and their families. We are dedicated to developing life-changing medicines for people with serious diseases—often with limited or no therapeutic options. We have a diverse portfolio of marketed medicines and novel product candidates, from early- to late-stage development, in neuroscience and oncology. Within these therapeutic areas, we are identifying new options for patients by actively exploring small molecules and biologics, and through innovative delivery technologies and cannabinoid science. Jazz is headquartered in Dublin, Ireland and has employees around the globe, serving patients in nearly 75 countries. For more information, please visit www.jazzpharmaceuticals.com and follow @JazzPharma on Twitter.

Contact:

Media
Kristin Bhavnani
Head of Global Corporate Communications
Jazz Pharmaceuticals plc
[email protected]
Ireland +353 1 697 2141
U.S. +1 215 867 4948

Investor:
Andrea N. Flynn, Ph.D.
Vice President, Head, Investor Relations
Jazz Pharmaceuticals plc
[email protected]
Ireland +353 1 634 3211
U.S. +1 650 496 2717

References

1 American Society of Hematology Annual Meeting and Exposition. 2307 Initial Results from a Phase 2/3 Study of Recombinant Erwinia Asparaginase (JZP458) in Patients with Acute Lymphoblastic Leukemia (ALL)/Lymphoblastic Lymphoma (LBL) Who Are Allergic/Hypersensitive to E. coli–Derived Asparaginases. Available at www.ash.confex.com/ash/2021/webprogram/Paper147023.html. Accessed January 2022.

2 Rylaze (asparaginase erwinia chrysanthemi (recombinant)-rywn) injection, for intramuscular use Prescribing Information. Palo Alto, CA: Jazz Pharmaceuticals, Inc.

3 NCCN Guidelines: Acute Lymphoblastic Leukemia. Available at https://www.nccn.org/login?ReturnURL=https://www.nccn.org/professionals/physician_gls/pdf/all.pdf. Accessed January 2022

4 National Cancer Institute. Adult Acute Lymphoblastic Leukemia Treatment (PDQ®)–Patient Version. Available at www.cancer.gov/types/leukemia/patient/adult-all-treatment-pdq. Accessed January 2022.

5 American Cancer Society. Key Statistics for Childhood Leukemia. Available at https://www.cancer.org/cancer/leukemia-in-children/about/key-statistics.html. Accessed January 2022.

6 American Cancer Society. Cancer Facts & Figures 2021. https://www.cancer.org/research/cancer-facts-statistics/all-cancer-facts-figures/cancer-facts-figures-2021.html. Accessed January 2022.

7 Pui C, Evans W. A 50-Year Journey to Cure Childhood Acute Lymphoblastic Leukemia. Seminars in Hematology. 2013;50(3), 185-196.

8 Salzer W, Bostrom B, Messinger Y et al. 2018. Asparaginase activity levels and monitoring in patients with acute lymphoblastic leukemia. Leukemia & Lymphoma. 59:8, 1797-1806, DOI: 10.1080/10428194.2017.1386305.

9 Hijiya N, van der Sluis IM. Asparaginase-associated toxicity in children with acute lymphoblastic leukemia. Leuk Lymphoma. 2016;57(4):748–757. DOI: 10.3109/10428194.2015.1101098.

10 Leukemia Foundation. Lymphoblastic Lymphoma. Available at https://www.leukaemia.org.au/disease-information/lymphomas/non-hodgkin-lymphoma/other-non-hodgkin-lymphomas/lymphoblastic-lymphoma/. Accessed January 2022.

11 Mayo Clinic. Acute Lymphocytic Leukemia Diagnosis. Available at https://www.mayoclinic.org/diseases-conditions/acute-lymphocytic-leukemia/diagnosis-treatment/drc-20369083. Accessed January 2022.

12 Cancer Research UK. High-Grade NHL. Available at https://www.cancerresearchuk.org/about-cancer/non-hodgkin-lymphoma/types/high-grade. Accessed January 2022.