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RecruitingNCT05922839Updated Apr 26, 2024

Zanubrutinib in the Treatment of Relapsed/Refractory wAIHA

A Phase 2 interventional study of Zanubrutinib in Warm Autoimmune Hemolytic Anemia, sponsored by Chen Miao. Recruiting at 1 site in China. Open to participants aged 18 Years to 90 Years. Per ClinicalTrials.gov, last updated 2024-04-26.

Sponsored by Chen Miao · Phase 2, Interventional, and Treatment

Phase
Phase 2
Study type
Interventional
Enrollment
22
Allocation
Not applicable
Ages
18 Years to 90 Years
Sex
All
01

Study summary

Autoimmune hemolytic anemia (AIHA) is a rare and heterogeneous disorder characterized by the destruction of red blood cells through warm or cold antibodies. Glucocorticoid (combined with rituximab) is the first-line treatment.

However, the recurrence rate is very high and some patients may not respond to steroids. Second-line therapies include cyclosporine A (CsA), cyclophosphamide, rituximab, azathioprine, and even splenectomy.

Bruton's tyrosine kinase (BTK) plays a crucial role in the signaling pathway of B-cell receptor (BCR), and has been found to be a major source of pathogenic signal transduction for various lymphoproliferative malignancies. The activity of BTK is related to the occurrence and progression of various B-cell lymphomas. Currently, BTK inhibitors are widely used in the treatment of B-cell lymphomas, including chronic lymphocytic leukemia (CLL), mantle cell lymphoma (MCL), Waldenstrom's macroglobulinemia (WM), and other B-cell lymphomas, showing significant efficacy. BTK affects the production of messenger molecules and regulates the BCR signaling pathway, causing B cells to transform into self-reactive B cells, which can trigger autoimmune diseases. Current research has shown that BTK activity increases in several autoimmune diseases, including systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) . Therefore, BTK inhibitors (BTKi) are important for the treatment of autoimmune diseases.

Ibrutinib, one kind of BTKi, has been proven to treat secondary autoimmune hemolytic anemia (AIHA) in CLL and control CLL progression, and is an effective drug for treating lymphoma-associated AIHA . One kind of second-generation selective BTKi, acalabrutinib, can also reduce the incidence of AIHA in relapsed or refractory CLL patients. Currently, phase-II clinical studies exploring the treatment of AIHA using Ibrutinib, acalabrutinib, and rilzabrutinib, another BTKi, are underway.

Zanubrutinib (BGB-3111, Brukinsa®, BeiGene) is a second-generation irreversible BTKi developed by Chinese company BeiGene. Compared to Ibrutinib, zanubrutinib has shown stronger effective activity and higher selectivity towards BTK, and weaker effects on other targets such as TEC, EGFR, and Src families, with low off-target side effects. Its efficacy, durability, oral absorption, and targeting are better than those of Ibrutinib. Zanubrutinib is approved for the treatment of various B-cell lymphomas, and clinical trials have shown excellent efficacy and tolerability in CLL and WM patients. In previously treated CLL patients, zanubrutinib exhibits better efficacy and safety than Ibrutinib. Currently, phase II clinical studies of zanubrutinib in ITP, antiphospholipid syndrome, IgG4-related immune diseases, and active proliferative lupus nephritis are underway. The therapeutic effect of zanubrutinib on refractory warm autoimmune hemolytic anemia, is worth exploring through exploratory research.

02

Conditions studied

  • Warm Autoimmune Hemolytic Anemia

Keywords

  • warm autoimmune hemolytic anemia
  • Zanubrutinib
03

Who can participate

Ages eligible
18 Years to 90 Years
Sexes eligible
All
Accepts healthy volunteers
No

Inclusion criteria

  1. Age ≥18 years.
  2. Confirmed diagnosis of wAIHA or Evans syndrome, primary or secondary to connective tissue disease. If it is secondary, there are no other treatment indications for systemic involvement of primary connective tissue disease.
  3. No response or relapse after glucocorticoid treatment.
  4. Baseline liver and kidney function (ALT, AST, Cr) is less than 2 times the normal range.
  5. Consent to sign the informed consent form.

Exclusion criteria

Exclusion Criteria:

  1. Other important organ involvement in connective tissue disease.
  2. Uncontrolled infection or bleeding with standard treatment.
  3. Active HIV, HCV or HBV infection uncontrolled with standard treatment.
  4. Concurrent uncontrolled advanced malignant tumors or lymphoma.
  5. The subject is receiving any of the following drugs and has not met the following conditions of stable drug treatment duration at a fixed dose during screening: corticosteroids for at least 4 weeks; iron, vitamin B12 or folic acid for at least 4 weeks;
  6. Liver cirrhosis or portal hypertension.
  7. Pregnant or lactating women.
  8. Participation in other clinical trials within the last 3 months.
04

Study design

Phase
Phase 2
Primary purpose
Treatment
Allocation
Not applicable
Intervention model
Single group
Masking
None (open label)
Enrollment
22 participants (estimated)

Study arms

  • Experimental
    Zanubrutinib in the Treatment of Relapsed/Refractory wAIHA

    Zanubrutinib 160mg, orally, twice daily, for a minimum of 3 months For effective patients, continue to use for 2 years after achieving optimal therapeutic effect.

    Drug: Zanubrutinib

Interventions

  • DrugZanubrutinib

    Zanubrutinib 160mg, orally, twice daily, for a minimum of 3 months, and for effective patients, continue to use for 2 years after achieving optimal therapeutic effect.

05

What researchers measure

Primary outcomes

  1. Overall response rate (ORR)

    ORR defined as the proportion of patients who met the criteria of either complete response (CR) or partial response (PR)

    Time frame: 3/6 months

  2. Complete response rate (CRR)

    CRR defined as the proportion of patients who met the criteria of complete response.

    Time frame: 3/6 months

Secondary outcomes

  1. Relapse rate

    Relapse rate defined as the proportion of patients whose response shift from PR or CR to no response (NR).

    Time frame: 3/6 months

  2. Adverse events

    Safety analyses include assessments of the incidence and severity of adverse events; all adverse events that occurred or worsened during the treatment period will be reported, as well as adverse events that occurred later but are considered by the investigator to be related to the trial drug.

    Time frame: 3/6 months

06

Study locations

1 of 1 sites recruiting
  • Peking Union Medical College Hospital
    Beijing, Beijing 100730, China
    Recruiting
07

References and documents

Publications

  • Jager U, Barcellini W, Broome CM, Gertz MA, Hill A, Hill QA, Jilma B, Kuter DJ, Michel M, Montillo M, Roth A, Zeerleder SS, Berentsen S. Diagnosis and treatment of autoimmune hemolytic anemia in adults: Recommendations from the First International Consensus Meeting. Blood Rev. 2020 May;41:100648. doi: 10.1016/j.blre.2019.100648. Epub 2019 Dec 5. PubMed 31839434 ↗
  • Birgens H, Frederiksen H, Hasselbalch HC, Rasmussen IH, Nielsen OJ, Kjeldsen L, Larsen H, Mourits-Andersen T, Plesner T, Ronnov-Jessen D, Vestergaard H, Klausen TW, Schollkopf C. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia. Br J Haematol. 2013 Nov;163(3):393-9. doi: 10.1111/bjh.12541. Epub 2013 Aug 24. PubMed 23981017 ↗
  • Zarrin AA, Bao K, Lupardus P, Vucic D. Kinase inhibition in autoimmunity and inflammation. Nat Rev Drug Discov. 2021 Jan;20(1):39-63. doi: 10.1038/s41573-020-0082-8. Epub 2020 Oct 19. PubMed 33077936 ↗
  • Ringheim GE, Wampole M, Oberoi K. Bruton's Tyrosine Kinase (BTK) Inhibitors and Autoimmune Diseases: Making Sense of BTK Inhibitor Specificity Profiles and Recent Clinical Trial Successes and Failures. Front Immunol. 2021 Nov 3;12:662223. doi: 10.3389/fimmu.2021.662223. eCollection 2021. PubMed 34803999 ↗
  • McDonald C, Xanthopoulos C, Kostareli E. The role of Bruton's tyrosine kinase in the immune system and disease. Immunology. 2021 Dec;164(4):722-736. doi: 10.1111/imm.13416. Epub 2021 Oct 4. PubMed 34534359 ↗
  • Liubchenko GA, Appleberry HC, Striebich CC, Franklin KE, Derber LA, Holers VM, Lyubchenko T. Rheumatoid arthritis is associated with signaling alterations in naturally occurring autoreactive B-lymphocytes. J Autoimmun. 2013 Feb;40:111-21. doi: 10.1016/j.jaut.2012.09.001. Epub 2012 Oct 8. PubMed 23058039 ↗
  • Iwata S, Tanaka Y. B-cell subsets, signaling and their roles in secretion of autoantibodies. Lupus. 2016 Jul;25(8):850-6. doi: 10.1177/0961203316643172. PubMed 27252261 ↗
  • Montillo M, O'Brien S, Tedeschi A, Byrd JC, Dearden C, Gill D, Brown JR, Barrientos JC, Mulligan SP, Furman RR, Cymbalista F, Plascencia C, Chang S, Hsu E, James DF, Hillmen P. Ibrutinib in previously treated chronic lymphocytic leukemia patients with autoimmune cytopenias in the RESONATE study. Blood Cancer J. 2017 Feb 3;7(2):e524. doi: 10.1038/bcj.2017.5. No abstract available. PubMed 28157216 ↗
  • Rogers KA, Ruppert AS, Bingman A, Andritsos LA, Awan FT, Blum KA, Flynn JM, Jaglowski SM, Lozanski G, Maddocks KJ, Byrd JC, Woyach JA, Jones JA. Incidence and description of autoimmune cytopenias during treatment with ibrutinib for chronic lymphocytic leukemia. Leukemia. 2016 Feb;30(2):346-50. doi: 10.1038/leu.2015.273. Epub 2015 Oct 7. PubMed 26442611 ↗
  • Manda S, Dunbar N, Marx-Wood CR, Danilov AV. Ibrutinib is an effective treatment of autoimmune haemolytic anaemia in chronic lymphocytic leukaemia. Br J Haematol. 2015 Sep;170(5):734-6. doi: 10.1111/bjh.13328. Epub 2015 Feb 25. No abstract available. PubMed 25716177 ↗
  • Galinier A, Delwail V, Puyade M. Ibrutinib Is Effective in the Treatment of Autoimmune Haemolytic Anaemia in Mantle Cell Lymphoma. Case Rep Oncol. 2017 Jan 27;10(1):127-129. doi: 10.1159/000456002. eCollection 2017 Jan-Apr. PubMed 28203175 ↗
  • Byrd JC, Wierda WG, Schuh A, Devereux S, Chaves JM, Brown JR, Hillmen P, Martin P, Awan FT, Stephens DM, Ghia P, Barrientos J, Pagel JM, Woyach JA, Burke K, Covey T, Gulrajani M, Hamdy A, Izumi R, Frigault MM, Patel P, Rothbaum W, Wang MH, O'Brien S, Furman RR. Acalabrutinib monotherapy in patients with relapsed/refractory chronic lymphocytic leukemia: updated phase 2 results. Blood. 2020 Apr 9;135(15):1204-1213. doi: 10.1182/blood.2018884940. PubMed 31876911 ↗
  • Robak E, Robak T. Bruton's Kinase Inhibitors for the Treatment of Immunological Diseases: Current Status and Perspectives. J Clin Med. 2022 May 16;11(10):2807. doi: 10.3390/jcm11102807. PubMed 35628931 ↗
  • Guo Y, Liu Y, Hu N, Yu D, Zhou C, Shi G, Zhang B, Wei M, Liu J, Luo L, Tang Z, Song H, Guo Y, Liu X, Su D, Zhang S, Song X, Zhou X, Hong Y, Chen S, Cheng Z, Young S, Wei Q, Wang H, Wang Q, Lv L, Wang F, Xu H, Sun H, Xing H, Li N, Zhang W, Wang Z, Liu G, Sun Z, Zhou D, Li W, Liu L, Wang L, Wang Z. Discovery of Zanubrutinib (BGB-3111), a Novel, Potent, and Selective Covalent Inhibitor of Bruton's Tyrosine Kinase. J Med Chem. 2019 Sep 12;62(17):7923-7940. doi: 10.1021/acs.jmedchem.9b00687. Epub 2019 Aug 19. PubMed 31381333 ↗

Individual participant data

Plan to share: No

08

Registry details

Key details

Study ID
NCT05922839
Lead sponsor
Chen Miao
Responsible party
Chen Miao (associate professor, Peking Union Medical College Hospital) — Sponsor-investigator
First posted
Jun 28, 2023
Start date
Nov 11, 2023
Primary completion
Dec 2024 (estimated)
Completion
Dec 2025 (estimated)
Last update
Apr 26, 2024

Study contacts

Miao Chen
Contact
chenm@pumch.cn
+86 13520112578
Bing Han
Contact
hanbingpumch@163.com

Oversight

Data monitoring committee
No
FDA-regulated drug
No
FDA-regulated device
No
View the source record on ClinicalTrials.gov ↗

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