What is the next step in treatment for a patient with a karyotype of 46 XX and residual trilineage dysplasia after 1 year of azacitidine (subcutaneous) and venetoclax (oral) therapy?

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Treatment Approach for MDS with Residual Dysplasia After 1 Year of Azacitidine-Venetoclax

Proceed to allogeneic hematopoietic cell transplantation (allo-HCT) evaluation immediately, as this patient has achieved cytogenetic normalization (46,XX karyotype) but retains residual trilineage dysplasia after 1 year of therapy—representing an optimal window for transplant with reduced disease burden. 1

Rationale for Transplant Evaluation

  • The NCCN 2025 MDS guidelines explicitly recommend allo-HCT for patients who achieve response to hypomethylating agents (HMAs) with or without venetoclax, particularly when blast counts are controlled. 1 Your patient has achieved cytogenetic response (normal karyotype) which represents meaningful disease control.

  • Most transplant centers require evidence of response to HMAs before proceeding to allo-HCT when baseline blasts are >10%. 1 The normalization of karyotype from presumably abnormal baseline constitutes such a response.

  • Continuing azacitidine-venetoclax as maintenance therapy while arranging transplant logistics is appropriate, as patients with clinical benefit should continue HMA treatment. 1

Critical Decision Points

If Transplant-Eligible:

  • Early referral for transplant evaluation is recommended to allow efficient transition to transplant. 1
  • Continue azacitidine-venetoclax as bridging therapy until transplant, as the regimen has demonstrated disease control evidenced by cytogenetic normalization. 1
  • Pre-transplant debulking to reduce marrow blasts to <5% is recommended to reduce post-transplant relapse risk. 1

If Transplant-Ineligible or Patient Declines:

  • Continue azacitidine-venetoclax indefinitely as maintenance therapy since the patient has achieved clinical benefit (cytogenetic response). 1
  • Re-evaluation with bone marrow and/or molecular testing is recommended to monitor for progression. 1

Monitoring Strategy During Continued Therapy

  • Perform bone marrow re-evaluation periodically to assess blast percentage and dysplasia progression. 1
  • Monitor for treatment failure, defined as loss of response or disease progression, which would trigger consideration of alternative therapies. 1
  • If IDH1 mutation is present and not previously tested, ivosidenib or olutasidenib may be considered if progression occurs. 1

Common Pitfalls to Avoid

  • Do not discontinue azacitidine-venetoclax simply because residual dysplasia persists—morphologic dysplasia can remain despite cytogenetic response and does not necessarily indicate treatment failure. 1
  • Do not delay transplant evaluation waiting for complete morphologic remission—cytogenetic normalization represents sufficient response to proceed. 1
  • Do not assume treatment failure based solely on persistent dysplasia without evidence of increasing blasts or cytogenetic relapse. 1

Alternative Considerations if Disease Progresses

  • For patients with mIDH1 mutations: single-agent ivosidenib, olutasidenib, or azacitidine combined with olutasidenib followed by allo-HCT. 1
  • Enrollment in clinical trials is recommended for patients without actionable mutations who progress. 1
  • High-intensity chemotherapy may be considered as bridge to transplant in select cases. 1

References

Guideline

Guideline Directed Topic Overview

Dr.Oracle Medical Advisory Board & Editors, 2025

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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