Azacitidine maintenance therapy for blastic plasmacytoid dendritic cell neoplasm allograft: A case report.

BACKGROUND

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, highly invasive malignant neoplasm. There is no universally accepted standard of care because of its rarity and the dearth of prospective research. It is still challenging for some patients to achieve persistent clinical remission or cure, despite the success of allogeneic hematopoietic stem cell transplantation (allo-HSCT), indicating that there is still a significant recurrence rate. We report a case of prevention of BPDCN allograft recurrence by azacitidine maintenance therapy and review the relevant literature.

CASE SUMMARY: We report a 41-year-old man with BPDCN who was admitted to hospital due to skin sclerosis for > 5 mo' duration. BPDCN was diagnosed by combined clinical assessment and laboratory examinations. Following diagnosis, the patients underwent induction consolidation chemotherapy to achieve the first complete remission, followed by bridging allo-HSCT. Post-transplantation, azacitidine (75 mg/m2 for 7 d) was administered as maintenance therapy, with repeat administration every 4-6 wk and appropriate extension of the chemotherapy cycle. After 10 cycles, the patient has been disease free for 26 mo after transplantation. Regular assessments of bone marrow morphology, minimal residual disease, full donor chimerism, Epstein-Barr virus, and cytomegalovirus all yielded normal results with no abnormalities detected.

CONCLUSION

Azacitidine may be a safe and effective maintenance treatment for BPDCN following transplantation because there were no overt adverse events during the course of treatment.