Acute myeloid leukemia with plasmacytoid dendritic cell proliferation: A case report and literature review

Zhixin Pei1, Yi Zhang2, Han Xu3

  • 1Department of Hematology, Jiaozuo People's Hospital, Jiaozuo, Henan 454000, P.R. China.

Oncology Letters
|August 5, 2025
PubMed

Insights

This case study highlights a rare acute myeloid leukemia subtype, plasmacytoid dendritic cell-associated AML. Despite initial response to venetoclax + azacitidine, the patient relapsed, underscoring treatment challenges.

Area of Science:

  • Hematology
  • Oncology
  • Rare Diseases

Background:

  • Plasmacytoid dendritic cell-associated acute myeloid leukemia (pDC-AML) is an exceptionally rare hematologic malignancy.
  • Standard treatment protocols and prognosis for pDC-AML are not well-established due to its rarity.

Observation:

  • A 70-year-old male with pDC-AML initially achieved a morphologic leukemia-free state (MLFS) after venetoclax + azacitidine (VA) induction chemotherapy.
  • The patient experienced persistent cytopenia, complicated by severe pneumonia and gastrointestinal bleeding, requiring supportive care.
  • Leukemia relapse was detected 3 weeks post-MLFS, despite initial treatment response.

Findings:

  • The VA regimen showed rapid efficacy in achieving MLFS but was associated with a substantial risk of relapse.
  • Subsequent treatment with selinexor and VA was limited by severe thrombocytopenia.
  • The patient's case illustrates the poor prognosis and low complete remission rates characteristic of pDC-AML.

Implications:

  • Current treatment strategies for pDC-AML require further optimization to improve remission rates and survival.
  • CD123-targeted therapies represent a promising avenue for future pDC-AML treatment.
  • Managing complications such as cytopenia and infections is critical in elderly patients with pDC-AML receiving chemotherapy.