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Updated: Sep 12, 2025

Culture of myeloid dendritic cells from bone marrow precursors
Published on: July 25, 2008
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.
Abstract:
The present study describes a rare case of plasmacytoid dendritic cell-associated acute myeloid leukemia (pDC-AML). A 70-year-old male patient was diagnosed with pDC-AML and underwent induction chemotherapy using the venetoclax + azacitidine (VA) regimen. After 3 weeks of treatment, bone marrow examination indicated a morphologic leukemia-free state (MLFS); however, the patient experienced persistent cytopenia, which was further complicated by severe pneumonia and gastrointestinal bleeding, both of which improved following treatment. After 3 weeks in MLFS, bone marrow morphology and minimal residual disease analysis revealed a relapse of leukemia. The patient subsequently underwent treatment with selinexor in conjunction with the VA regimen; however, due to severe thrombocytopenia, the family decided to discontinue further treatment. The patient subsequently succumbed shortly after discharge. pDC-AML is an extremely rare disease characterized by low complete remission rates and a poor prognosis. While the VA regimen demonstrates rapid efficacy and favorable safety in elderly patients, especially those unable to tolerate intensive chemotherapy, the risk of relapse remains substantial. CD123-targeted therapies may present potential new therapeutic options for this disease. Improving remission rates and extending survival in patients with pDC-AML remain pressing clinical challenges.
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.
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