Clinical Characteristics and Treatment of Blastic Plasmacytoid Dendritic Cell Neoplasm

by Selleckchem | Mar 08 2023

Objective: To explore the clinical manifestations, diagnosis, treatment and prognosis of blastic plasmacytoid dendritic cell neoplasm(BPDCN).

Methods: The clinical features, bone marrow morphology and immunophenotyping, treatment and prognosis of 4 patients with BPDCN were analyzed retrospectively.

Results: 4 patients had bone marrow， spleen and lymph nodes involvement， 2 patients had skin lesions， and 3 patients had central nervous system infiltration． Tailing phenomenon of abnormally cells could be seen in bone marrow. The immunophenotyping showed that CD56, CD4 and CD123 expression was observed in 4 patients， and CD304 in 3 patients． One patient refused chemotherapy and died early. Both patients achieved complete remission after the initial treatment with DA+VP regimen, 1 of them achieved complete remission after recurrence by using the same regimen again． One patient failed to respond to reduced dose of DA+VP chemotherapy, and then achieved complete remission with venetoclax+azacitidine.

Conclusion: The malignant cells in BPDCN patients often infiltrate bone marrow, spleen and lymph nodes, and have specical phenotypes, with poor prognosis. The treatment should take into account both myeloid and lymphatic systems. The treatment containing new drugs such as BCL-2 inhibitors combined with demethylation drugs is worth trying.

Comments：

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive hematological malignancy that mainly affects elderly patients. The clinical manifestations of BPDCN are diverse and may involve multiple organs. Bone marrow involvement is the most common feature and patients may present with pancytopenia, while spleen and lymph nodes are also commonly affected. In addition, skin lesions, central nervous system infiltration and other symptoms may also be observed.

Diagnosis of BPDCN requires a combination of clinical, morphological and immunophenotypic features. In bone marrow aspirate, the tailing phenomenon of abnormal cells is a characteristic finding. Immunophenotyping is important for identifying the unique phenotypic markers of BPDCN, which include CD56, CD4, CD123 and CD304.

The treatment of BPDCN is challenging due to the rarity of the disease and lack of established guidelines. Combination chemotherapy regimens, such as DA+VP, have been used in clinical practice with varying success rates. Newer therapies such as venetoclax and demethylation drugs may be effective in patients who do not respond to standard chemotherapy. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is also an option for eligible patients.

Prognosis of BPDCN is generally poor, with a median overall survival of 12-14 months. Early diagnosis and timely treatment may improve the prognosis. Long-term survival is possible for some patients who achieve complete remission and undergo allo-HSCT.

In summary, BPDCN is a rare and aggressive hematological malignancy with diverse clinical manifestations and poor prognosis. The diagnosis of BPDCN requires a combination of clinical, morphological and immunophenotypic features. Treatment should be individualized based on the patient's age, disease status and other factors. Newer therapies and allo-HSCT may be effective in selected patients.