Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a highly malignant hematological tumor derived from plasmacytoid dendritic cells (pDC) precursor cells, which has both lymphoma and leukemia characteristics, and is clinically rare. The disease easily involves the bone marrow, with low long-term survival rate and poor prognosis. Skin lesions can be seen in most BPDCN patients as the fi rst symptom, and their skin manifestations lack speci fi city, so is easy to be misdiagnosed and missed in clinic. High dose induction chemotherapy combined with hematopoietic stem cell transplantation (HSCT) are the main treatment methods for BPDCN,and new targeted therapies and immunotherapeutic drugs have also been put into clinical use. The de fi nition, epidemiology, pathogenesis,diagnosis and differential diagnosis, the latest research progress of treatment and prognosis of BPDCN have been summarized and discussed in present article, aiming to provide new ideas and references for the clinical diagnosis and treatment of BPDCN.
| 科 Family | 属数 Number of genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) | 属 Genus | 种数 Number of species | 占总种数比例 Percentage of total species (%) |
|---|---|---|---|---|---|---|
| 鹅膏菌科Amanitaceae | 2 | 11 | 5.26 | 鹅膏菌属 Amanita | 10 | 4.78 |
| 小菇科 Mycenaceae | 2 | 12 | 5.74 | 丝盖伞属 Inocybe | 5 | 2.39 |
| 多孔菌科 Polyporaceae | 8 | 14 | 6.70 | 蜡蘑属 Laccaria | 5 | 2.39 |
| 红菇科 Russulaceae | 3 | 23 | 11.00 | 小皮伞属 Marasmius | 6 | 2.87 |
| 小菇属 Mycena | 11 | 5.26 | ||||
| 光柄菇属 Pluteus | 5 | 2.39 | ||||
| 红菇属 Russula | 17 | 8.13 | ||||
| 栓菌属 Trametes | 5 | 2.39 |