Clinical Analysis on 41 Cases of BPDCN
RAO Jin
YIN Li
LIN Zhi-mei
LI Shi-yun
XIONG Ping
Abstract：Objective To investigate the clinical diagnosis, treatment and prognosis of BPDCN, and to improve the understanding and treatment of BPDCN. Methods Literatures on BPDCN, blastic natural-killer-cell lymphoma or CD4 +CD56+ haematodermic neoplasm published in journals between January 2009 and December 2013 were identified by searching Wanfang, PubMed and CNKI. A retrospective review was carried out. Results A total of 41 BPDCN patients were found from the 28 literatures, including 29 males, 12 females;the median age was 63. 7 years. Major clinical manifestations were skin lesions and often involving lymph nodes and bone marrow. The tumors were CD4 +, CD56 + and CD123 +. Lineage specific markers for B-and T-cell were negative and the tumors did not express myelopemxidase. 33 of the 41 patients accepted lymphoma, acute myeloid leukemia ( AML) , acute Lymphocytic leukemia-like chemothpy and single agent chemotherapy at-tained hematological remission, and 20 patients obtained complete remission. 15 patients were treated with allogeneic SCT, and 14 of the 15 patients obtained hematological remission. The median overall survival ( MS) time of 41cases was 12. 3 months. Conclusion BPDC hematodermic neoplasm is a rare hematopoietic malignancy, which primarily affects elderly pa-tients. The diagnosis of this disease is mainly based on the clinical presentations, pathologic and immunohistochemical fea-tures. Clinical course is rapidly and fatally aggressive. Patients with BPDCN usually have a poor prognosis.
Keywords：BPDCNSkin ManifestationsImmunophenotypingTreatment outcomePrognosis
Publication Date：2014-01-01
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：4( 25-28 )