Retrospective study , To analyze the clinical features and treatment outcomes in Korean blastic plasmacytoid dendritic cell neoplasm.
Blastic plasmacytoid dendritic cell neoplasm (BPDCN), with a synonym of blastic NK-cell lymphoma, agranular CD4+ natural killer cell leukaemia, blastic natural killer leukaemia/lymphoma, and agranular CD4+CD56+ haematodermic neoplasm/tumour, has been classified under "acute myeloid leukemia (AML) and related precursor neoplasms" since 2008 according to the World Health Organization (WHO) classification and among "myeloid neoplasm and acute leukemia" following 2016 revision of WHO classification. The plasmacytoid dendritic cells originates professional type I interferon-producing cells or plasmacytoid monocytes. Therefore, the prerequisite for diagnosis of BPDCN is the CD4+ and CD 56+ co-expression without common lymphoid or myeloid lineage markers1,2. This rare type of malignancy affecting predominantly elderly man, is reported to comprise 0.44% of hematologic malignancy3 and 0.7% of cutaneous lymphomas4, and the leukemic presentation or transformation is observed at initial presentation or even in the course of disease progression5. Skin in¬volvement is a predominant clinical feature of BPDCN ranging in appearance from small bruise-like areas to patches, nodules, and ulcerated masses, but lymphadenopathy, splenomegaly, hepatomegaly are also commonly observed. There is no definite treatment guideline for BPDCN. Retrospective studies including acute myeloid leukemia (AML) or acute lymphoblastic leukemia (ALL)/lymphoma-like chemotherapy for management of BPDCN reported 53-89% of high complete remission rates but an eventual very poor overall survival of 12-23 months, with a preponderance of ALL/lymphoma- over AML-like treatment5. Recently, targeted therapy with SL401, an IL-3 fusion protein which binds to CD123, is promising and the results of the clinical trial will be unveiled in the near future6. Although several retrospective and small case series has been published so far7,8, there is still no multicenter study on BPDCN classified after 2008 WHO classification in Asian population. This study aims to retrospectively collect data of BPDCN patients from centers participating the Consortium for improving survival of lymphoma (CISL) and analyze the clinical features and treatment outcomes in this rare type of hematologic malignancy.
Study Type
OBSERVATIONAL
Enrollment
36
Samsung medical center
Seoul, Gang Nam, South Korea
Overall Survival Rate
From the date of diagnosis to the date of death, or from the date of diagnosis to the last follow-up date.
Time frame: from the date of the IRB approval until June 30, 2019
Therapeutic Response Rate
Therapeutic response analysis is based on the evaluation of the response of common leukemia and lymphoma
Time frame: from the date of the IRB approval until June 30, 2019
Disease-free Survival Rate
the time from the treatment start date until the patient recurs.
Time frame: from the date of the IRB approval until June 30, 2019
Number of Factors affecting overall survival
multivariate analysis of age, ECOG, Involving organs, Response to treatment, Treatment, Autologous transplantation/Allogeneic transplantation affecting overall survival
Time frame: from the date of the IRB approval until June 30, 2019
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