Blastic Plasmacytoid Dendritic Cell Neoplasm Clinical Trial
Official title:
Blastic Plasmacytoid Dendritic Cell Neoplasm in Korean Population: A Multicenter Study
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.
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