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NCT Number: NCT03974971

Blastic Plasmacytoid Dendritic Cell Neoplasm in Korean Population.

Retrospective study , To analyze the clinical features and treatment outcomes in Korean blastic plasmacytoid dendritic cell neoplasm.

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Key information

Conditions

Blastic Plasmacytoid Dendritic Cell Neoplasm Hematologic Diseases Hematologic Neoplasms Hemic and Lymphatic Diseases Histiocytic Disorders, Malignant Leukemia Lymphoma Neoplasms Neoplasms by Histologic Type Neoplasms by Site Skin Diseases Skin Neoplasms Skin and Connective Tissue Diseases

Sex eligibility

All sexes

Study type

Observational

Primary location

Samsung medical center

Seoul, Gang Nam, 676, South Korea

About this study

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.

Who can participate

Healthy volunteers accepted: No

Only the study team can determine whether someone qualifies for participation.

<Inclusion Criteria>

  • Patients ≥ 18 years
  • Pathologically confirmed diagnosis by tissue or bone marrow at each center with
  • Blastic plasmacytoid dendritic cell neoplasm
  • Blastic NK-cell lymphoma
  • Agranular CD4+ natural killer cell leukaemia
  • Blastic natural killer leukaemia/lymphoma
  • Agranular CD4+CD56+ haematodermic neoplasm/tumour
  • Antigen expression of CD4 and/or CD56 coupled with at least one plasmacytoid dendritic cell-associated antigen among CD123, TCL1, CD2AP and BDCA2/CD303

<Exclusion Criteria>

  • Acute myeloid leukemia
  • Acute lymphoblastic leukemia
  • Mixed phenotype acute leukemia
  • Any type of B- or T-/NK/T-cell lymphomas
  • Expression of lineage-specific markers for B cells (CD20, CD79a) T cells (CD3) Myeloid cells (myeloperoxidase) Monocytes (CD11c, CD163, lysozyme). CD34

Treatment and study plan

Primary outcomes

  1. Overall Survival Rate

    Time frame: from the date of the IRB approval until June 30, 2019

    From the date of diagnosis to the date of death, or from the date of diagnosis to the last follow-up date.

Secondary outcomes

  1. Therapeutic Response Rate

    Time frame: from the date of the IRB approval until June 30, 2019

    Therapeutic response analysis is based on the evaluation of the response of common leukemia and lymphoma

  2. Disease-free Survival Rate

    Time frame: from the date of the IRB approval until June 30, 2019

    the time from the treatment start date until the patient recurs.

  3. Number of Factors affecting overall survival

    Time frame: from the date of the IRB approval until June 30, 2019

    multivariate analysis of age, ECOG, Involving organs, Response to treatment, Treatment, Autologous transplantation/Allogeneic transplantation affecting overall survival

Sponsors and collaborators

Lead sponsor

Samsung Medical Center

Other

Registry information

Official study title

Blastic Plasmacytoid Dendritic Cell Neoplasm in Korean Population: A Multicenter Study

Acronym: KBPDCN

Important dates

Study start
2019
Primary completion
2019
Study completion
2020
First posted
Jun 5, 2019
Registry last updated
Sep 3, 2020

OpenTrials presents study information sourced from ClinicalTrials.gov. The official registry record should be consulted for the latest information.

View the official ClinicalTrials.gov record (opens in a new tab)

This listing is for discovery and informational purposes only. It is not medical advice, does not guarantee that a study is recruiting, and does not determine eligibility. Contact the study team and a qualified healthcare professional when considering participation.

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