EHA Library - The official digital education library of European Hematology Association (EHA)

BLASTIC PLASMACYTOID DENDRITIC CELL NEOPLASMS - UNUSUAL PRESENTATIONS AND UNFAVOURABLE OUTCOMES
Author(s): ,
Margarita Guenova
Affiliations:
National Specialised Hospital for Active Treatment of Hematological Diseases,Sofia,Bulgaria
,
Antoaneta Michova
Affiliations:
National Specialised Hospital for Active Treatment of Hematological Diseases,Sofia,Bulgaria
,
Tihomir Dikov
Affiliations:
National Specialised Hospital for Active Treatment of Hematological Diseases,Sofia,Bulgaria
,
Yanitza Georgieva
Affiliations:
National Specialised Hospital for Active Treatment of Hematological Diseases,Sofia,Bulgaria
,
Branimir Spassov
Affiliations:
National Specialised Hospital for Active Treatment of Hematological Diseases,Sofia,Bulgaria
,
Kamelia Prisadashka
Affiliations:
Alexandrovska University Hospital,Sofia,Bulgaria
,
Maria Balabanova
Affiliations:
Alexandrovska University Hospital,Sofia,Bulgaria
,
Valeria Kaleva
Affiliations:
University Hospital St.Marina,Varna,Bulgaria
Gueorgui Balatzenko
Affiliations:
National Specialised Hospital for Active Treatment of Hematological Diseases,Sofia,Bulgaria
(Abstract release date: 05/18/17) EHA Library. Guenova M. 05/18/17; 182407; PB1693
Prof. Dr. Margarita Guenova
Prof. Dr. Margarita Guenova
Contributions
Abstract

Abstract: PB1693

Type: Publication Only

Background

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy with an aggressive clinical course. Most patients (pts) with BPDCN have skin lesions and involvement of the peripheral blood, bone marrow, and lymph nodes. Very few cases have been described with lack of skin and or bone marrow manifestations at the time of diagnosis.

Aims

To characterise the clinical presentation and clinical outcomes of a cohort of consecutive patients with a rare blastic plasmacytoid dendritic cell neoplasm in a single institution.

Methods
Patients diagnosed with BPDCN at the National Hematology Hospital between 2010 and 2016 were retrieved from the database. The diagnosis was confirmed by morphology and immunophenotying by flow cytometry and/or immunohistochemistry, according to 2008 WHO Classification of Hematopoietic Neoplasms. The relevant clinicopathologic features were reviewed.

Results
We identified 8 adult patients at a median age of 70 years (range: 37-84 years) with a male:female ratio of 6:2 (75%:25%) and only 1 child. Mean values of blood cell counts were as follows: WBC 5.109/L; hemoglobin 99 g/L; platelets 116.109/L. LDH was generally elevated with a mean of 962.8 U/L. At diagnosis the skin was invoved in 5/9 patients. Five patients developed leukemic presentation with 40-95% of bone marrow infiltration. Interestingly, in 4 pts (50% of adult pts) the initial presentation affected other tissues and organs such as testis, bronchial wall, stomach and periorbital soft tissues, however, only the latter one case presented with a leukemic picture. Biopsies revealed diffuse, monomorphous infiltrate of medium-sized blast cells with irregular nuclei, fine chromatin with ≥1 nucleoli, scant and agranular cytoplasm, without angioinvasion or coagulation necrosis. Immunophenotype generally demonstrated CD45+, CD4+, CD56+, CD123+. No standard therapies were applied. Patients received CHOP or HyperCVAD or AML-induction therapy. However, response rates in adult patients were low and the mean OS was 2.6 months (ranging from early deaths before any treatment could be initiated to 10 months).

Conclusion

BPDCN is a rare aggressive disease that typically affects elderly patients. The most commonly affected non-hematopoietic organ is the skin, however any other organ or tissues can also be involved. Response to therapy if any is relatively short and long-term prognosis is poor despite of the site of presentation. Larger scale studies are warranted to understand the pathophysiology of the disease and to find optimal management.
Acknowledgements: Partial support by the National Science Fund.

Session topic: 4. Acute myeloid leukemia - Clinical

Keyword(s): Leukemia, Dendritic cell, CD56, CD4

Abstract: PB1693

Type: Publication Only

Background

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematological malignancy with an aggressive clinical course. Most patients (pts) with BPDCN have skin lesions and involvement of the peripheral blood, bone marrow, and lymph nodes. Very few cases have been described with lack of skin and or bone marrow manifestations at the time of diagnosis.

Aims

To characterise the clinical presentation and clinical outcomes of a cohort of consecutive patients with a rare blastic plasmacytoid dendritic cell neoplasm in a single institution.

Methods
Patients diagnosed with BPDCN at the National Hematology Hospital between 2010 and 2016 were retrieved from the database. The diagnosis was confirmed by morphology and immunophenotying by flow cytometry and/or immunohistochemistry, according to 2008 WHO Classification of Hematopoietic Neoplasms. The relevant clinicopathologic features were reviewed.

Results
We identified 8 adult patients at a median age of 70 years (range: 37-84 years) with a male:female ratio of 6:2 (75%:25%) and only 1 child. Mean values of blood cell counts were as follows: WBC 5.109/L; hemoglobin 99 g/L; platelets 116.109/L. LDH was generally elevated with a mean of 962.8 U/L. At diagnosis the skin was invoved in 5/9 patients. Five patients developed leukemic presentation with 40-95% of bone marrow infiltration. Interestingly, in 4 pts (50% of adult pts) the initial presentation affected other tissues and organs such as testis, bronchial wall, stomach and periorbital soft tissues, however, only the latter one case presented with a leukemic picture. Biopsies revealed diffuse, monomorphous infiltrate of medium-sized blast cells with irregular nuclei, fine chromatin with ≥1 nucleoli, scant and agranular cytoplasm, without angioinvasion or coagulation necrosis. Immunophenotype generally demonstrated CD45+, CD4+, CD56+, CD123+. No standard therapies were applied. Patients received CHOP or HyperCVAD or AML-induction therapy. However, response rates in adult patients were low and the mean OS was 2.6 months (ranging from early deaths before any treatment could be initiated to 10 months).

Conclusion

BPDCN is a rare aggressive disease that typically affects elderly patients. The most commonly affected non-hematopoietic organ is the skin, however any other organ or tissues can also be involved. Response to therapy if any is relatively short and long-term prognosis is poor despite of the site of presentation. Larger scale studies are warranted to understand the pathophysiology of the disease and to find optimal management.
Acknowledgements: Partial support by the National Science Fund.

Session topic: 4. Acute myeloid leukemia - Clinical

Keyword(s): Leukemia, Dendritic cell, CD56, CD4

By clicking “Accept Terms & all Cookies” or by continuing to browse, you agree to the storing of third-party cookies on your device to enhance your user experience and agree to the user terms and conditions of this learning management system (LMS).

Cookie Settings
Accept Terms & all Cookies