Contributions
Abstract: PB1704
Type: Publication Only
Background
Blastic plasmocytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive haematological malignancy that originates from clonal proliferation of plasmocytoid dendritic cells and their precursors. BPDCN is rare, represents less than 1% of acute leukemias. The disease has two patterns of presentations: cutaneus and leukemic. The main histological differential diagnosis includes: cutaneous NK/T-cell lymphoma; cutaneous T-cell lymphoma with co-expression of CD56 and CD56+ acute myeloid leukemia with monocytic differentiation.
Aims
The aim of study was to analyze heterogeneity of BPDCN differential diagnosis, especially with regards to clinical, immunological and cytomorphological characteristics of blastoid cells in terms of the optimal treatment.
Methods
During period 2010-2016. at the Clinic of Hematology, eight patients with BPDCN were diagnosed (M/F 6/2; average age 38 yrs, range 26-60yrs). In the blood count, average concentration of Hb was 108g/l (range 87-154); WBC 6,38x10e9/l (range 2,6-12); Plt 147,8x10e9/l (range 20-282). Hemorrhagic diathesis was registered in 3/8; splenomegaly in 6/8 (average diameter by ultrasound exam 140mm, 110-150mm); and hepatomegaly existed in 3/8 pts (average diameter 166mm, 140-200mm). Cutaneous infiltrations were present in 5/8 pts as livid maculopapular rash along lower extremities in 5 pts, and in 1 female pts in the breast region of 1-4cm diameter. In all 5 pts, immunocytochemistry confirmed BPDCN diagnosis. In the bone marrow aspirates of 7/8 pts, average 75% infiltration (27-89%) with blasts was revealed. Cells were of median size, with high nucleus cytoplasm ratio, with visible oval or slightly imprinted nucleoli. Basic immunophenotype profile was characterised by expression of CD56+CD4+CD123+high CD45RA+, and negativity for CMPO- cCD79a- cCD3c- in 4 cases. Immunohistochemitry staining in the rest of 4 pts, characterized with dry aspiration, revealed LCA+CD43+CD56+CD4+CD33+ positivity and MPO- TDt-CD34 -CD117- CD68- HLADR- negativity. Only 1 pts had CD4 negativity. Cytogenetic analysis revealed normal karyotype in 4 pts, while the rest of 4 pts had pathological findings: 1. 92,XX, XY; 2. 80-120,XXYY,+ mar (16)/46XY; 3. 46XY,del 5q/46XY; and 4. 46XX,del 12p, respectively.
Results
Four pts were treated with 3+7 chemotherapy. Complete remission (CR) was achieved in 3 pts, and treatment was continued according to the HIDAC and IDAC protocol. The duration of remission was 3, 8 and 11 months respectively, followed with relapse and letal outcome. One of the pts died within first 0,5 months after BPDCN was diagnosed. Three pts, treated with HyperCVAD, are alive and in CR with duration of 1, 3 and 10 months respectively. The continuation of the treatment within the programme of allogeneic stem cell transplantation is planned in 2 pts.
Conclusion
BPDCN diagnostics is difficult due to the heterogeneity of immunological characteristics of disease. Aggressive course of disease with median survival of 12-18 months, in the view of the unique treatment recommendations indicates necessity of further clinical investigations on larger patients groups.
Session topic: 4. Acute myeloid leukemia - Clinical
Keyword(s): Treatment, prognosis, Diagnosis, Acute Myeloid Leukemia
Abstract: PB1704
Type: Publication Only
Background
Blastic plasmocytoid dendritic cell neoplasm (BPDCN) is a clinically aggressive haematological malignancy that originates from clonal proliferation of plasmocytoid dendritic cells and their precursors. BPDCN is rare, represents less than 1% of acute leukemias. The disease has two patterns of presentations: cutaneus and leukemic. The main histological differential diagnosis includes: cutaneous NK/T-cell lymphoma; cutaneous T-cell lymphoma with co-expression of CD56 and CD56+ acute myeloid leukemia with monocytic differentiation.
Aims
The aim of study was to analyze heterogeneity of BPDCN differential diagnosis, especially with regards to clinical, immunological and cytomorphological characteristics of blastoid cells in terms of the optimal treatment.
Methods
During period 2010-2016. at the Clinic of Hematology, eight patients with BPDCN were diagnosed (M/F 6/2; average age 38 yrs, range 26-60yrs). In the blood count, average concentration of Hb was 108g/l (range 87-154); WBC 6,38x10e9/l (range 2,6-12); Plt 147,8x10e9/l (range 20-282). Hemorrhagic diathesis was registered in 3/8; splenomegaly in 6/8 (average diameter by ultrasound exam 140mm, 110-150mm); and hepatomegaly existed in 3/8 pts (average diameter 166mm, 140-200mm). Cutaneous infiltrations were present in 5/8 pts as livid maculopapular rash along lower extremities in 5 pts, and in 1 female pts in the breast region of 1-4cm diameter. In all 5 pts, immunocytochemistry confirmed BPDCN diagnosis. In the bone marrow aspirates of 7/8 pts, average 75% infiltration (27-89%) with blasts was revealed. Cells were of median size, with high nucleus cytoplasm ratio, with visible oval or slightly imprinted nucleoli. Basic immunophenotype profile was characterised by expression of CD56+CD4+CD123+high CD45RA+, and negativity for CMPO- cCD79a- cCD3c- in 4 cases. Immunohistochemitry staining in the rest of 4 pts, characterized with dry aspiration, revealed LCA+CD43+CD56+CD4+CD33+ positivity and MPO- TDt-CD34 -CD117- CD68- HLADR- negativity. Only 1 pts had CD4 negativity. Cytogenetic analysis revealed normal karyotype in 4 pts, while the rest of 4 pts had pathological findings: 1. 92,XX, XY; 2. 80-120,XXYY,+ mar (16)/46XY; 3. 46XY,del 5q/46XY; and 4. 46XX,del 12p, respectively.
Results
Four pts were treated with 3+7 chemotherapy. Complete remission (CR) was achieved in 3 pts, and treatment was continued according to the HIDAC and IDAC protocol. The duration of remission was 3, 8 and 11 months respectively, followed with relapse and letal outcome. One of the pts died within first 0,5 months after BPDCN was diagnosed. Three pts, treated with HyperCVAD, are alive and in CR with duration of 1, 3 and 10 months respectively. The continuation of the treatment within the programme of allogeneic stem cell transplantation is planned in 2 pts.
Conclusion
BPDCN diagnostics is difficult due to the heterogeneity of immunological characteristics of disease. Aggressive course of disease with median survival of 12-18 months, in the view of the unique treatment recommendations indicates necessity of further clinical investigations on larger patients groups.
Session topic: 4. Acute myeloid leukemia - Clinical
Keyword(s): Treatment, prognosis, Diagnosis, Acute Myeloid Leukemia