Acute lymphoplasmacytoid dendritic cell (DC2) leukemia: results from the Hellenic Dendritic Cell Leukemia Study Group.
We present a cohort of 22 patients with type 2 dendritic cell (DC2) acute leukemia (or blastic plasmacytoid dendritic cell neoplasm-BPDCN, as it has been recently named), diagnosed in Greece over the past 12-year period, according to the main clinical and immunophenotypic features of this entity. Four additional cases are discussed, classified as leukemia of ambiguous lineage (LAL), because of the simultaneous detection of a CD56 negative DC2 population and of a second myeloid precursor cell population. The morphological features and cytogenetic findings of the typical BPDCN cases were similar to those previously described. Acute lymphoblastic leukemia-type chemotherapeutic regimens were more efficient in controlling the disease. Immunophenotyping of typical BPDCN cases revealed CD4(+), CD56(+), HLA-DR(+) and CD123(bright) neoplastic cells, in the absence of major B-, T- and myeloid-associated markers, while the phenotype of the four cases characterized as LAL highlights the risk of misdiagnosis. Based on our experience, we propose a flow cytometric algorithmic approach for the distinction of typical BPDCN from certain types of acute myeloid leukemia, but also for the identification of acute myeloid leukemia, admixed with CD56 negative DC2 cells, which could be misdiagnosed as BPDCN.
Tsagarakis
Nikolaos J
NJ
Flow Cytometry Laboratory, Department of Immunology, Athens Regional General Hospital G Gennimatas, Mesogion Avenue 154, Athens 11527, Greece. nikolaostsagarakis@gmail.com
Kentrou
Nektaria A
NA
Papadimitriou
Konstantinos A
KA
Pagoni
Maria
M
Kokkini
Garyfallia
G
Papadaki
Helen
H
Pappa
Vassiliki
V
Marinakis
Theodoros
T
Anagnostopoulos
Nikolaos I
NI
Vadikolia
Chrissanthi
C
Anagnostopoulos
Achilleas
A
Angelopoulou
Maria K
MK
Terpos
Evangelos
E
Poziopoulos
Christos
C
Anargyrou
Konstantinos
K
Rontogianni
Dimitra
D
Papadaki
Theodora
T
Psarra
Aikaterini
A
Kontopidou
Flora N
FN
Skoumi
Dimitra
D
Papadhimitriou
Stefanos I
SI
Paterakis
Georgios
G
D000208:Acute Disease; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D020732:Cytogenetic Analysis; D003713:Dendritic Cells / Q000473:pathology*; D003937:Diagnosis, Differential; D005260:Female; D006115:Greece; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D007938:Leukemia / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology / Q000473:pathology; D015470:Leukemia, Myeloid, Acute / Q000175:diagnosis / Q000235:genetics / Q000276:immunology; D008297:Male; D008875:Middle Aged; D012878:Skin Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000276:immunology / Q000473:pathology