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Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion

Marco Lucioni, Francesca Novara, Giacomo Fiandrino, Roberta Riboni, Daniele Fanoni, Mariarosa Arra, Luigia Venegoni, Marta Nicola, Elena Dallera, Luca Arcaini, Francesco Onida, Pamela Vezzoli, Erica Travaglino, Emanuela Boveri, Orsetta Zuffardi, Marco Paulli and Emilio Berti

Data supplements

Article Figures & Data

Figures

  • Figure 1

    Copy number alterations detected by aCGH. Summary of the chromosomal losses and gains detected by aCGH in our patients (chromosomes are shown as ideograms). The positions of oligomers refer to Human Genome March 2006 assembly (hg18). In each experiment, log2 ratios estimated the percentage of anomalous cells for each copy number alteration. Green bars on the left side of each chromosome represent chromosomal losses; and red bars, chromosomal gains. The width of the bars is proportional to the occurrence of a given anomaly in the 21 analyzed samples.

Tables

  • Table 1

    Clinical picture, therapy, and follow-up for each patient

    Case no.Age (median 64 y)/sex ratio (5:2 male/female)Sites of involvement at diagnosisInitial treatmentResponseRelapse, monthsSites of relapseTherapy at relapseFollow-up, months
    Skin (n = 21)BM (n =10)Peripheral blood (n =9)Lymph node (n =5)Other (n =2)
    164/MMu++ALL-type*CR11S/BM/BCHTDOD 23
    283/MD+++NoneDOD 3
    338/MMu+SCTCR17S/BM/B/LyCHTDOD 20
    467/FDALL-type*PR5SCHTAWD 11
    584/FDmCHTPR4BDOD 6
    664/MD++ALL-typeCR10S/BM/BCHTDOD 26
    719/MMuNHL-type§CR36SSCTAWD 72
    862/MMu+++ALL-type*CRADF 13
    961/MMuNHL-type§CR30S/BM/B/LyCHTDOD 35
    1083/MD++MALTNHL-type§PR8S/BM/B/OCHTDOD 10
    1140/MLmCHT + RTPR20BM/BCHTDOD 30
    1260/MD+SCTCRDTR 60
    1376/MLRTCR8BMCHTAWD 13
    149/FD++ALL-type*CRADF 12
    1558/MDALL-type*CRADF 28
    1679/MLRTCRADF 14
    1775/FDALL-type*CRADF 8
    1839/FLALL-type*CRDTR 12
    1966/FMu++NHL-type§PR5S/BM/B/OCHTDOD 20
    2064/MD+++MALTALL-type*PR4S/BSCTDOD 12
    2183/ML+++NoneDOD 1
    • BM indicates bone marrow; Mu, multiple noncontiguous skin lesions; D, diffuse skin involvement; L, localized skin disease; Ly, lymph node; MALT, mucosa-associated lymphoid tissue; O, other; B, peripheral blood; ALL, acute lymphoblastic leukemia; SCT, allogeneic stem cell transplantation; mCHT, monochemotherapy; NHL, non-Hodgkin lymphoma; RT, radiotherapy; CR, complete remission; —, not available; PR, partial remission; S, skin; CHT, chemotherapy; DOD, dead of disease; AWD, alive with disease; ADF, alive disease-free; and DTR, death therapy-related.

    • * Hyper-CVAD regimen (hyperfractionated cyclophosphamide, vincristine, doxorubicin, dexamethasone, alternating with high-dose methotrexate and cytarabine).

    • Hydroxyurea.

    • IVA regimen (ifosfamide, vincristine, actinomycin-D).

    • § CHOP regimen (cyclophosphamide, doxorubicin, vincristine, prednisolone).

    • This patient had skin-limited disease at diagnosis, but she refused treatment until 8 months later when bone marrow involvement developed.