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Case Reports
. 2016 Jan 20:2016:bcr2015214093.
doi: 10.1136/bcr-2015-214093.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN): a rare entity

Ming Sheng Lim  1 , Karla Lemmert  2 , Anoop Enjeti  1
Affiliations
Case Reports

Blastic plasmacytoid dendritic cell neoplasm (BPDCN): a rare entity

Ming Sheng Lim et al. BMJ Case Rep. .

Abstract

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive haematological malignancy in the elderly, with a high frequency of cutaneous and bone marrow involvement and poor prognosis. We report a case of BPDCN with classic presentation and discuss its treatment and the value of different investigation tools used in diagnosis and response assessment.

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Figures

Figure 1
Figure 1
(A) Photograph showing multiple bruise-like or violaceous nodules and plaques on the (A) trunk and (B) face. (B) Photograph showing multiple bruise-like or violaceous nodules and plaques on the (A) trunk and (B) face. (C) Close-up view of the bruise-like plaques on the trunk.
Figure 2
Figure 2
(A) Skin biopsy from the chest. (A) An extensive slightly patchy dermal infiltrate without epidermotrophism (H&E, ×40). (B) Skin biopsy from the chest. (B) High power view showing uniform cells with oval-shaped nuclei, fine chromatin and inconspicuous cytoplasm. (H&E, ×400).
Figure 3
Figure 3
(A) Preinduction bone marrow aspirate and trephine biopsy. Aspirate (A) showing a heterogeneous population of small to medium-sized cells with oval or irregular nuclei, fine chromatin, prominent nucleoli and scant to moderate basophilic agranular cytoplasm. (H&E, ×400). (B) Preinduction bone marrow aspirate and trephine biopsy. (B) Trephine showing a hypercellular marrow with extensive infiltration by a heterogeneous population of primitive cells. (H&E staining, ×400).
Figure 4
Figure 4
(A) Flow cytometric analysis of a bone marrow aspirate sample at diagnosis. The abnormal blast population is positive for (A) CD45 (dim), (B) CD4++, (C) HLA-Dr++, CD56++, (D) CD38+, (E) CD123+++. This population is negative for lineage markers including (F) MPO, CD79a, cytoplasmic CD3 and myeloid markers CD13, CD33, CD64 and CD14 (not shown).
Figure 5
Figure 5
(A) Postinduction bone marrow aspirate and trephine biopsy. (A and B) Significant reduction in blast percentage on bone marrow aspirate and trephine. (H&E, ×400). (B) Postinduction bone marrow aspirate and trephine biopsy. (A and B) Significant reduction in blast percentage on bone marrow aspirate and trephine. (H&E, ×400). (C) Postinduction bone marrow aspirate and trephine biopsy. (C) Immunostaining for CD123 reveals residual clusters of CD123 cells corresponding to the malignant blast cells seen on H&E (×400).
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References

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