Ann Lab Med.  2014 Mar;34(2):148-151. 10.3343/alm.2014.34.2.148.

A Rare Case of Acute Leukemic Presentation of Blastic Plasmacytoid Dendritic Cell Neoplasm without Cutaneous Lesions

• Yu S
• Kwon MJ
• Kim K
• Koo DH
• Woo HY
• Park H

• KMID: 1791909
• DOI: http://doi.org/10.3343/alm.2014.34.2.148

Abstract

No abstract available.

MeSH Terms

Acute Disease
Adult
Antigens, CD4/metabolism
Antigens, CD45/metabolism
Antigens, CD56/metabolism
Bone Marrow Cells/cytology
Flow Cytometry
Hematologic Neoplasms/*diagnosis/pathology
Humans
Interleukin-3 Receptor alpha Subunit/metabolism
Lymph Nodes/metabolism/pathology
Male
Tomography, X-Ray Computed
Antigens, CD4
Antigens, CD45
Antigens, CD56
Interleukin-3 Receptor alpha Subunit

Figure

• 

  Fig. 1 (A) Peripheral blood smear (Wright-Giemsa stain, ×1,000): large atypical cells with high nuclear cytoplasmic (N/C) ratios, basophilic cytoplasm, and prominent nucleoli accounted for up to 25% of the leukocyte population. (B) In the bone marrow aspirate (Wright-Giemsa stain, ×1,000), 61.4% of all nucleated cells were atypical in appearance.

• 

  Fig. 2 Flow cytometric analysis of the bone marrow aspirate. CD45dim cells within the blast gated cells were negative for CD34 and positive for CD56, CD33, and CD7 (red circles).

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  Fig. 3 Immunohistochemical staining of the right supraclavicular lymph nodes biopsy. (A) CD4+, (B) CD56+, (C) leukocyte common antigen (LCA)+, and (D) CD123+.

Reference

1. Feuillard J, Jacob MC, Valensi F, Maynadie M, Gressin R, Chaperot L, et al. Clinical and biologic features of CD4(+)CD56(+) malignancies. Blood. 2002; 99:1556–1563. PMID: 11861268.

2. Vardiman JW, Thiele J, Arber DA, Brunning RD, Borowitz MJ, Porwit A, et al. The 2008 revision of the World Health Organization (WHO) classification of myeloid neoplasms and acute leukemia: rationale and important changes. Blood. 2009; 114:937–951. PMID: 19357394.

3. Burg G, Kempf W, Cozzio A, Feit J, Willemze R, E SJ, et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol. 2005; 32:647–674. PMID: 16293178.

4. Cota C, Vale E, Viana I, Requena L, Ferrara G, Anemona L, et al. Cutaneous manifestations of blastic plasmacytoid dendritic cell neoplasm-morphologic and phenotypic variability in a series of 33 patients. Am J Surg Pathol. 2010; 34:75–87. PMID: 19956058.

5. Leroux D, Mugneret F, Callanan M, Radford-Weiss I, Dastugue N, Feuillard J, et al. CD4(+), CD56(+) DC2 acute leukemia is characterized by recurrent clonal chromosomal changes affecting 6 major targets: a study of 21 cases by the Groupe Francais de Cytogenetique Hematologique. Blood. 2002; 99:4154–4159. PMID: 12010820.

6. Lucioni M, Novara F, Fiandrino G, Riboni R, Fanoni D, Arra M, et al. Twenty-one cases of blastic plasmacytoid dendritic cell neoplasm: focus on biallelic locus 9p21.3 deletion. Blood. 2011; 118:4591–4594. PMID: 21900200.

7. Wiesner T, Obenauf AC, Cota C, Fried I, Speicher MR, Cerroni L. Alterations of the cell-cycle inhibitors p27(KIP1) and p16(INK4a) are frequent in blastic plasmacytoid dendritic cell neoplasms. J Invest Dermatol. 2010; 130:1152–1157. PMID: 19924135.

8. Toya T, Nishimoto N, Koya J, Nakagawa M, Nakamura F, Kandabashi K, et al. The first case of blastic plasmacytoid dendritic cell neoplasm with MLL-ENL rearrangement. Leuk Res. 2012; 36:117–118. PMID: 21831428.

9. Wang H, Cao J, Hong X. Blastic plasmacytoid dendritic cell neoplasm without cutaneous lesion at presentation: case report and literature review. Acta Haematol. 2012; 127:124–127. PMID: 22236870.

10. Bekkenk MW, Jansen PM, Meijer CJ, Willemze R. CD56+ hematological neoplasms presenting in the skin: a retrospective analysis of 23 new cases and 130 cases from the literature. Ann Oncol. 2004; 15:1097–1108. PMID: 15205205.

11. Rauh MJ, Rahman F, Good D, Silverman J, Brennan MK, Dimov N, et al. Blastic plasmacytoid dendritic cell neoplasm with leukemic presentation, lacking cutaneous involvement: Case series and literature review. Leuk Res. 2012; 36:81–86. PMID: 21890199.

12. Ham JC, Janssen JJ, Boers JE, Kluin PM, Verdonck LF. Allogeneic stem-cell transplantation for blastic plasmacytoid dendritic cell neoplasm. J Clin Oncol. 2012; 30:e102–e103. PMID: 22331943.