Blastic plasmacytoid dendritic cell neoplasm with absolute monocytosis at presentation

Blastic plasmacytoid dendritic cell neoplasm is an uncommon malignancy derived from precursors of plasmacytoid dendritic cells. Nearly all patients present initially with cutaneous manifestations, with many having extracutaneous disease additionally. While response to chemotherapy initially is effec...

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Published inPathology and laboratory medicine international Vol. 7; no. default; pp. 7 - 10
Main Authors Jaworski, Joseph M, Swami, Vanlila K, Heintzelman, Rebecca C, Cusack, Carrie A, Chung, Christina L, Peck, Jeremy, Fanelli, Matthew, Styler, Micheal, Rizk, Sanaa, Hou, J.Steve
Format Journal Article
LanguageEnglish
Published Macclesfield Dove Medical Press Limited 01.01.2015
Taylor & Francis Ltd
Dove Medical Press
Subjects
Abdomen
Blood
Bone marrow
Chemotherapy
Dendritic cells
Flow cytometry
Immunohistochemistry
Leukemia
Lymphoma
Medical prognosis
Medicine
Monocytes
Patients
Stains & staining
Stem cells
Transplants & implants
Tumors
University colleges
United States > US
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Summary:Blastic plasmacytoid dendritic cell neoplasm is an uncommon malignancy derived from precursors of plasmacytoid dendritic cells. Nearly all patients present initially with cutaneous manifestations, with many having extracutaneous disease additionally. While response to chemotherapy initially is effective, relapse occurs in most, with a leukemic phase ultimately developing. The prognosis is dismal. While most of the clinical and pathologic features are well described, the association and possible prognostic significance between peripheral blood absolute monocytosis (>1.0 K/µL) and blastic plasmacytoid dendritic cell neoplasm have not been reported. We report a case of a 68-year-old man who presented with a rash for 4-5 months. On physical examination, there were multiple, dull-pink, indurated plaques on the trunk and extremities. Complete blood count revealed thrombocytopenia, absolute monocytosis of 1.7 K/µL, and a negative flow cytometry study. Biopsy of an abdominal lesion revealed typical features of blastic plasmacytoid dendritic cell neoplasm. Patients having both hematologic and nonhematologic malignancies have an increased incidence of absolute monocytosis. Recent studies examining Hodgkin and non-Hodgkin lymphoma patients have suggested that this is a negative prognostic factor. The association between blastic plasmacytoid dendritic cell neoplasm and absolute monocytosis has not been described and may, in fact, have prognostic value. Keywords: blastic, plasmacytoid, dendritic, monocytosis
ISSN:1179-2698
1179-2698
DOI:10.2147/PLMI.S71492