title: Nodal and extranodal tumor-forming accumulation of plasmacytoid monocytes/interferon-producing cells associated with myeloid disorders.
abstract: Nodal tumor-forming accumulations of plasmacytoid monocytes/interferon-producing cells (PMs/IPCs) have been described in patients with myeloproliferative disorders. Here we report a series of 9 additional cases of such association. The patients were predominantly adult (median, 62 years), males (male/female ratio, 7:2), who presented with chronic myelomonocytic leukemia (4 cases), acute myeloid leukemia (1), acute monocytic leukemia (2), unclassifiable chronic myeloproliferative (1), or myeloproliferative/myelodysplastic disease (1). The prognosis was poor (median survival, 24 months) and related to progression of the underlying myeloid neoplasm. We found that in addition to lymph nodes, PMs/IPCs accumulated to bone marrow (8 cases) and skin (4 cases). Immunohistochemical markers typically expressed by PMs/IPCs (CD68, CLA/HECA452, CD123) were found in all cases and shown useful to identify cells with variations from classic morphology. In addition, PMs/IPCs expressed the interferon-alpha (IFN-alpha) inducible protein MxA, the B-cell oncogene TCL1, and granzyme B. The biologic and clinical significance of the association between PMs/IPCs and myeloid disorders remains not clarified. Using fluorescence in situ hybridization analysis in a case known to harbor monosomy 7 in the myeloid leukemia, we demonstrated that PMs/IPCs share the same chromosomal abnormality, thus indicating that they are clonal, neoplastic in nature, and closely related to the associated myeloid tumor. Recently, a novel CD56+ hematologic neoplasm has been reported and retained to stem from PMs/IPCs. The majority of PMs/IPCs in the present series failed to express CD56, thus indicating that variants of PMs/IPCs neoplasms exist, which might represent parts of a spectrum.
authors.affiliation: Department of Pathology, University of Brescia, Brescia, Italy.
authors.forename: William
authors.initials: W
authors.lastname: Vermi
authors.forename: Fabio
authors.initials: F
authors.lastname: Facchetti
authors.forename: Stefano
authors.initials: S
authors.lastname: Rosati
authors.forename: Federica
authors.initials: F
authors.lastname: Vergoni
authors.forename: Elisa
authors.initials: E
authors.lastname: Rossi
authors.forename: Silvana
authors.initials: S
authors.lastname: Festa
authors.forename: Daniele
authors.initials: D
authors.lastname: Remotti
authors.forename: Piergiovanni
authors.initials: P
authors.lastname: Grigolato
authors.forename: Giovannino
authors.initials: G
authors.lastname: Massarelli
authors.forename: Glauco
authors.initials: G
authors.lastname: Frizzera
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D014408:Biomarkers, Tumor / Q000378:metabolism; D001853:Bone Marrow / Q000378:metabolism / Q000473:pathology; D002999:Clone Cells; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D006801:Humans; D007124:Immunoenzyme Techniques; D017404:In Situ Hybridization, Fluorescence; D007372:Interferons / Q000378:metabolism*; D007399:Interphase; D007938:Leukemia / Q000378:metabolism / Q000473:pathology*; D008198:Lymph Nodes / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009000:Monocytes / Q000378:metabolism / Q000473:pathology*; D009196:Myeloproliferative Disorders / Q000378:metabolism / Q000473:pathology*; D010950:Plasma Cells / Q000378:metabolism / Q000473:pathology*; D012867:Skin / Q000378:metabolism / Q000473:pathology
chemical_list: D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D007372:Interferons