title: Primary Plasma Cell Leukemia Associated with t(6;14)(p21;q32) and IGH Rearrangement: A Case Study and Review of the Literature.
abstract: Because plasma cell leukemia (PCL) is a rare and distinct variant among plasma cell dyscrasias, recent clinical and cytogenetic studies have been performed in different ethnic groups to define the characteristics of these PCL patients. As far as we know, IGH rearrangements involving t(11;14) and (14;16) are significantly more frequent in PCL than in myeloma patients. However, PCL cases associated with t(6;14)(p21;q32) or IGH-CCND3 rearrangement are extremely rare in the literature; only one PCL case with t(6;14) has been documented. A 61-year-old female was admitted due to fatigue, weight loss, and exertional dyspnea. Plasmacytoid cells were counted up to 76% at a peripheral blood film, but bone marrow aspiration failed because of dry-tapping. Flow cytometric analysis showed positive for CD138 and cytoplasmic kappa light chain. Chromosome analysis revealed t(6;14)(p21;q32), which was confirmed by an IGH split-out probe in FISH analysis. Immunofixation electrophoresis also presented monoclonal bands identified as IgG and kappa light chain. Finally, she was diagnosed as primary PCL associated with t(6;14) and IGH rearrangement. Although considerable advances have been made in the understanding of the biology and molecular pathogenesis of PCL, further clinical, laboratory, and genetic studies of PCL associated with such a rare IGH rearrangement would be necessary in the future. To the best of our knowledge, this is the first report of PCL associated with t(6;14) as a sole chromosomal abnormality.
authors.affiliation: Department of Laboratory Medicine, School of Medicine, Kyung Hee University, Seoul, Korea.
authors.forename: Sun Young
authors.initials: SY
authors.lastname: Cho
authors.forename: Gayoung
authors.initials: G
authors.lastname: Lim
authors.forename: Seung Hwan
authors.initials: SH
authors.lastname: Oh
authors.forename: Hee Joo
authors.initials: HJ
authors.lastname: Lee
authors.forename: Jin-Tae
authors.initials: JT
authors.lastname: Suh
authors.forename: Juhie
authors.initials: J
authors.lastname: Lee
authors.forename: Woo-In
authors.initials: WI
authors.lastname: Lee
authors.forename: Hong Ghi
authors.initials: HG
authors.lastname: Lee
authors.forename: Hwi-Joong
authors.initials: HJ
authors.lastname: Yoon
authors.forename: Tae Sung
authors.initials: TS
authors.lastname: Park
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism; D001854:Bone Marrow Cells / Q000378:metabolism / Q000473:pathology; D002883:Chromosomes, Human, Pair 14; D002896:Chromosomes, Human, Pair 6; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D015321:Gene Rearrangement; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics*; D017404:In Situ Hybridization, Fluorescence; D007952:Leukemia, Plasma Cell / Q000097:blood / Q000235:genetics* / Q000473:pathology; D008875:Middle Aged; D053668:Syndecan-1 / Q000097:blood; D014178:Translocation, Genetic
chemical_list: D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D007143:Immunoglobulin Heavy Chains; C508241:SDC1 protein, human; D053668:Syndecan-1