title: Aberrant expression of CD30 in neoplastic mast cells in high-grade mastocytosis.
abstract: Systemic mastocytosis either presents as aggressive neoplasm with short survival time or indolent systemic mastocytosis with normal life expectancy. In both instances, neoplastic mast cells usually harbor the D816V-mutated variant of KIT. Phenotypically, mast cells in systemic mastocytosis usually express CD25. However, no robust marker that discriminates between aggressive and indolent variants of systemic mastocytosis has been identified yet. We here report that CD30, also known as Ki-1 antigen, is expressed in neoplastic mast cells in a majority of patients with advanced systemic mastocytosis (11/13, 85%), whereas in most patients with indolent systemic mastocytosis (12/45, 27%; P<0.001), only a few if any mast cells stained positive for CD30. These results could be confirmed by TissueFAXS analysis in subsets of patients with indolent systemic mastocytosis (n=7) and advanced systemic mastocytosis (n=4; P=0.008). The mast cell leukemia cell line HMC-1, derived from a patient with aggressive systemic mastocytosis also expressed the CD30 protein. In addition, we were able to detect CD30 mRNA in HMC-1 cells as well as in bone marrow biopsy samples in patients with systemic mastocytosis. In contrast, CD30 transcripts could not be detected in bone marrow biopsies in cases of reactive mast cell hyperplasia and in various other myeloid neoplasms. In conclusion, CD30 is preferentially expressed in neoplastic mast cells in advanced mast cell neoplasms. Upregulated expression of CD30 in advanced systemic mastocytosis may thus be employed as a potential marker for grading systemic mastocytosis in hematopathology.
authors.affiliation: Institute of Pathology, University of Munich, Munich, Germany. Karl.Sotlar@med.uni-muenchen.de
authors.forename: Karl
authors.initials: K
authors.lastname: Sotlar
authors.forename: Sabine
authors.initials: S
authors.lastname: Cerny-Reiterer
authors.forename: Karina
authors.initials: K
authors.lastname: Petat-Dutter
authors.forename: Harald
authors.initials: H
authors.lastname: Hessel
authors.forename: Sabina
authors.initials: S
authors.lastname: Berezowska
authors.forename: Leonhard
authors.initials: L
authors.lastname: Müllauer
authors.forename: Peter
authors.initials: P
authors.lastname: Valent
authors.forename: Hans-Peter
authors.initials: HP
authors.lastname: Horny
mesh_terms: D000328:Adult; D000368:Aged; D014408:Biomarkers, Tumor / Q000032:analysis* / Q000235:genetics; D001706:Biopsy; D001853:Bone Marrow / Q000276:immunology / Q000473:pathology; D001856:Bone Marrow Examination; D004252:DNA Mutational Analysis; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D017730:Ki-1 Antigen / Q000032:analysis* / Q000235:genetics; D008297:Male; D008407:Mast Cells / Q000276:immunology* / Q000473:pathology; D034721:Mastocytosis, Systemic / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D008875:Middle Aged; D009154:Mutation; D011379:Prognosis; D012333:RNA, Messenger / Q000032:analysis; D020133:Reverse Transcriptase Polymerase Chain Reaction; D015854:Up-Regulation; D055815:Young Adult
chemical_list: D014408:Biomarkers, Tumor; D017730:Ki-1 Antigen; D012333:RNA, Messenger