1749
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Systemic mastocytosis associated with lamda-clonal plasmacytoma with amyloidosis (Congo red)
Bone Marrow
Congo red
62 years
female
AL-Amyloidosis with IgA Lambda.
Slightly hypercellular bone marrow with an infiltration by plasma cells (around 10%) and mastocytes (5-7%). Vascular amyloid deposits in a periosteal fragment (top of the slide).
Amyloid depositis are immunohistochemically positive for lambda light chains
The criteria of a systemic mastocytosis are fulfilled (major and minor criteria). Because of the concurrent plasma cell neoplasia the diagnosis is systemic mastocytosis with associated clonal hematological non-mast cell lineage disease (SMAHNMD). A mutationanalysis was performed and, as expected, a mutation of the C-KIT Gene (p.D816V in Exon 17) was detected.
Manuel, Schlageter, University of Basel
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