1748
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Systemic mastocytosis associated with lamda-clonal plasmacytoma with amyloidosis
Bone Marrow
Haematoxylin & Eosin (H&E)
62 years
female
AL-Amyloidosis with IgA Lambda.
Slightly hypercellular bone marrow with an infiltration by plasma cells (around 10%) and mast cells (5-7%). Lambda light chain restriction (kappa:lambda ratio 1:30). Furthermore compact aggregations of atypical spindly mast cells. Perivascular amyloid deposition (lambda light chain positive).
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 mutation analysis 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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