Systemic mastocytosis with plasma cell dyscrasia: report of a case.
Systemic mastocytosis (SM) comprises a heterogeneous group of disorders characterized by infiltration of bone marrow and other tissues by neoplastic mast cells. A subset of patients with SM has associated hematologic malignancy usually of myeloid origin and comprises an entity termed systemic mastocytosis with associated clonal hematological non-mast cell lineage disease (SM-AHNMD) by the current WHO classification. Reports of clonal lymphoid malignancies associated with SM are rare. We describe a patient who was simultaneously diagnosed with indolent SM and a plasma cell dyscrasia fitting the definition of monoclonal gammopathy of undetermined significance (MGUS). We also discuss the pathologic interaction between the neoplastic mast cells of SM and the lymphoid/plasma cell malignancy when these two entities coexist.
Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, 10833 Le Conte Avenue, Los Angeles, CA 90095, United States. spullarkat@mednet.ucla.edu
Sheeja T
ST
Pullarkat
Franklin
F
Sedarat
Ronald
R
Paquette
Jonathan
J
Said
D000368:Aged; D000369:Aged, 80 and over; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D008415:Mastocytosis / Q000473:pathology*; D010265:Paraproteinemias / Q000473:pathology*