title: Polyclonal B-cell lymphocytosis and hypergammaglobulinemia in patients with Gaucher disease.
abstract: Sera from 23 individuals with Gaucher disease (GD) were analyzed for hypergammaglobulinemia and oligoclonal and monoclonal gammopathies. Serum IgG level was elevated in 15/23 (65%) patients, and a diffuse hypergammaglobulinemia was present in 10/23 (43%) patients. An oligoclonal gammopathy was noted in six patients, and a monoclonal gammopathy in two. Lymphocyte subset analysis was also carried out in eight individuals with GD. Four of five individuals showed increased surface Ig-positive lymphocytes, while 7/7 were positive for either increased CD19- and/or CD20-positive lymphocytes. An eighth patient was found to have a B-cell leukemia. Statistical analysis of kappa and lambda histograms were suggestive of a monoclonal excess. However, restriction enzyme analysis of four individuals with GD and increased B cells failed to show any evidence of Ig gene rearrangements. Serum Ig abnormalities and perhaps B-cell lymphocytosis appear to be common in the GD patient population and are not associated with circulating monoclonal lymphocytes.
authors.affiliation: Hematology Service, NINCDS, Bethesda, MD 20892.
authors.forename: G E
authors.initials: GE
authors.lastname: Marti
authors.forename: E T
authors.initials: ET
authors.lastname: Ryan
authors.forename: N M
authors.initials: NM
authors.lastname: Papadopoulos
authors.forename: M
authors.initials: M
authors.lastname: Filling-Katz
authors.forename: N
authors.initials: N
authors.lastname: Barton
authors.forename: T A
authors.initials: TA
authors.lastname: Fleischer
authors.forename: M
authors.initials: M
authors.lastname: Rick
authors.forename: H R
authors.initials: HR
authors.lastname: Gralnick
mesh_terms: D000328:Adult; D001402:B-Lymphocytes / Q000473:pathology*; D015139:Blotting, Southern; D005776:Gaucher Disease / Q000150:complications*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000209:etiology*; D007074:Immunoglobulin G; D007136:Immunoglobulins / Q000032:analysis; D008214:Lymphocytes / Q000145:classification; D008218:Lymphocytosis / Q000209:etiology* / Q000473:pathology; D008875:Middle Aged
chemical_list: D007074:Immunoglobulin G; D007136:Immunoglobulins