title: Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly.
abstract: Five cases of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly are reported; three were splenectomized. BCL2/IGH rearrangements were found in three cases; HLA-DRB1*07 in all. Bone marrow (BM) trephines showed a moderate lymphoid infiltrate with intrasinusoidal distribution resembling a splenic marginal-zone lymphoma. Splenic white pulp revealed an enlargement of the marginal-zone area; red pulp was infiltrated by the same lymphocytes engulfing the sinuses. Splenic and BM B-lymphocytes were CD79a(+)/CD20(+)/IgM(+)/IgD(+)/bcl-2(+)/CD27(+)/DBA.44(-)/CD31(-) and polyclonal by immunophenotype/polymerase chain reaction. PPBL features an expansion of splenic marginal-zone B-lymphocytes, which infiltrate BM sinusoids and circulate in the blood with no evidence of clonality, even in cases with progressive splenomegaly.
authors.affiliation: Department of Cellular Biotechnologies and Haematology, Division of Haematology, Sapienza University, Rome, Italy.
authors.forename: Ilaria
authors.initials: I
authors.lastname: Del Giudice
authors.forename: Stefano A
authors.initials: SA
authors.lastname: Pileri
authors.forename: Maura
authors.initials: M
authors.lastname: Rossi
authors.forename: Elena
authors.initials: E
authors.lastname: Sabattini
authors.forename: Cristina
authors.initials: C
authors.lastname: Campidelli
authors.forename: Irene Della
authors.initials: ID
authors.lastname: Starza
authors.forename: Maria S
authors.initials: MS
authors.lastname: De Propris
authors.forename: Francesca
authors.initials: F
authors.lastname: Mancini
authors.forename: Maria P
authors.initials: MP
authors.lastname: Perrone
authors.forename: Paola
authors.initials: P
authors.lastname: Gesuiti
authors.forename: Daniele
authors.initials: D
authors.lastname: Armiento
authors.forename: Luisa
authors.initials: L
authors.lastname: Quattrocchi
authors.forename: Agostino
authors.initials: A
authors.lastname: Tafuri
authors.forename: Angela
authors.initials: A
authors.lastname: Amendola
authors.forename: Francesca R
authors.initials: FR
authors.lastname: Mauro
authors.forename: Anna
authors.initials: A
authors.lastname: Guarini
authors.forename: Robin
authors.initials: R
authors.lastname: Foà
mesh_terms: D000293:Adolescent; D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology*; D001853:Bone Marrow / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D015321:Gene Rearrangement; D006684:HLA-DR Antigens / Q000032:analysis; D059811:HLA-DRB1 Chains; D006801:Humans; D007143:Immunoglobulin Heavy Chains / Q000235:genetics / Q000276:immunology; D016130:Immunophenotyping; D018655:Lymphocyte Count; D008218:Lymphocytosis / Q000276:immunology* / Q000601:surgery; D008297:Male; D016133:Polymerase Chain Reaction; D019253:Proto-Oncogene Proteins c-bcl-2 / Q000235:genetics / Q000276:immunology; D012907:Smoking / Q000276:immunology*; D013154:Spleen / Q000276:immunology*; D013156:Splenectomy; D013163:Splenomegaly / Q000276:immunology*; D055815:Young Adult
chemical_list: D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains; C456920:HLA-DRB1*07 antigen; D007143:Immunoglobulin Heavy Chains; D019253:Proto-Oncogene Proteins c-bcl-2