Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly.
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.
Del Giudice
Ilaria
I
Department of Cellular Biotechnologies and Haematology, Division of Haematology, Sapienza University, Rome, Italy.
Pileri
Stefano A
SA
Rossi
Maura
M
Sabattini
Elena
E
Campidelli
Cristina
C
Starza
Irene Della
ID
De Propris
Maria S
MS
Mancini
Francesca
F
Perrone
Maria P
MP
Gesuiti
Paola
P
Armiento
Daniele
D
Quattrocchi
Luisa
L
Tafuri
Agostino
A
Amendola
Angela
A
Mauro
Francesca R
FR
Guarini
Anna
A
Foà
Robin
R
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
D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains; C456920:HLA-DRB1*07 antigen; D007143:Immunoglobulin Heavy Chains; D019253:Proto-Oncogene Proteins c-bcl-2