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.
Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly / DEL GIUDICE, Ilaria; Stefano A., Pileri; Maura, Rossi; Elena, Sabattini; Cristina, Campidelli; DELLA STARZA, Irene; Maria S., De Propris; Francesca, Mancini; Maria P., Perrone; Paola, Gesuiti; Daniele, Armiento; Luisa, Quattrocchi; Tafuri, Agostino; Angela, Amendola; Mauro, Francesca Romana; Guarini, Anna; Foa, Roberto. - In: BRITISH JOURNAL OF HAEMATOLOGY. - ISSN 0007-1048. - 144:5(2009), pp. 726-731. [10.1111/j.1365-2141.2008.07551.x]
Histopathological and molecular features of persistent polyclonal B-cell lymphocytosis (PPBL) with progressive splenomegaly
DEL GIUDICE, ILARIA;DELLA STARZA, IRENE;TAFURI, Agostino;MAURO, Francesca Romana;GUARINI, Anna;FOA, Roberto
2009
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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.