Abstract
Persistent polyclonal B-cell lymphocytosis (PPBL) is rare and intriguing hematological disorder predominantly reported in young to middle- aged smoking women. It is characterized by persistent moderate polyclonal B-cell lymphocytosis with circulating hallmark binucleated lymphocytes and elevated polyclonal serum IgM. Most patients have benign clinical course on long-term follow-up. Some pathologic features of PPBL may resemble malignant lymphoma, including morphology as well as frequent cytogenetic and molecular abnormalities. Significant symptomatic splenomegaly requiring splenectomy is very unusual for this disorder; therefore there is a lack of descriptions of the morphologic features of the spleen in the literature. We present here one of the first detailed descriptions of the morphologic and immunohistochemical features of the spleen from a young female with PPBL who developed massive splenomegaly during 6-year follow up. Splenectomy was performed for symptomatic relief and suspicion of malignant process. The morphological and immunohistochemical features of the spleen closely mimicked involvement by B-cell lymphoma, however there was no monotypic surface light chain restriction seen by flow cytometry and no clonal rearrangement of IgH gene was detected by molecular analysis. Evaluating a splenectomy sample in cases like this may present a diagnostic challenge to pathologists. Therefore, correlation with B cell clonality studies (by flow cytometry and molecular analysis), clinical findings and peripheral blood morphology searching for characteristic binucleated lymphocytes is essential to avoid misdiagnosing this benign process as B-cell lymphoma. We also present here a literature review on pathogenesis of PPBL.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/5329558967545656
Highlights
Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare clinically benign lymphoproliferative disorder first described by Gordon et al in 1982 [1]
PPBL is characterized by chronic mild-to-moderate peripheral polyclonal lymphocytosis of B cell origin as evidenced by flow cytometry and variable number of
Immunophenotyping by flow cytometry Mononuclear cells from fresh peripheral blood were separated by density-gradient centrifugation and were characterized using four-color immunostaining by a FACSCalibur flow cytometer (Becton, Dickinson and Company, San Jose, CA) and Cell Quest software (Becton, Dickinson and Company, San Jose, CA)
Summary
Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare clinically benign lymphoproliferative disorder first described by Gordon et al in 1982 [1]. PPBL is characterized by chronic mild-to-moderate peripheral polyclonal lymphocytosis of B cell origin as evidenced by flow cytometry and variable number of the literature. As most patients have an indolent clinical course and peripheral lymphocytosis is sometimes absent, this condition is likely under-recognized. PPBL resembles malignant lymphoproliferative disorder in many aspects, both morphologically and at the molecular/cytogenetic levels. We here report histological and immunohistochemical features of the spleen in a young female with PPBL who underwent splenectomy due to progressive splenomegaly on her 6th annual follow-up. Italian investigators Del Giudice et al described similar histopathological findings of the spleen in three patients with PPBL and progressive splenomegaly who underwent splenectomy [8]. To avoid unnecessary aggressive treatment, it is important for pathologists to distinguish this clinically benign disorder from malignant lymphoproliferative disorders
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