Histopathological and molecular features of persistent polyclonal B‐cell lymphocytosis (PPBL) with progressive splenomegaly

Giudice, Ilaria Del; Pileri, Stefano; Rossi, Mosè; Sabattini, Elena; Campidelli, Cristina; Starza, Irene Della; Propris, M. S. De; Mancini, Francesca; Perrone, Maria Paola; Gesuiti, Paola; Armiento, Daniele; Quattrocchi, Luisa; Tafuri, Agostino; Amendola, Angela; Mauro, Francesca Romana; Guarini, Anna; Foà, Robin

doi:10.1111/j.1365-2141.2008.07551.x

Cited by 37 publications

(20 citation statements)

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“…In their series of 5 patients Del Giudice et al from Italy recently reported very similar findings to ours in three of their five PPBL patients who developed massive splenomegaly and underwent splenectomy [8]. The B cells present both in bone marrow and spleen show same immunophenotype including expression of BCL-2 [7,8]. In addition, the B cells in our patient were also positive for CD43.…”

Section: Discussionsupporting

confidence: 85%

“…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 6 th annual follow-up. Very recently, 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]. PPBL can be further confused with a malignant lymphoproliferative disorder as PPBL is frequently associated with chromosomal abnormalities and multiple BCL2/IG gene rearrangements as seen in some lymphomas [9,10].…”

Section: Introductionmentioning

confidence: 99%

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Histological and immunohistochemical features of the spleen in persistent polyclonal B-cell lymphocytosis closely mimic splenic B-cell lymphoma

Sun

Juskevicius

2012

Diagn Pathol

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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

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Section: Discussionsupporting

confidence: 85%

Section: Introductionmentioning

confidence: 99%

Histological and immunohistochemical features of the spleen in persistent polyclonal B-cell lymphocytosis closely mimic splenic B-cell lymphoma

Sun

Juskevicius

2012

Diagn Pathol

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“…Nevertheless, some features could suggest a link between PPBL and SMZL. Both entities share same histologic patterns: 1/expansion of the white pulp of the spleen, with an enlargement of the marginal zone area of the follicles, and an intrasinusoidal infiltration of the red pulp [7,8] and 2/ intrasinusoidal infiltration by polyclonal B-cells in bone marrow [7,9]. Phenotypical characterization by MFC is also similar between the two entities, with the expression of surface IgM, CD19, CD20 and CD79b, but lacking the expression of CD5, CD10, CD23, CD43 and CD103 (WHO classification, 2008).…”

Section: Discussionmentioning

confidence: 99%

Occurrence of Non-Hodgkin Lymphoma after the Diagnosis of Persistent Polyclonal B-cell Lymphocytosis

Mear¹

2015

J Leuk

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on a routine blood count in 1988. A mild thrombopenia, constantly above 100×10 9 /L was iteratively reported on blood count since 1996, and binucleated lymphocytes were described on blood smears since 1997. The patient reported no clinical symptomatology. Clinical examination at diagnosis revealed a splenomegaly. There was no hepatomegaly or enlarged lymph nodes. On blood count, hemoglobin was 131 g/L, with 123×10 Though polyclonal, recurrent genetic abnormalities have been described in PPBL, supernumerary isochromosome 3q (+i(3)(q10)) being the most frequently described [3]. We report the cases of two patients with PPBL, where B-NHL occurred after PPBL diagnosis, splenic zone marginal lymphoma (SMZL) and diffuse large B-cell 41 lymphoma (DLBCL), leading us to raise the question of a relationshipbetween PPBL and B-42 NHL. Material and Methods Multiparameter flow cytometry (MFC)Lymphocytes were isolated from blood samples by gradient density. For splenic samples, cell suspension was obtained after mechanical dissociation of tissue. Lymphocytes were immunophenotyped by using 4-color direct immunofluorescence on a BD FACS CANTO II (Becton Dickinson, Franklin Lake, NJ, USA). PCR amplification, cloning, and sequencing analysisDNA was extracted from samples using routine procedures. PCR analysis of B-cell clonality was performed following the recommendations of BIOMED 2 protocols [4]. CytogeneticsConventional cytogenetics and interphase Fluorescence In Situ Hybridization (FISH) were performed on peripheral blood and on spleen lysates as described elsewhere [5]. First case: Occurrence of pulmonary DLBCL 13 years after PPBL diagnosisThe first patient is a 42-year-old man, who smoked 30 cigarettes each day. The lymphocytosis (8.5×10 9 /L) was fortuitously discovered AbstractPersistent polyclonal B cell lymphocytosis (PPBL) is a rare indolent condition characterized by a polyclonal expansion of B cells associated with binucleated lymphocytes observable on blood smears. Though polyclonal, recurrent genetic abnormalities have been described in PPBL, supernumerary isochromosome 3q (+i(3)(q10)) being the most frequently described. We report here two clinical observations showing the occurrence of NHL after the diagnosis of PPBL. These observations lead us to recommend a closer follow-up of PPBL patients and raise the question of a relationship between PPBL and NHL. Journal of LeukemiaJ o u r n a l of Le u k e m ia

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“…Patterns are generally mixed in any individual specimen, apart from pure paratrabecular involvement in some cases of FL and pure nodular infiltration in some examples of SLL/CLL. Pure or almost exclusively intrasinusoidal small B-cell infiltration should raise the possibility of persistent polyclonal B lymphocytosis, a rare disorder found typically in female patients who are heavy smokers 17. The cellular immunophenotype in persistent polyclonal B lymphocytosis resembles that of SMZL apart from the absence of light chain restriction.…”

Section: Lymphoid Infiltratesmentioning

confidence: 99%

Pitfalls in bone marrow pathology: avoiding errors in bone marrow trephine biopsy diagnosis

Wilkins

2011

J Clin Pathol

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Avoiding errors in the histological interpretation of bone marrow trephine biopsy specimens requires an unprecedented degree of collaboration between histopathologists, haematologists, specimen requesters, specimen takers, laboratory technical staff and other scientific staff. A specimen of good quality, with full, relevant clinical information is the essential starting point. This must then be processed optimally and investigated appropriately, involving immunophenotyping and molecular testing when needed. A wide range of pathologies may involve bone marrow haemopoietic and stromal components, and a systematic approach to analysing each of the components in turn is required to avoid overlooking abnormalities; correlation with bone marrow cells aspirated in parallel is particularly important. Final interpretation should be a synthesis of the histological findings with information from such haematological and other investigations, interpreted with due regard to clinical context.

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Histopathological and molecular features of persistent polyclonal B‐cell lymphocytosis (PPBL) with progressive splenomegaly

Cited by 37 publications

References 12 publications

Histological and immunohistochemical features of the spleen in persistent polyclonal B-cell lymphocytosis closely mimic splenic B-cell lymphoma

Histological and immunohistochemical features of the spleen in persistent polyclonal B-cell lymphocytosis closely mimic splenic B-cell lymphoma

Occurrence of Non-Hodgkin Lymphoma after the Diagnosis of Persistent Polyclonal B-cell Lymphocytosis

Pitfalls in bone marrow pathology: avoiding errors in bone marrow trephine biopsy diagnosis

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