Division of Hematopathology, Diagnostic Services of Manitoba, University of Manitoba, MS559S-820 Sherbrook Street, Winnipeg R3A 1R9MB, Canada.
Diagn Pathol. 2012 Aug 19;7:107. doi: 10.1186/1746-1596-7-107.
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.
The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/5329558967545656.
持续性多克隆 B 细胞淋巴增生症（PPBL）是一种罕见且有趣的血液系统疾病，主要发生在年轻到中年吸烟女性中。其特征为持续性中度多克隆 B 细胞淋巴增生，伴有循环特征性双核淋巴细胞和升高的多克隆血清 IgM。大多数患者在长期随访中有良性的临床过程。PPBL 的一些病理特征可能类似于恶性淋巴瘤，包括形态以及频繁的细胞遗传学和分子异常。由于该疾病显著的症状性脾肿大需要进行脾切除术是非常罕见的；因此，文献中缺乏对脾脏形态特征的描述。我们在此介绍了首例持续性多克隆 B 细胞淋巴增生症患者的脾脏形态学和免疫组织化学特征，该患者在 6 年的随访中出现了巨大的脾肿大。为了缓解症状和怀疑恶性过程而行脾切除术。脾脏的形态学和免疫组织化学特征非常类似于 B 细胞淋巴瘤的累及，但流式细胞术未发现单克隆表面轻链限制，分子分析也未检测到 IgH 基因的克隆重排。在这种情况下评估脾切除术标本可能对病理学家构成诊断挑战。因此，与 B 细胞克隆性研究（流式细胞术和分子分析）、临床发现和外周血形态学寻找特征性双核淋巴细胞进行相关性分析对于避免将这一良性过程误诊为 B 细胞淋巴瘤至关重要。我们还在此介绍了对持续性多克隆 B 细胞淋巴增生症发病机制的文献综述。
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