持续性多克隆B细胞淋巴细胞增多症中出现症状性巨脾肿大且需行脾切除术
Symptomatic Massive Splenomegaly in Persistent Polyclonal B-cell Lymphocytosis Requiring Splenectomy.
作者信息
Bhagwandin Shanel B, Weisenberg Elliot S, Ozer Howard, Maker Ajay V
机构信息
Department of Surgery, University of Illinois at Chicago Medical Center, Chicago, Illinois.
Department of Pathology, University of Illinois at Chicago Medical Center, Chicago, Illinois.
出版信息
Open J Clin Med Case Rep. 2015;1(3). Epub 2015 Jun 29.
INTRODUCTION
Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare lymphoproliferative hematological disease characterized by binucleated lymphocytes, CD 19 CD 5lymphocytosis, and elevated levels of serum immunoglobulin M (IgM). It can rarely be associated with splenomegaly, though the disease usually remains indolent.
CASE PRESENTATION
We present a case of PPBL in a young man with massive splenomegaly that mimicked isolated splenic lymphoma requiring splenectomy for persistent pain, symptoms, and diagnosis.
DISCUSSION
Determining the etiology of splenomegaly in these patients is often confounding due to a lack of a tissue diagnosis and the limited morphological and immuno-histochemical features of PPBL, therefore, the presentation remains highly concerning for lymphoma.
CONCLUSION
The presentation, surgical treatment, tissue and peripheral blood molecular analysis, and flow cytometry integral to managing these patients and to prevent an assumptive and misleading diagnosis are reviewed.
引言
持续性多克隆B细胞淋巴细胞增多症（PPBL）是一种罕见的淋巴增生性血液疾病，其特征为双核淋巴细胞、CD19+CD5+淋巴细胞增多以及血清免疫球蛋白M（IgM）水平升高。虽然该疾病通常呈惰性，但很少会伴有脾肿大。
病例报告
我们报告了一例年轻男性的PPBL病例，该患者有巨大脾肿大，类似于孤立性脾淋巴瘤，因持续疼痛、症状及诊断需要而行脾切除术。
讨论
由于缺乏组织诊断以及PPBL有限的形态学和免疫组织化学特征，确定这些患者脾肿大的病因常常令人困惑，因此，该表现仍高度怀疑为淋巴瘤。
结论
本文回顾了对这些患者进行管理以及预防假定和误导性诊断所必需的临床表现、手术治疗、组织和外周血分子分析以及流式细胞术。
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