Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients
Bone marrow histopathology in POEMS syndrome: a distinctive combination of plasma cell, lymphoid, and myeloid findings in 87 patients
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DOI：
10.1182/blood-2010-11-316935
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发表时间：
2011-06-16
期刊：
影响因子：
20.3
通讯作者：
Hoyer, James D.
中科院分区：
文献类型：
--
作者：
Dao, Linda N.;Hanson, Curtis A.;Hoyer, James D.
POEMS是一种罕见的综合征性疾病，其特征是多发性神经病、器官肿大、内分泌病、单克隆蛋白和皮肤改变。POEMS的骨髓病理学描述很少，因此，对87例POEMS患者（共143例，治疗前67例，治疗后76例）的外周血涂片、骨髓穿刺和活检进行了研究。通过流式细胞术、免疫组织化学和/或原位杂交分析浆细胞克隆性。在44例治疗前病例（66%）中检测到单型浆细胞;大多数浆细胞表达λ轻链（91%）。单型浆细胞通常存在于多型浆细胞增加的背景中。在33例（49%）治疗前病例中发现了类圆形聚集体，大多数病例（97%）被浆细胞包围。巨核细胞增生（36例）和簇（62例）是常见的;然而，43例受试者中没有一例发生JAK 2（V617 F）突变。总之，我们已经确定了一个新的星座的特点，应强烈建议POEMS综合征的鉴别诊断的一部分。在骨髓中，尤其是在周围神经病变的背景下，一系列的限制性单克隆丙种球蛋白病，浆细胞周围的淋巴聚集，巨核细胞增生是高度提示这种诊断。（血。2011;117（24）：6438-6444）
POEMS is an uncommon syndromic disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes. There are few descriptions of the bone marrow pathology of POEMS; therefore, peripheral blood smears and bone marrow aspirates and biopsies from 87 patients (143 total, 67 pretreatment, 76 posttreatment cases) with POEMS were studied. Plasma cell clonality was analyzed by flow cytometry, immunohistochemistry, and/or in situ hybridization. Monotypic plasma cells were detected in 44 pretreatment cases (66%); the majority of plasma cells expressed lambda light chain (91%). The monotypic plasma cells typically were present in a background of increased polytypic plasma cells. Lymphoid aggregates were found in 33 (49%) pretreatment cases and in most cases were rimmed by plasma cells (97%). Megakaryocyte hyperplasia (36 cases) and clusters (62 cases) were frequent; however, none of the 43 cases tested had the JAK2(V617F) mutation. In summary, we have identified a novel constellation of features that should strongly suggest POEMS syndrome as part of the differential diagnosis. The constellation of lambda-restricted monoclonal gammopathy, plasma cell rimming around lymphoid aggregates, and megakaryocytic hyperplasia in a bone marrow is highly suggestive of this diagnosis, especially in the context of a peripheral neuropathy. (Blood. 2011;117(24):6438-6444)