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伴有抗红细胞生成因子的腹腔内血管滤泡性淋巴结增生(浆细胞变异型)

Intra-abdominal, angiofollicular lymph node hyperplasia (plasma-cell variant) with an antierythropoietic factor.

作者信息

Burgert E O, Gilchrist G S, Fairbanks V F, Lynn H B, Dukes P P, Harrison E G

出版信息

Mayo Clin Proc. 1975 Sep;50(9):542-6.

PMID:51137
Abstract

An 11-year-old girl presented with a refractory hypochromic microcytic anemia, hypoferremia, normoblastic hyperplastic bone marrow, hypergammaglobulinemia, and growth retardation. Many varied treatments failed to produce any improvement. Ferrokinetic studies revealed rapid plasma clearance and increased plasma iron turnover, but impaired incorporation of 59Fe. Excretion of 57Co after an oral dose indicated an increased iron absorption. A (99M)Tc-sulfur colloid scintigram of the abdomen failed to demonstrate abnormal uptake. A nodal mass showing the plasma-cell variant of angiofollicular hyperplasia was removed from the gastrolienal ligament. Follow-up studies at 3 and 6 months revealed complete correction of the anemia, a 4.8-cm increase in height, and normal serum gamma-globulin levels. Serum obtained before operation inhibited the incorporation of 59Fe that was induced by a standard dose of erthyropoietin in the exhypoxic mouse system, and this inhibition persisted in serum obtained 3 days after surgery but disappeared by 6 days. The data suggest that the hyperplastic angiofollicular lymph node (plasma-cell variant) secreted a substance the inhibited erythropoiesis.

摘要

一名11岁女孩出现难治性低色素小细胞性贫血、低铁血症、正成红细胞性骨髓增生、高球蛋白血症和生长发育迟缓。多种治疗均未能带来任何改善。铁动力学研究显示血浆清除迅速且血浆铁周转率增加,但59Fe的掺入受损。口服一剂57Co后的排泄表明铁吸收增加。腹部的(99M)Tc-硫胶体闪烁图未显示异常摄取。从胃脾韧带切除了一个显示血管滤泡性增生浆细胞变体的淋巴结肿块。3个月和6个月的随访研究显示贫血完全纠正,身高增加4.8厘米,血清γ-球蛋白水平正常。术前获得的血清抑制了低氧小鼠系统中标准剂量促红细胞生成素诱导的59Fe掺入,这种抑制在术后3天获得的血清中持续存在,但在6天时消失。数据表明增生性血管滤泡性淋巴结(浆细胞变体)分泌了一种抑制红细胞生成的物质。

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