Aggressive Systemic Mastocytosis Associated with Mesangioproliferative Glomerulonephritis
Aggressive Systemic Mastocytosis Associated with Mesangioproliferative Glomerulonephritis
复制标题
DOI：
10.1159/000322286
复制
发表时间：
2010-12
影响因子：
2.4
通讯作者：
M. Diamantidis;A. Myrou;G. Kaiafa;V. Kaloutsi;G. Karayannopoulou;A. Theodoridis;A. Adamidou;A. Papadopoulos;D. Grekas
中科院分区：
文献类型：
--
作者：
M. Diamantidis;A. Myrou;G. Kaiafa;V. Kaloutsi;G. Karayannopoulou;A. Theodoridis;A. Adamidou;A. Papadopoulos;D. Grekas
背景/目的/方法：侵袭性系统性肥大细胞增多症（ASM）是系统性肥大细胞增多症的一种亚型，其包括一组异质性疾病，其特征在于肿瘤性肥大细胞浸润骨髓、皮肤、肝、脾、淋巴结和胃肠道。由于肾脏不是ASM影响的已知器官，因此缺乏ASM与肾脏受累相关性的数据。结果/结论：据我们所知，这是第一例ASM与系膜增生性肾小球肾炎和单克隆丙种球蛋白病的意义不明，没有肾病综合征的存在。病人的临床过程和有趣的家族史，沿着治疗选择的描述。最后，提出了可能的病理生理机制解释肾脏参与我们的病人进行了讨论。
Background/Aims/Methods: Aggressive systemic mastocytosis (ASM) is a subtype of systemic mastocytosis, which comprises a heterogenous group of disorders characterized by infiltration of bone marrow, skin, liver, spleen, lymph nodes and gastrointestinal tract by neoplastic mast cells. There is lack of data on the association of ASM with renal involvement, as kidney is not among the known organs affected by ASM. Results/Conclusions: To the best of our knowledge, this is the first case of ASM associated with mesangioproliferative glomerulonephritis and monoclonal gammopathy of undetermined significance, without the presence of nephrotic syndrome. The patient’s clinical course and the intriguing family history, along with the treatment selection are described. Finally, the proposed possible pathophysiological mechanisms explaining the renal involvement of our patient are discussed.