甘露糖苷贮积症中的全血细胞减少症。
Pancytopenia in mannosidosis.
作者信息
Press O W, Fingert H, Lott I T, Dickersin C R
出版信息
Arch Intern Med. 1983 Jun;143(6):1266-8.
PMID:6860058
Abstract
A case of autoimmune pancytopenia is described in a patient with mannosidosis who developed anti-platelet and anti-neutrophil antibodies and a low haptoglobin level. Bone marrow biopsy and 111InCl3 bone marrow scanning demonstrated hypoplasia of the marrow with a paucity of "storage cells." Pathogenic mechanisms and implications for other inborn errors of metabolism are discussed.
摘要
本文描述了一例患有甘露糖苷贮积症的患者发生自身免疫性全血细胞减少症，该患者产生了抗血小板和抗中性粒细胞抗体，且触珠蛋白水平较低。骨髓活检和铟-111 骨髓扫描显示骨髓发育不全，“贮积细胞”数量稀少。文中讨论了致病机制以及对其他先天性代谢缺陷的影响。
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