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进行性骨化性纤维发育不良极早期病变中的急性淋巴细胞浸润。

Acute lymphocytic infiltration in an extremely early lesion of fibrodysplasia ossificans progressiva.

作者信息

Gannon F H, Valentine B A, Shore E M, Zasloff M A, Kaplan F S

机构信息

Division of Metabolic Bone Diseases and Molecular Orthopaedics, University of Pennsylvania School of Medicine, Philadelphia 19104, USA.

出版信息

Clin Orthop Relat Res. 1998 Jan(346):19-25.

PMID:9577406
Abstract

A 2-year-old child with fibrodysplasia ossificans progressiva underwent a muscle biopsy of a very early lesion, and had findings that showed the earliest stage ever seen in the histopathology of fibrodysplasia ossificans progressiva. This very early stage consisted of intense perivascular lymphocytic infiltration into normal appearing skeletal muscle. A nearly identical histopathologic sequence was noted in a cat with phenotypic features similar to those of fibrodysplasia ossificans progressiva in humans. These new findings represent the earliest documented changes that have ever been noted in fibrodysplasia ossificans progressiva, and provide further histopathologic support for the recent discovery that lymphocytes may play a role in the pathogenesis of heterotopic ossification in fibrodysplasia ossificans progressiva.

摘要

一名患有进行性骨化性纤维发育不良的2岁儿童接受了一个非常早期病变的肌肉活检,其结果显示了进行性骨化性纤维发育不良组织病理学中所见过的最早阶段。这个非常早期阶段包括血管周围密集的淋巴细胞浸润到看似正常的骨骼肌中。在一只具有与人类进行性骨化性纤维发育不良相似表型特征的猫身上,也观察到了几乎相同的组织病理学序列。这些新发现代表了进行性骨化性纤维发育不良中记录到的最早变化,并为最近发现淋巴细胞可能在进行性骨化性纤维发育不良的异位骨化发病机制中起作用提供了进一步的组织病理学支持。

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