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[髓外造血。佩吉特病性脊髓压迫的一种罕见病因]

[Extramedullary hematopoiesis. An unusual cause of Pagetic spinal cord compression].

作者信息

Ducloux J M, Maugars Y, Moreau A, Nizard J, Gaillard F, Prost A

机构信息

Service de Rhumatologie, CHRU de Nantes.

出版信息

Rev Rhum Ed Fr. 1993 Jan;60(1):23-7.

PMID:8242022
Abstract

An original case is reported: spinal cord compression due to extramedullary hematopoiesis of the epidural space, related to Paget's disease of bone, successfully treated by surgery. Extramedullary hematopoiesis, usually occurring in cases of hematological disease, can also complicate Paget's disease, probably by bone effraction mechanism. A review of literature has found 5 other cases of extramedullary hematopoiesis occurring in Paget's disease, without hematological disease. Medullar scintigraphy (Indium111, Technetium99 Sulfur colloid) can confirm the diagnosis after X-ray findings, without the need for biopsy. Awareness knowledge of this complication can sometimes avoid invasive investigation. Low dose radiotherapy is useful in the case of hematological disorders. In Paget's disease, there is a risk of sarcomatous degeneration. In our patient, surgery cured cord compression, but it relapsed because of extensive fibrosis, in complicating surgical hemorrhage.

摘要

报告了一例原发性病例

硬膜外间隙髓外造血导致脊髓压迫,与骨佩吉特病相关,通过手术成功治疗。髓外造血通常发生在血液系统疾病病例中,也可能使佩吉特病复杂化,可能是通过骨侵蚀机制。文献回顾发现另外5例佩吉特病患者发生髓外造血,且无血液系统疾病。髓核闪烁扫描(铟111、锝99硫胶体)可在X线检查结果后确诊,无需活检。了解这种并发症有时可避免进行侵入性检查。低剂量放疗对血液系统疾病有用。在佩吉特病中,存在肉瘤样变性的风险。在我们的患者中,手术治愈了脊髓压迫,但由于广泛纤维化并伴有手术出血,病情复发。

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