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[癌症所致骨髓纤维化:一例诊断极为困难的临床病例报告]

[Myelofibrosis caused by cancer: presentation of a clinical case with a very difficult diagnosis].

作者信息

Fortunato A, Mazzone A, Ricevuti G

出版信息

Minerva Med. 1985 Aug 25;76(32-33):1501-6.

PMID:4034049
Abstract

Fibrosis of the bone marrow or myelofibrosis, is a connective tissue response of the bone marrow, giving rise to alterations of various nature. From an etiological point of view, it may be divided into primitive or secondary forms. Since the therapeutic implications differ, it is important to make a correct differential diagnosis between the 2 forms. With this in mind, particular importance must be given to myelofibrosis secondary to neoplastic metastasis since it frequently presents with a clinical and hematological picture and bone marrow morphology typical of acute or chronic idiopathic myelofibrosis. In such a situation the particular course of the disease and the description fibrosis-sclerosis of the bone marrow are highly suggestive of myelofibrosis secondary to neoplastic metastasis. On the basis of these considerations, we found it interesting to report a case which came under our observations. The patient in this case presented a complex differential diagnosis which offered us the occasion to review and highlight the clinical and histological criteria fundamental to the 2 forms. The patient, a 40 year old woman, presented with a clinical picture typical of myelofibrosis following surgery for a cystic ovary. For 3 years she had felt violent bone pains and had progressively declined in her physical state to the point of cachexia. A careful histological exam of the bone marrow revealed metastasis from the gastric tumor thus giving a clear diagnosis of secondary myelofibrosis.

摘要

骨髓纤维化,即髓样化生,是骨髓的一种结缔组织反应,会引发各种性质的改变。从病因学角度来看,它可分为原发性或继发性形式。由于治疗意义不同,正确区分这两种形式的鉴别诊断很重要。考虑到这一点,对于继发于肿瘤转移的骨髓纤维化必须给予特别重视,因为它常常呈现出急性或慢性特发性骨髓纤维化典型的临床、血液学表现及骨髓形态。在这种情况下,疾病的特殊病程以及骨髓纤维化 - 硬化的描述高度提示继发于肿瘤转移的骨髓纤维化。基于这些考虑,我们认为报告我们所观察到的一个病例很有意思。该病例中的患者面临复杂的鉴别诊断,这为我们提供了回顾和强调这两种形式的临床及组织学基本标准的机会。患者为一名40岁女性,在接受卵巢囊肿手术后出现了典型的骨髓纤维化临床表现。三年来,她一直感到剧烈的骨痛,身体状况逐渐恶化至恶病质状态。对骨髓进行仔细的组织学检查发现了胃肿瘤的转移,从而明确诊断为继发性骨髓纤维化。

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