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骨髓纤维化时髓样化生常限于脾、肝和淋巴结。有时也发生在其他部位,如肺、肾和胃肠道。中枢神经系髓样化生的病例较罕见,而颅内髓样化生更属罕见。作者报道1例患真性红细胞增多症14年的62岁男性患者,骨髓纤维化阶段出现颅内压增高的症状,CT扫描以及右颈内动脉造影均显示脑膜内有丰富血管形成的肿块,患者最终死于心内膜炎和进行性心衰。尸检证实颅内肿块是中枢神经系统的髓样化生,其病情经过如下: 患者于1965年确诊患有真性红细胞增多症。当时红细胞比积为80％,肝脾肿大,曾用放血和~(32)P治疗。
Myeloid metaplasia is often limited to the spleen, liver and lymph nodes in myelofibrosis. Occasionally occurs in other areas, such as lung, kidney and gastrointestinal tract. Central nervous system myeloid metaplasia cases rare, and intracranial myeloid metaplasia is even rare. The authors report a 62-year-old male patient with polycythemia vera for 14 years presenting with symptoms of increased intracranial pressure at the stage of myelofibrosis. CT scan and right internal carotid artery angiography showed abundant intravascular mesenchymal masses in the meninges. The patient eventually Died of endocarditis and progressive heart failure. An autopsy confirmed that the intracranial mass was myeloid metaplasia of the central nervous system and its condition was as follows: The patient was diagnosed with polycythemia vera in 1965. At that time, hematocrit was 80%, hepatosplenomegaly, had bleeding and ~ (32) P treatment.