Taki T, Wakabayashi T, Kishimoto H
Acta Pathol Jpn. 1980 Jul;30(4):565-78. doi: 10.1111/j.1440-1827.1980.tb01352.x.
An autopsy case of erythremia with sideroblastic tumor cell proliferation is described. A 60-year-old man was admitted to the hospital due to general fatigue and anorexia. Bone marrow aspiration revealed abnormalities in erythropoiesis (megaloblasts, 4%; sideroblasts, 84%; ring-formed, 39%, and PAS-positive, 5%). Therapy was directed to pulmonary tuberculosis. Anemia was not improved despite repeated whole blood and platelet transfusions. Serum iron and percentage saturation of the total iron-binding capacity rose during the course. Administration of vitamin B12, B6 or folic acid was inefffective. INAH was replaced by its derivative, IHMS, during the course, but the population of sideroblasts especially of ring-sideroblasts was invariably large (78%-100% and 39%-65% for total sideroblasts and ring-sideroblasts, respectively). He died with increasing abdominal pain and jaundice after three months' hospitalization. Main autopsy findings were: diffuse proliferation of atypical erythroblasts in the bone marrow, systemic lymph nodes, liver, spleen and kidneys. Most of the cells positively stained with iron. Tuberculosis of lungs with cavity formation. Discussion is focussed on the relationship between erythremia and sideroblastic anemia.
报告一例伴有铁粒幼细胞肿瘤细胞增殖的真性红细胞增多症尸检病例。一名60岁男性因全身乏力和厌食入院。骨髓穿刺显示红细胞生成异常（巨幼细胞，4%；铁粒幼细胞，84%；环形，39%，PAS阳性，5%）。针对肺结核进行治疗。尽管反复输注全血和血小板，贫血仍未改善。病程中血清铁和总铁结合力百分比饱和度升高。给予维生素B12、B6或叶酸无效。病程中INAH被其衍生物IHMS替代，但铁粒幼细胞尤其是环形铁粒幼细胞的比例始终很高（总铁粒幼细胞和环形铁粒幼细胞分别为78%-100%和39%-65%）。住院三个月后，他因腹痛和黄疸加重而死亡。主要尸检发现为：骨髓、全身淋巴结、肝脏、脾脏和肾脏中出现非典型成红细胞弥漫性增殖。大多数细胞铁染色阳性。肺部有空洞形成的肺结核。讨论集中在真性红细胞增多症与铁粒幼细胞贫血之间的关系。
