Sato Y, Shibuya A, Kudo H, Adachi Y, Kinugasa K, Matsumoto M, Aoki M
Tokyo Metropolitan Bokuto Hospital.
Rinsho Ketsueki. 1990 Feb;31(2):204-8.
A 70-year-old man, who had been diagnosed as primary myelofibrosis in 1987 at Tokyo Women's Medical School, was admitted to our hospital because of left hip-joint pain in May 1988. Physical examinations revealed marked hepatosplenomegaly and multiple reddish papules on the skin. The peripheral blood showed marked leukoerythroblastosis and severe anemia with poikilocytosis. Bone marrow aspirations were dry tap. Needle biopsy of iliac crest showed a diffuse fibrosis. Biopsy of the papules showed an extramedullary haematopoiesis. He was treated with low dose Ara-C for seven days. Although hepatosplenomegaly and skin papules were reduced, he died of pneumonia a month later. At autopsy, leukemic cells massively infiltrated into the multiple organs, including bone marrow. The cells were identified with megakaryoblast, as those were positive for factor VIII related antigen. As far as we know, this is the third case of primary myelofibrosis transformed into acute megakaryoblastic leukemia.
一名70岁男性，1987年在东京女子医科大学被诊断为原发性骨髓纤维化，1988年5月因左髋关节疼痛入院。体格检查发现明显的肝脾肿大和皮肤上有多个红色丘疹。外周血显示明显的幼粒-幼红细胞增多和严重贫血伴异形红细胞症。骨髓穿刺呈干抽。髂嵴穿刺活检显示弥漫性纤维化。丘疹活检显示髓外造血。他接受了低剂量阿糖胞苷治疗7天。尽管肝脾肿大和皮肤丘疹有所减轻，但1个月后他死于肺炎。尸检时，白血病细胞大量浸润包括骨髓在内的多个器官。这些细胞被鉴定为巨核母细胞，因为它们对因子VIII相关抗原呈阳性。据我们所知，这是原发性骨髓纤维化转化为急性巨核细胞白血病的第三例。
