Harashima S, Okamura T, Umeno M, Takaki K
Department of Internal Medicine, Sawara Hospital, Fukuoka.
Rinsho Ketsueki. 1996 Aug;37(8):713-8.
A 60-year-old Japanese woman was admitted to our hospital because of fatigue, weight loss and abdominal distension. Myelofibrosis was diagnosed, based on anemia, huge hepatosplenomegaly, leukoerythroblastosis and bone marrow fibrosis. Following treatment with ranimustine, anemia and splenomegaly improved. Seven months after initial therapy of ranimustine, however, polycythemia (RBC 7.39 x 10(6)/microliter; Hb 19.1 g/dl, Ht 65.9%) developed gradually, then RBC decreased to normal level following venesection (total 1,200 ml). After 32 months, blastic transformation occurred. The blasts were negative for myeloperoxidase. By flow cytometric analysis, the cells were positive for CD2, CD13, CD33 and HLA DR. Thus, AML (M0) was diagnosed. Despite of treatment with multicytotoxic agents, she died of DIC 36 months after the initial diagnosis of myelofibrosis. The progression from myelofibrosis to polycythemia is rare and only 15 cases have been reported so far. In addition, although a chromosomal abnormality, 46, XX, t(3; 12) (q25; p11), was present at the time of first diagnosis of myelofibrosis, the development of an additional abnormality, del(11) (q-), might be related to the transformation to AML.
一名60岁的日本女性因疲劳、体重减轻和腹胀入住我院。根据贫血、巨大肝脾肿大、幼稚粒-幼红细胞血象和骨髓纤维化诊断为骨髓纤维化。接受雷莫司汀治疗后，贫血和脾肿大有所改善。然而，在雷莫司汀初始治疗7个月后，逐渐出现红细胞增多症（红细胞7.39×10⁶/微升；血红蛋白19.1克/分升，血细胞比容65.9%），随后在放血（总量1200毫升）后红细胞降至正常水平。32个月后，发生原始细胞转化。原始细胞髓过氧化物酶阴性。通过流式细胞术分析，细胞CD2、CDl3、CD33和HLA DR阳性。因此，诊断为急性髓系白血病（M0）。尽管接受了多种细胞毒性药物治疗，她在骨髓纤维化初始诊断36个月后死于弥散性血管内凝血。骨髓纤维化进展为红细胞增多症较为罕见，迄今为止仅报道过15例。此外，虽然在骨髓纤维化首次诊断时存在染色体异常46, XX, t(3; 12) (q25; p11)，但额外异常del(11) (q-)的出现可能与向急性髓系白血病的转化有关。
