Yasunaga M, Hodohara K, Bamba F, Kitamura K, Fukuda M, Boku T, Miyagawa A, Inoue T, Kitoh K, Andoh A
2nd Department of Internal Medicine, Shiga University of Medical Science, Otsu.
Rinsho Byori. 1995 Oct;43(10):1078-82.
A 45-year-old man was admitted with high fever and leukocytosis in August 1993. The diagnosis of acute myelogenous leukemia (AML; M2) was made on the basis of morphological, cytochemical and immunological characteristics of the blasts in the bone marrow. The induction therapy with BHAC, daunorubicin, 6-MP was unsuccessful in achieving remission; the bone marrow biopsy specimen revealed the proliferation of the remaining leukemic cells and massive fibrosis accompanied with unusual megakaryocyte-like giant bizarre cells. These megakaryocyte-like giant cells were positive for myeloperoxidase and CD34, but not GPIIIa and factor VIII, indicating that those were derived from myelogenous stem cells. Following the low-dose Ara-C therapy, improvement of fibrosis and disappearance of these giant cells were observed in the bone marrow. After the reinduction therapy with high-dose Ara-C and MIT against markedly increased blasts, the patient died of systemic fungal infection. The presence of myelofibrosis and giant atypical blasts might allow resistance to therapy and poor prognosis.
1993年8月，一名45岁男性因高热和白细胞增多症入院。根据骨髓中原始细胞的形态、细胞化学和免疫学特征，诊断为急性髓系白血病（AML；M2）。使用BHAC、柔红霉素、6-巯基嘌呤进行诱导治疗未能实现缓解；骨髓活检标本显示残留白血病细胞增殖以及大量纤维化，并伴有异常的巨核细胞样巨大怪异细胞。这些巨核细胞样巨细胞髓过氧化物酶和CD34呈阳性，但GPIIIa和因子VIII呈阴性，表明它们源自髓系干细胞。低剂量阿糖胞苷治疗后，骨髓中纤维化改善，这些巨细胞消失。在用高剂量阿糖胞苷和米托蒽醌针对明显增多的原始细胞进行再诱导治疗后，患者死于全身性真菌感染。骨髓纤维化和巨大非典型原始细胞的存在可能导致治疗抵抗和预后不良。
