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发育不全性急性白血病

Hypoplastic acute leukemia.

作者信息

Needleman S W, Burns C P, Dick F R, Armitage J O

出版信息

Cancer. 1981 Sep 15;48(6):1410-4. doi: 10.1002/1097-0142(19810915)48:6<1410::aid-cncr2820480624>3.0.co;2-4.

Abstract

There have been few reports of acute leukemia presenting with a hypocellular bone marrow. All patients diagnosed as having acute leukemia were identified during a recent six-year interval who had blast cells plus promyelocytes of greater than 30% and marrow cellularity of needle biopsy less than or equal to 50%. Of 195 patients analyzed, 15 (7.7%) fulfilled the criteria. Ten patients were men and five women; the median age was 68 years with a range of 40-82. Seven complained of fatigue of 6-12 months duration, five were seen with occult infection, and three were asymptomatic. Hepatosplenomegaly was absent in 93% and none had lymphadenopathy. Fourteen patients were pancytopenic with median leukocyte count at presentation of 1.5 X 10(9)/liter, hemoglobin of 9.0 g/dl, and platelet count of 55 X 10(9)/liter. Circulating blast cells were not observed in ten patients; in the other five they were less than 0.7 X 10(9)/liter. The morphology of all cases appeared myeloid and Auer rods were seen in three patients; however, in one the peroxidase was negative. Classification according to FAB criteria revealed ten to be M1, three to be M2, one M4, and one L2. Median survival of the entire group was seven months. Of seven patients receiving no chemotherapy, two survived longer than 1 year (14, 24.5 months), one is alive at 7+ months, and the median survival was seven months. Eight patients with life-threatening complications received various combination regimens including an anthracycline, cytosine arabinoside, 6-thioguanine, vincristine, and prednisone. Five died of treatment complications; two achieved durable complete remission and are free of disease at 17 and 27 months. It can be concluded that hypoplastic acute leukemia is a distinct nosologic entity affecting primarily older patients with myeloid leukemia. Remission induction therapy in patients who are seriously ill has a low success rate, and in some patients prolonged survival is possible with supportive care alone.

摘要

关于急性白血病表现为骨髓细胞减少的报道较少。在最近六年期间,所有被诊断为急性白血病的患者均符合以下条件:原始细胞加早幼粒细胞大于30%,针吸活检骨髓细胞密度小于或等于50%。在分析的195例患者中,15例(7.7%)符合标准。10例为男性,5例为女性;中位年龄为68岁,范围为40 - 82岁。7例主诉有6 - 12个月持续时间的疲劳,5例因隐匿性感染就诊,3例无症状。93%的患者无肝脾肿大,且均无淋巴结病。14例患者全血细胞减少,就诊时白细胞计数中位数为1.5×10⁹/L,血红蛋白为9.0g/dl,血小板计数为55×10⁹/L。10例患者未观察到循环原始细胞;另外5例患者循环原始细胞小于0.7×10⁹/L。所有病例的形态学表现均为髓系,3例患者可见Auer小体;然而,其中1例过氧化物酶阴性。根据FAB标准分类,10例为M1,3例为M2,1例为M4,1例为L2。整个组的中位生存期为7个月。7例未接受化疗的患者中,2例存活超过1年(14个月、24.5个月),1例在7个多月时仍存活,中位生存期为7个月。8例有危及生命并发症的患者接受了包括蒽环类药物、阿糖胞苷、6 - 硫鸟嘌呤、长春新碱和泼尼松在内的各种联合方案治疗。5例死于治疗并发症;2例实现了持久完全缓解,分别在17个月和27个月时无病生存。可以得出结论,增生低下型急性白血病是一种独特的疾病实体,主要影响老年髓系白血病患者。病情严重患者的缓解诱导治疗成功率较低,部分患者仅通过支持治疗即可实现较长生存期。

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