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[伴有强烈成红细胞反应的急性嗜碱性粒细胞白血病]

[Acute basophilic leukemia with an intense erythroblastic reaction].

作者信息

Batlle M, Ribera J M, García-Vidal O, Roncalés J, Millá F, Feliu E

机构信息

Servicio de Hematología-Hemoterapia, Hospital Universitari Germans Trias i Pujol, Badalona.

出版信息

Sangre (Barc). 1993 Oct;38(5):385-7.

PMID:8140501
Abstract

A patient is presented who had acute basophilic leukaemia with intense erythroblastic reaction. The patient, a 66 year-old man, complained of general malaise, increased abdominal perimeter and melena. Leucocytosis, as well as severe anaemia and thrombocytopenia, were found in his peripheral blood. Basophils were present in all maturation stages, along with 7% blast-cells showing basophilic stippling, and there were 210 erythroblasts per 100 white cells. Erythropoietic hyperplasia (75%) was found in the bone-marrow aspirate, without dyserythropoietic signs; the PAS-stain reaction was negative. Of the non-erythroid cells, 63% were basophils and 34% blast-cells, some of them showing basophilic stippling plus metachromasia for tholuidin-blue, positivity for omegaexonuclease and negativity for peroxidase stains. The diagnosis of acute basophilic leukaemia was confirmed upon demonstration of basophilic stippling in the ultrastructural study of the blast-cells. The patient developed acute liver failure and renal insufficiency which led him to death. The basis of the diagnosis of acute basophilic leukaemia is discussed, as well as the differential diagnosis with other conditions presenting with basophilia and the probably reactive erythroblastic increase appearing in this case.

摘要

本文报告一例患有急性嗜碱性粒细胞白血病并伴有强烈成红细胞反应的患者。该患者为一名66岁男性,主诉全身不适、腹围增大和黑便。其外周血检查发现白细胞增多,同时伴有严重贫血和血小板减少。外周血中可见各成熟阶段的嗜碱性粒细胞,还有7%的原始细胞呈现嗜碱性点彩,每100个白细胞中有210个成红细胞。骨髓穿刺显示红细胞生成增生(75%),无红细胞生成异常迹象;过碘酸雪夫(PAS)染色反应为阴性。在非红细胞系细胞中,63%为嗜碱性粒细胞,34%为原始细胞,其中一些原始细胞呈现嗜碱性点彩并对甲苯胺蓝有异染性,对ω-外切核酸酶呈阳性反应,而过氧化物酶染色呈阴性。在原始细胞的超微结构研究中发现嗜碱性点彩,从而确诊为急性嗜碱性粒细胞白血病。该患者出现急性肝衰竭和肾功能不全,最终死亡。本文讨论了急性嗜碱性粒细胞白血病的诊断依据,以及与其他伴有嗜碱性粒细胞增多的疾病的鉴别诊断,还有本例中可能出现的反应性成红细胞增多。

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