Villamor N, Zarco M A, Rozman M, Ribera J M, Feliu E, Montserrat E
Servei d'Hematologia, Hospital Clinic de Barcelona, Spain.
Leukemia. 1998 Jul;12(7):1071-5. doi: 10.1038/sj.leu.2401074.
AML-M0 is an infrequent form of acute myeloblastic leukemia characterized by negative reaction with myeloperoxidase (MPO), Sudan Black and lymphoid antigens and positivity for CD13 or CD33. In the present study we describe the immunophenotypical and ultrastructural characteristics of a group of AML-M0 in adult patients. Nine out 218 AML leukemias (4.1%) fulfilled the AML-M0 criteria. CD13 or CD33 were positive in eight out nine cases, with two or more positive myeloid antigens being present in 82% of the cases. Immunological MPO was positive in 57% of the cases and CD68 in 33%. In no case megakaryocytic and erythroid markers present. Four cases (44%) expressed CD7 and TdT but only two coexpressed both antigens. In none of the cases was CD3 or CD22 cytoplasmic expression found. Ultrastructurally, a low number of granules was seen in all cases whereas ferritin particles or rhopheocytosis were not observed. Ultrastructural MPO was positive in one out of five cases and platelet peroxidase (PPO) was negative in the four cases studied. Two out of six cases showed karyotypic abnormalities (hypotetraploidy and a complex karyotype, respectively). In two out three cases a rearranged pattern for JH gene was observed. TCR (Cbeta and Jgamma) rearrangements were not detected in any case. AML-M0 is an infrequent form of acute myeloblastic leukemia. A large panel of myeloid monoclonal antibodies (MoAb) and the study of the cytoplasmic expression of myeloid antigens is necessary to diagnose this form of leukemia. AML-M0 usually coexpress lymphoid markers. Ultrastructural studies may be of help to discard an immature erythroid proliferation.
急性髓系白血病微分化型(AML-M0)是急性髓系白血病的一种罕见类型,其特征为髓过氧化物酶(MPO)、苏丹黑及淋巴样抗原呈阴性反应,而CD13或CD33呈阳性。在本研究中,我们描述了一组成年患者AML-M0的免疫表型和超微结构特征。218例急性髓系白血病中有9例(4.1%)符合AML-M0标准。9例中有8例CD13或CD33呈阳性,82%的病例存在两种或更多阳性髓系抗原。免疫组化MPO在57%的病例中呈阳性,CD68在33%的病例中呈阳性。无一例出现巨核细胞和红系标志物。4例(44%)表达CD7和末端脱氧核苷酸转移酶(TdT),但只有2例同时表达这两种抗原。所有病例均未发现CD3或CD22的胞质表达。超微结构方面,所有病例均可见少量颗粒,而未观察到铁蛋白颗粒或吞噬现象。5例中有1例超微结构MPO呈阳性,所研究的4例血小板过氧化物酶(PPO)均为阴性。6例中有2例显示核型异常(分别为亚四倍体和复杂核型)。3例中有2例观察到JH基因重排模式。所有病例均未检测到T细胞受体(Cβ和Jγ)重排。AML-M0是急性髓系白血病的一种罕见类型。诊断这种白血病需要使用大量髓系单克隆抗体(MoAb)并研究髓系抗原的胞质表达。AML-M0通常同时表达淋巴样标志物。超微结构研究可能有助于排除未成熟红系增殖。