Atkins J N, Muss H B
Am J Med Sci. 1985 Mar;289(3):110-3. doi: 10.1097/00000441-198503000-00004.
A patient whose initial hematologic evaluation suggested the diagnosis of a microangiopathic hemolytic anemia (MAHA) was further evaluated and found to have erythroleukemia (DiGuglielmo's syndrome). This prompted us to review retrospectively the peripheral blood morphology of 12 patients with erythroleukemia. Anisocytosis, poikilocytosis, macrocytosis, and nucleated red cells have been described in patients with erythroleukemia; however, changes characteristic of a microangiopathic hemolytic process (schistocytes) have not been previously described. Our patients with erythroleukemia had prominent helmet and fragmented red cells, as well as elliptocytosis. Six of our 12 patients with erythroleukemia did not have blasts on their peripheral smear, and platelets were decreased (platelet count ranged from 2 to 92 X 10(3)/microliter), resulting in changes similar to patients with MAHA due to thrombotic thrombocytopenic purpura (TTP), traumatic RBC lysis, and disseminated intravascular coagulation. Our data indicate the RBC changes characteristic of MAHA are commonly seen in erythroleukemia, and that as many as half of these patients may not have white cell changes suggestive of leukemia on the peripheral smear. Patients presenting with microangiopathic hemolytic anemia require a bone marrow examination to confirm or exclude a myelodysplastic syndrome.
一名患者最初的血液学评估提示诊断为微血管病性溶血性贫血（MAHA），进一步检查后发现患有红白血病（迪古列尔莫综合征）。这促使我们回顾性分析12例红白血病患者的外周血形态。红白血病患者中曾有关于红细胞大小不均、异形红细胞症、大红细胞症和有核红细胞的描述；然而，微血管病性溶血过程的特征性变化（裂红细胞）此前尚未见报道。我们的红白血病患者有明显的盔形红细胞和破碎红细胞，以及椭圆形红细胞症。我们的12例红白血病患者中有6例外周血涂片未见原始细胞，且血小板减少（血小板计数范围为2至92×10³/微升），导致出现与血栓性血小板减少性紫癜（TTP）、创伤性红细胞溶解和弥散性血管内凝血所致MAHA患者相似的变化。我们的数据表明，MAHA特征性的红细胞变化在红白血病中很常见，并且这些患者中多达一半在外周血涂片上可能没有提示白血病的白细胞变化。表现为微血管病性溶血性贫血的患者需要进行骨髓检查以确诊或排除骨髓增生异常综合征。
