Huang M J, Li C Y, Nichols W L, Young J H, Katzmann J A
Blood. 1984 Aug;64(2):427-39.
Acute leukemia with megakaryocytic differentiation has been an uncommonly recognized disorder. We used specific monoclonal and polyclonal antibody reagents (HP1-1D antibody and anti-factor VIII antibody, respectively) and an immunocytochemical staining technique to identify the megakaryocytic nature of the leukemic cells of 12 patients who presented with acute leukemia. The leukemic cells of our patients demonstrated the presence of one or both of these platelet- and megakaryocyte-related antigens, but were negative for all of the commonly employed cytochemical and immunocytochemical staining reactions, except for diffuse acid phosphatase activity and granular PAS positivity. Morphologically, the leukemic cells varied in size from 10 to 40 microns in diameter, frequently had cytoplasmic budding, and contained occasional vacuoles and/or peroxidase-negative azurophilic granules. Five patients presented with syndromes of acute myelofibrosis, and seven patients had otherwise unclassifiable acute leukemias, including three patients who had secondary leukemias. Diffuse reticulin myelofibrosis was present in all cases in which it was sought. Chromosomal abnormalities of leukemic cells were found in five cases. Two patients had deficiencies of plasma coagulation factor V. Study of one patient revealed significant platelet dysfunction. When cytoreductive chemotherapy of leukemia was attempted, the observed response was generally poor, with the exceptions of one patient who has remained in complete remission following treatment with etoposide (VP-16) and a second patient who attained remission following bone marrow transplantation. These cases of acute megakaryoblastic leukemia represented from 3.6% to 9.3% of all acute leukemia cases diagnosed concomitantly in our institution. Acute leukemia with megakaryocytic differentiation may occur more frequently than previously recognized, may present with differing syndromic features, and can be identified by the use of specific antibody reagents and relatively simple immunocytochemical techniques.
具有巨核细胞分化的急性白血病一直是一种较少被认识的疾病。我们使用了特异性单克隆和多克隆抗体试剂（分别为HP1 - 1D抗体和抗因子VIII抗体）以及免疫细胞化学染色技术，来鉴定12例急性白血病患者白血病细胞的巨核细胞性质。我们患者的白血病细胞显示出存在这些与血小板和巨核细胞相关抗原中的一种或两种，但对于所有常用的细胞化学和免疫细胞化学染色反应均为阴性，除了弥漫性酸性磷酸酶活性和颗粒状PAS阳性。形态学上，白血病细胞直径大小从10到40微米不等，经常有细胞质芽生，并且偶尔含有空泡和/或过氧化物酶阴性的嗜天青颗粒。5例患者表现为急性骨髓纤维化综合征，7例患者患有其他无法分类的急性白血病，其中包括3例继发性白血病患者。在所有进行检查的病例中均发现有弥漫性网状纤维骨髓纤维化。5例白血病细胞存在染色体异常。2例患者血浆凝血因子V缺乏。对1例患者的研究显示有明显的血小板功能障碍。当尝试进行白血病的细胞减灭化疗时，观察到的反应通常较差，但有1例患者在接受依托泊苷（VP - 16）治疗后一直处于完全缓解状态，另1例患者在骨髓移植后达到缓解。这些急性巨核细胞白血病病例占我们机构同期诊断的所有急性白血病病例的3.6%至9.3%。具有巨核细胞分化的急性白血病可能比以前认识到的更常见，可能表现出不同的综合征特征，并且可以通过使用特异性抗体试剂和相对简单的免疫细胞化学技术来识别。
