Okazaki Shoichiro
Hematology, Ichinomiya Municipal Hospital, Ichinomiya, JPN.
Cureus. 2023 Jul 8;15(7):e41581. doi: 10.7759/cureus.41581. eCollection 2023 Jul.
A 62-year-old male patient presented with malaise and severe macrocytic anemia. Computed tomography revealed an osteolytic lesion in the left iliac bone. Bone marrow examination revealed that 90% of erythroblasts were large with periodic acid-Schiff (PAS)-positive staining while flow cytometry and immunostaining revealed CD71 (+), GP-A (+), p53 (+), CD117 (+), and CD34 (-) results, indicating pure erythroid leukemia (PEL) diagnosis. A needle biopsy of the osteolytic lesion revealed the same characteristics as PEL. Azacitidine therapy was administered as the first-line treatment, and his general condition temporarily improved. However, PEL quickly deteriorated, and he died 42 days of hospitalization after initial admission. PEL is an extremely rare form of acute myeloid leukemia (AML) and has presented cytogenetic characteristics in addition to the TP53 mutation. Other AML treatment is used because a standard treatment method is unavailable. However, the prognosis is extremely poor. Furthermore, few cases of concurrent bone lesions are reported globally, and more cases must be accumulated and analyzed.
一名62岁男性患者出现全身乏力和严重的大细胞性贫血。计算机断层扫描显示左髂骨有溶骨性病变。骨髓检查显示90%的成红细胞体积较大，糖原染色（PAS）呈阳性，而流式细胞术和免疫染色结果显示CD71（+）、GP-A（+）、p53（+）、CD117（+）和CD34（-），提示诊断为纯红细胞白血病（PEL）。对溶骨性病变进行穿刺活检，结果显示与PEL相同的特征。阿扎胞苷治疗作为一线治疗方案，患者的一般状况暂时有所改善。然而，PEL病情迅速恶化，初次入院后42天死亡。PEL是急性髓系白血病（AML）中极为罕见的一种形式，除了TP53突变外还具有细胞遗传学特征。由于没有标准的治疗方法，所以采用其他AML治疗方法。然而，其预后极差。此外，全球报道的并发骨病变病例很少，必须积累和分析更多病例。
