Tsurumi H, Takahashi T, Koshino Y, Oyama M, Matsutomo K, Yasuda M, Moriwaki H, Muto Y
Department of Internal Medicine, Kisogawa Hospital.
Rinsho Ketsueki. 1991 Nov;32(11):1475-80.
A 64-year-old man was admitted to our hospital with leukopenia. On admission, leukocyte count in the peripheral blood was 1,600/microliters, containing 24.5% blasts of lymphoid appearance, which were negative for myeloperoxidase. A bone marrow aspiration showed hypoplasia with increased blasts (31.6%). The blasts were ultrastructurally positive for platelet peroxidase (PPO) and positive for platelet membrane glycoprotein IIb/IIIa complex. A diagnosis of acute megakaryoblastic leukemia was made. Chemotherapy with behenoyl-ara C (BH-AC) (150 mg/day) was transiently effective. However, after three months, numerous nodules without itching appeared over the entire body, particularly on the anterior chest. A biopsy of the skin lesion revealed a diffuse fibrosis with infiltrations of the blasts. Bone marrow aspirations were dry tap, and a bone marrow biopsy showed marked myelofibrosis. Then, severe headache, vomiting, and loss of consciousness developed, and a lumbar puncture revealed infiltrations of blasts. Although methotrexate was intrathecally injected, he died due to the respiratory failure. As far as we know, a case of acute megakaryoblastic leukemia with leukemia cutis and meningeal leukemia is quite rare. In addition, it is interesting that megakaryoblastic leukemia was accompanied with both the fibrosis of skin and the myelofibrosis.
一名64岁男性因白细胞减少症入住我院。入院时，外周血白细胞计数为1600/微升，其中24.5%为淋巴样原始细胞，髓过氧化物酶阴性。骨髓穿刺显示骨髓增生低下，原始细胞增多（31.6%）。原始细胞超微结构血小板过氧化物酶（PPO）阳性，血小板膜糖蛋白IIb/IIIa复合物阳性。诊断为急性巨核细胞白血病。用山嵛酰阿糖胞苷（BH-AC）（150毫克/天）化疗有短暂疗效。然而，三个月后，全身出现大量无瘙痒的结节，尤其是前胸。皮肤病变活检显示弥漫性纤维化伴原始细胞浸润。骨髓穿刺干抽，骨髓活检显示明显的骨髓纤维化。随后，患者出现严重头痛、呕吐和意识丧失，腰椎穿刺显示有原始细胞浸润。尽管鞘内注射了甲氨蝶呤，但患者因呼吸衰竭死亡。据我们所知，急性巨核细胞白血病伴皮肤白血病和脑膜白血病的病例非常罕见。此外，有趣的是，巨核细胞白血病同时伴有皮肤纤维化和骨髓纤维化。
