Hamamoto K, Taniguchi H, Ohga S, Nagano T, Kishimoto Y, Kitajima H, Fujimoto M, Kimura T, Fujitake H, Yasunaga K
First Department of Internal Medicine, Kansai Medical University.
Rinsho Ketsueki. 1991 Nov;32(11):1481-5.
A 83-year-old man was diagnosed with primary myelofibrosis based on the presence of leukoerythroblastosis, splenomegaly, chromosome 46 XY, a dry tap bone marrow aspiration and fibrosis on bone marrow biopsy, when he was admitted for herpes zoster in June 1987. He was admitted for a second time with multiple subcutaneous tumors over his entire body in July, 1989. He had mild splenomegaly, but no hepatomegaly nor lymphadenopathy. Laboratory tests were as follows: RBC 214 x 10(4)/microliters, Hb 5.1 g/dl, Ht 17.7%, WBC 3,200/microliters with leukoerythroblastosis, platelets 11.6 x 10(4)/microliters, s-lysozyme 251 micrograms/ml, u-lysozyme 770 micrograms/ml, NAP ratio 98%, score 278. Bone marrow aspiration resulted in a dry tap. Bone marrow biopsy showed marked fibrosis. Histologic examination of subcutaneous tumor biopsy specimens revealed a diffuse infiltration of monocytes with flexuous nuclei. These cells were positive for alpha-naphtyl butyrate esterase stain, and negative for peroxidase, alpha-naphtol ASD chloroacetate esterase stain and platelet glycoprotein IIb/IIIa stain (APAAP). Ultrastructurally, these cells were mostly monocytes and promonocytes, while phenotypically, CD11b, CD13, CD14, CD33 and HLA-DR were positive. These date indicated that the subcutaneous tumors originated from monocytes.
一名83岁男性于1987年6月因带状疱疹入院时，根据存在幼稚粒-幼红细胞血象、脾肿大、染色体46 XY、骨髓穿刺干抽以及骨髓活检显示纤维化，被诊断为原发性骨髓纤维化。1989年7月，他因全身多处皮下肿瘤再次入院。他有轻度脾肿大，但无肝肿大及淋巴结病。实验室检查结果如下：红细胞214×10⁴/微升，血红蛋白5.1克/分升，血细胞比容17.7%，白细胞3200/微升，伴有幼稚粒-幼红细胞血象，血小板11.6×10⁴/微升，血清溶菌酶251微克/毫升，尿溶菌酶770微克/毫升，中性粒细胞碱性磷酸酶比值98%，积分278。骨髓穿刺干抽。骨髓活检显示明显纤维化。皮下肿瘤活检标本的组织学检查显示单核细胞弥漫性浸润，细胞核呈弯曲状。这些细胞α-萘丁酸酯酶染色阳性，而过氧化物酶、α-萘酚ASD氯乙酸酯酶染色及血小板糖蛋白IIb/IIIa染色（APAAP）阴性。超微结构上，这些细胞大多为单核细胞和原单核细胞，而表型上，CD11b、CD13、CD14、CD33和HLA-DR呈阳性。这些数据表明皮下肿瘤起源于单核细胞。
