Ulukutlu L, Koç O N, Taşyürekli M, Cullu F, Tüzüner N, Ulutin O N, Oz F, Seger R A, Sağlamer L
Department of Pediatrics, Cerrahpaşa Medical School, University of Istanbul, Turkey.
Acta Paediatr Jpn. 1995 Apr;37(2):177-81. doi: 10.1111/j.1442-200x.1995.tb03293.x.
Multiple persistent vacuoles were seen in the neutrophils, monocytes and eosinophils of a 9 year old boy and his 10 year old sister. The siblings were both asymptomatic. In the bone marrow, the cytoplasmic vacuoles were also present in the promyelocytes, myelocytes and metamyelocytes, but not in the myeloblasts and they tended to be single and large in immature cells. The cytoplasmic vacuoles did not stain with PAS, Sudan Black or Oil Red O; Sudan III positivity of the vacuoles was found only in a very small number of granulocytes. The vacuoles appeared as round and bright bodies with phase contrast microscopy. By electron microscopy, the vacuoles contained material of low electron density and had no surrounding membrane. Granulocyte functions were unimpaired. Muscle biopsy showed normal morphology. This anomalous vacuolization of the leukocytes is consistent with familial Jordans anomaly.
在一名9岁男孩及其10岁姐姐的中性粒细胞、单核细胞和嗜酸性粒细胞中发现了多个持续性空泡。这对姐弟均无任何症状。在骨髓中，早幼粒细胞、中幼粒细胞和晚幼粒细胞的细胞质中也有空泡，但原始粒细胞中没有，且未成熟细胞中的空泡往往单个且较大。细胞质空泡对PAS、苏丹黑或油红O均不着色；仅在极少数粒细胞中发现空泡呈苏丹III阳性。在相差显微镜下，空泡呈现为圆形明亮小体。通过电子显微镜观察，空泡内含有低电子密度物质，且无周围膜结构。粒细胞功能未受损害。肌肉活检显示形态正常。这种白细胞异常空泡化与家族性乔丹氏异常相符。
