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骨髓停留症(myelokathexis)是一种罕见的疾病。其特征是由于骨髓细胞停留而引起严重的中性粒细胞减少。伴细胞形态异常。病人和方法现报道2例同胞姐妹患WHIM综合征(四肢及颈部有多发性扁平疣、低丙种球蛋白血症、反复窦肺部感染和骨髓停留症)。下列观察可证明为本综合征:骨髓检查显示髓样和淋巴样细胞增加,骨髓中分叶粒细胞增多,核右移。口服强的松40mg,
Myelokathexis is a rare disease. It is characterized by severe neutropenia caused by the presence of myeloid cells. With abnormal cell morphology. Patients and Methods Two siblings with WHIM syndrome (multiple flat warts on both extremities and neck, hypoagglutaminemia, repeated sinus infections, and bone marrow arrest) are now reported. The following observations can prove to be syndrome: bone marrow examination showed increased myeloid and lymphoid cells, bone marrow more than the number of leaf cells, the nucleus right shift. Oral prednisone 40mg,