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Diamond—Blackfan贫血又叫先天性纯红再障。起病缓慢,多见生后2~3个月发病,1岁后少见。其特点是发育落后和特殊面容,突眼、斜视、眼距宽、上唇厚、唇腭裂、指(趾)特别是拇指畸形、皮肤色素异常、先天性心脏病、骨骼畸形、肾发育不良、多囊肾、肾盂积水。血红蛋白常低于4克,网织红细胞减低,白细胞和血小板不减少。骨髓有核红细胞极少且多伴空泡,常可见数目不等原血细胞,比普通淋巴细胞小,核染色质均匀,少量
Diamond-Blackfan anemia is also called pure pure azoospermia. Slow onset, more common 2 to 3 months after onset of disease, rare after 1 year of age. It is characterized by poor development and special face, exophthalmus, strabismus wide, thick upper lip, cleft lip and palate, finger (toe) especially the thumb deformity, skin pigmentation disorders, congenital heart disease, skeletal deformities, renal dysplasia, and more Cystic kidney, hydronephrosis. Often less than 4 grams of hemoglobin, reticulocytes decreased, white blood cells and platelets do not reduce. Bone marrow rare erythrocytes and many associated with vacuoles, often visible ranging from the original number of blood cells, smaller than normal lymphocytes, nuclear chromatin even, a small amount