tailieunhanh - ANEMIAS AND OTHER RED CELL DISORDERS - PART 6

Một loạt các rối loạn phát sinh trong giai đoạn phôi thai và thời thơ ấu làm gián đoạn sản xuất tế bào bằng một hoặc nhiều hơn ba dòng máu. Một số các rối loạn ảnh hưởng đến một dòng duy nhất trong khi những người khác sản xuất pancytopenia. Một số rối loạn, | IV SECTION Stem Cell Dysfunction Copyright 2008 by The McGraw-Hill Companies Inc. Click here for terms of use. This page intentionally left blank 10 CHAPTER PEDIATRIC BONE MARROW FAILURE SYNDROMES FANCONI S ANEMIA CONGENITAL APLASTIC ANEMIA 186 CONGENITAL HYPOPLASTIC ANEMIA DIAMOND-BLACKFAN ANEMIA ERYTHROGENESIS IMPERFECTA 187 TRANSIENT ERYTHROBLASTIC ANEMIA OF CHILDHOOD 188 RED CELL APLASIA ASSOCIATED WITH PARVOVIRUS B19 INFECTION 189 PEARSON SYNDROME 190 1 DYSKERATOSIS CONGENITA 191 A wide range of disorders that arise during infancy and childhood disrupt cell production by one or more of the three hematopoietic lines. Some of the disorders affect a single lineage while others produce pancytopenia. Some disorders such as Fanconi s anemia FA commonly cause derangements in nonhematopoietic cells and tissues with the consequent finding of associated congenital anomalies. Most of the disorders present in the neonatal period or early in childhood. Improved diagnostic tools along with recognition of the variability inherent in these conditions now reveal mild manifestations or forme fruste in adolescents or even young adults. The uneven clinical texture of these conditions is a continuing challenge to clinicians. 185 Copyright 2008 by The McGraw-Hill Companies Inc. Click here for terms of .