Minireview: Clinical severity in sickle cell disease: the challenges of definition and prognostication

Experimental Biology and Medicine - Tập 241 Số 7 - Trang 679-688 - 2016
Charles T. Quinn1
1Division of Hematology, Cincinnati Children’s Hospital Medical Center, 3333 Burnet Avenue, Cincinnati, OH 45220, USA

Tóm tắt

Sickle cell disease (SCD) is a monogenic, yet highly phenotypically variable disease with multisystem pathology. This manuscript provides an overview of many of the known determinants, modifiers, and correlates of disease severity in SCD. Despite this wealth of data, modeling the variable and multisystem pathology of SCD continues to be difficult. The current status of prediction of specific adverse outcomes and global disease severity in SCD is also reviewed, highlighting recent successes and ongoing challenges.

Từ khóa


Tài liệu tham khảo

10.1182/blood-2009-07-233700

10.3324/haematol.10937

10.1002/pbc.25608

10.1016/S0140-6736(00)04132-5

10.1177/003335491312800206

10.1056/NEJM199406093302303

10.1111/j.1365-2141.2005.05411.x

10.1182/blood-2011-03-325258

10.1182/blood-2013-09-528067

10.1111/j.1365-2141.1982.tb03915.x

10.1093/hmg/ddp401

10.1073/pnas.0804799105

10.1002/ajh.22221

10.1056/NEJM198202043060504

Ohene-Frempong K, 1998, Blood, 91, 288

10.1007/s00277-014-2016-1

10.1111/bjh.12791

10.1056/NEJM199111213252104

10.1182/blood.V63.6.1353.1353

Nouraie M, 2010, Br J Haematol, 150, 218, 10.1111/j.1365-2141.2010.08215.x

10.1111/bjh.12590

10.1002/ajh.21958

10.1111/bjh.12034

10.1182/blood-2008-03-143891

10.1111/j.1600-0609.2005.00477.x

10.1371/journal.pone.0034741

10.1034/j.1399-0004.2003.00113.x

10.1182/blood-2004-02-0521

10.1097/00043426-200110000-00011

10.1371/journal.pone.0077681

Chaar V, 2005, Haematologica, 90, 188

10.1155/2013/173474

10.1016/j.hoc.2004.07.004

10.1097/COH.0b013e32833ed177

10.3181/0807-RM-220

10.1056/NEJM200001133420203

10.1002/ajh.21846

10.1111/j.1600-0609.2011.01705.x

10.1056/NEJM199202273260905

10.1056/NEJM199807023390102

10.1002/pbc.24392

10.1016/S0140-6736(15)01041-7

10.1016/j.jcmg.2015.05.013

10.1056/NEJMoa035477

10.1016/j.jacc.2006.09.038

10.1111/bjh.12391

10.1111/j.1365-2141.2009.07779.x

Hagar RW, 2008, Br J Haematol, 140, 104, 10.1111/j.1365-2141.2007.06822.x

10.1097/MPH.0000000000000184

10.1182/blood.V87.6.2573.bloodjournal8762573

10.1182/blood.V96.9.3276

10.1542/peds.2004-2107

10.1080/03630260600642260

10.1111/j.1365-2141.2006.06409.x

10.1111/j.1365-2141.2012.09105.x

10.1182/blood-2007-07-100719

10.1182/blood-2007-04-084921

10.1001/jama.289.13.1645

10.1182/blood-2009-05-221333

10.1111/bjh.12323

10.1182/blood-2003-11-3758