Table 1. The spectrum of sickle cell... | American Society of Hematology

Table 1.

The spectrum of sickle cell disease manifestations and effects of transplant

Organ	Pathology	Clinical manifestations	Effect of transplant
Blood and vessels	Red cell polymerization Red cell shape distortion ↓ membrane fluidity Abnormal rheology Red cell–leukocyte–endothelial adhesive interaction Oxidative stress	Hemolysis Chronic anemia Inflammation Infarction	Reversed
Brain	Stenosis Velocity perfusion imbalance Vessel beading Moyamoya	Posterior reversible encephalopathy syndrome Ischemic stroke Hemorrhagic stroke Silent infarcts	Improved or stabilized
Cardiopulmonary	Hemolysis Chronic anemia Ischemia reperfusion injury Red cell polymerization	Increased left ventricular mass Left ventricular diastolic dysfunction Pulmonary hypertension Acute chest syndrome Obstructive lung disease Restrictive lung disease	Improved or stabilized
Liver	Red cell polymerization Hemolysis Hyperbilirubinemia Iron accumulation	Sickle hepatopathy Transfusional hemosiderosis Biliary disease Hepatic fibrosis/cirrhosis	Improved or reversed
Spleen	Congestion, hemolysis Infarction	Splenic crisis and congestion Autosplenectomy	Improved or stabilized
Kidneys	Intramedullary sickling Papillary necrosis Hyperfiltration	Hematuria Acute kidney injury Chronic kidney disease Renal failure	Improved or reversed
Gonads	Iron deposition Microvascular occlusion Ovarian and testicular infarction	Urinary tract infection Dysmenorrhea Low ovarian reserve Priapism Low sperm count	Stabilized
Bone and joints	Perfusion impediment	Osteonecrosis Avascular necrosis	Progression halted
Muscles	Perfusion impediment	Vaso-occlusive episodes	Reversed

Organ	Pathology	Clinical manifestations	Effect of transplant
Blood and vessels	Red cell polymerization Red cell shape distortion ↓ membrane fluidity Abnormal rheology Red cell–leukocyte–endothelial adhesive interaction Oxidative stress	Hemolysis Chronic anemia Inflammation Infarction	Reversed
Brain	Stenosis Velocity perfusion imbalance Vessel beading Moyamoya	Posterior reversible encephalopathy syndrome Ischemic stroke Hemorrhagic stroke Silent infarcts	Improved or stabilized
Cardiopulmonary	Hemolysis Chronic anemia Ischemia reperfusion injury Red cell polymerization	Increased left ventricular mass Left ventricular diastolic dysfunction Pulmonary hypertension Acute chest syndrome Obstructive lung disease Restrictive lung disease	Improved or stabilized
Liver	Red cell polymerization Hemolysis Hyperbilirubinemia Iron accumulation	Sickle hepatopathy Transfusional hemosiderosis Biliary disease Hepatic fibrosis/cirrhosis	Improved or reversed
Spleen	Congestion, hemolysis Infarction	Splenic crisis and congestion Autosplenectomy	Improved or stabilized
Kidneys	Intramedullary sickling Papillary necrosis Hyperfiltration	Hematuria Acute kidney injury Chronic kidney disease Renal failure	Improved or reversed
Gonads	Iron deposition Microvascular occlusion Ovarian and testicular infarction	Urinary tract infection Dysmenorrhea Low ovarian reserve Priapism Low sperm count	Stabilized
Bone and joints	Perfusion impediment	Osteonecrosis Avascular necrosis	Progression halted
Muscles	Perfusion impediment	Vaso-occlusive episodes	Reversed