Hemoglobin SC is less severe than hemoglobin SS but it may be associated with a number of serious complications.
Pathogenesis: increased blood viscosity
The risk for vaso-occlusive disease increases when the hemoglobin is above 10-11 g/dL.
The rate of vasculopathy-related conditions is less than in hemoglobin SS. This is associated with better survival.
Complications of hemoglobin SC include:
(1) vaso-occlusive crisis (VOC)
(2) thromboembolic (deep vein thrombosis, pulmonary embolism, stroke)
(3) acute chest syndrome (ACS)
(4) priapism
(5) bacterial sepsis
(6) retinopathy (including vitreous hemorrhage, retinal detachment, etc)
(7) avascular necrosis
(8) osteopenia or osteoporosis
(9) nephropathy, including papillary necrosis
(10) sensorineural hearing loss
(11) leg ulcer
(12) pulmonary hypertension
