Naessens et al reported an algorithm for the management of a patient with Hemoglobin SC disease. The authors are from McGill University and the University of Toronto.
Patient selection: hemoglobin SC disease
Trigger - one or both of the following:
(1) chronic complications
(2) acute complications
If the baseline hematocrit is <= 35%:
(1) hydroxycarbamide titrated to maximal tolerated dose (MTD)
If baseline hematocrit is > 35%:
(1) phlebotomy to hematocrit < 35% (or hemoglobin 95-110 g/L)
(2) consider measuring viscosity and RBC deformability
(3) if baseline hemoglobin F > 2% consider hydroxycarbamide therapy titrated to MTD
A patient with a history of acute cerebrovascular events should be considered for chronic exchange blood transfusion.
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