The severity of sickle cell disease (SCD) is usually associated with the level of hemoglobin S (HbS). For example, HbSS and HbSB0 thalassemia, which generally have higher HbS levels, are usually more severe than HbSC and HbSB+ thalassemia. While patients with HbSC and HbSB+ thalassemia typically hav...

I wonder what Sickle cell experts think about this: SC and SBeta plus have a different natural history than SS and SBeta zero, and calling them less severe may be a misnomer. They wouldn’t have a typical “sickle cell crisis” as often, but in adulthood, they often suffer from Severe...

I agree with Dr. @Kenneth I. Ataga. His observation is clear and correct. The clinical and hematological features of patients with SS are variable. The same observation applies to patients with SBo Thalassem. I learned something important during my 37 years of providing care to patients with sickle ...

Agree, SS and SB0 generally have severe phenotype compared to SC and SB+ thal. However, this is not always true as there are several other factors (as mentioned above) that play a role in the pathophysiology. Knowing why some patients with SS have minimal symptoms will help develop therapeutics and ...