Table 2.

Clinical phenotypes

Clinical phenotypeFrequency
TypeDefinitionn%
Hemolytic anemia with circulatory symptoms grade 1 or absent 146 69.5 
Hemolytic anemia with circulatory symptoms grade 2-3 44 21.0 
Circulatory symptoms with compensated hemolysis 20 9.5 
All patients with available data 210 100.0 
Clinical phenotypeFrequency
TypeDefinitionn%
Hemolytic anemia with circulatory symptoms grade 1 or absent 146 69.5 
Hemolytic anemia with circulatory symptoms grade 2-3 44 21.0 
Circulatory symptoms with compensated hemolysis 20 9.5 
All patients with available data 210 100.0 

Circulatory symptoms grade 1, acrocyanosis only; grade 2, Raynaud-like symptoms interfering with daily living; grade 3, gangrene or ulcerations.

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