Table 1.

The guidelines of the American Society of Hematology (ASH) discussed in this review

Recommendations or suggestions of ASHGrading of recommendationsRemarks
Questions 3 and 4:
Use of immunosuppressive therapy20  
ASH SUGGESTS:
Immunosuppressive therapy over no immunosuppressive therapy for high risk of acute hemolytic transfusion reaction or severe history of DHTR 
Conditional recommendation based on very low certainty in the evidence about effects Share decision-making process is critical to weigh the potential benefits and harms associated with transfusion versus the effect of ongoing SC symptoms
 
 ASH SUGGESTS:
Immunosupressive therapy over no immunosuppressive therapy in patients with DHTR and ongoing hyperhemolysis 
Conditional recommendation based on very low certainty in the evidence about effects Share decision-making process
Immunotherapy should be initiated promptly if ongoing hyperhemolysis with:
- First line: IVIG and high dose steroids
- Second line: Eculizumab
- Rituximab only to prevent additional antibodies
Precipitation of VOC with steroids should be considered 
Recommendations or suggestions of ASHGrading of recommendationsRemarks
Questions 3 and 4:
Use of immunosuppressive therapy20  
ASH SUGGESTS:
Immunosuppressive therapy over no immunosuppressive therapy for high risk of acute hemolytic transfusion reaction or severe history of DHTR 
Conditional recommendation based on very low certainty in the evidence about effects Share decision-making process is critical to weigh the potential benefits and harms associated with transfusion versus the effect of ongoing SC symptoms
 
 ASH SUGGESTS:
Immunosupressive therapy over no immunosuppressive therapy in patients with DHTR and ongoing hyperhemolysis 
Conditional recommendation based on very low certainty in the evidence about effects Share decision-making process
Immunotherapy should be initiated promptly if ongoing hyperhemolysis with:
- First line: IVIG and high dose steroids
- Second line: Eculizumab
- Rituximab only to prevent additional antibodies
Precipitation of VOC with steroids should be considered 

DHTR, delayed hemolytic transfusion reaction; IVIG, intra veinous immunoglobulin; SC, sickle cell; VOC, vaso-occlusive crisis.

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