Warm autoimmune hemolytic anemia

Section editor
	Benjamin Tillman, MD Vanderbilt University Nashville, TN

4 regimens on this page 3 variants on this page

Guidelines

British Society for Haematology

• 2017: Guidelines on the management of drug-induced immune and secondary autoimmune, haemolytic anaemia PubMed
• 2016: The diagnosis and management of primary autoimmune haemolytic anaemia PubMed

"How I Treat"

• 2021: Barcellini & Fattizzo How I treat warm autoimmune hemolytic anemia

All lines of therapy

Prednisolone monotherapy

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Regimen

Study	Evidence	Comparator	Efficacy
Birgens et al. 2013	Phase III (C)	Prednisolone & Rituximab	Seems to have inferior RFS

Immunosuppressive therapy

• Prednisolone (Millipred) 1.5 mg/kg/day PO for 2 weeks, then 0.75 mg/kg/day PO for 1 week (week 3), then 0.5 mg/kg/d PO for 1 week (week 4), followed by gradual reduction over the next 4-8 weeks to the lowest dose that was effective in maintaining a normal hemoglobin level.

References

1. Birgens H, Frederiksen H, Hasselbalch HC, Rasmussen IH, Nielsen OJ, Kjeldsen L, Larsen H, Mourits-Andersen T, Plesner T, Rønnov-Jessen D, Vestergaard H, Klausen TW, Schöllkopf C. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia. Br J Haematol. 2013 Nov;163(3):393-9. Epub 2013 Aug 24. link to original article PubMed

Prednisone monotherapy

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Regimen

Study	Evidence	Comparator	Efficacy
Michel et al. 2016 (RAIHA)	Phase III (C)	Prednisone & Rituximab	Seems to have inferior ORR

Immunosuppressive therapy

• Prednisone (Sterapred)

References

1. RAIHA: Michel M, Terriou L, Roudot-Thoraval F, Hamidou M, Ebbo M, Le Guenno G, Galicier L, Audia S, Royer B, Morin AS, Marie Michot J, Jaccard A, Frenzel L, Khellaf M, Godeau B. A randomized and double-blind controlled trial evaluating the safety and efficacy of rituximab for warm auto-immune hemolytic anemia in adults (the RAIHA study). Am J Hematol. 2017 Jan;92(1):23-27. Epub 2016 Nov 10. link to original article PubMed

Prednisolone & Rituximab

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Regimen

Study	Evidence	Comparator	Efficacy
Birgens et al. 2013	Phase III (E-esc)	Prednisolone	Seems to have superior RFS

Immunosuppressive therapy

• Prednisolone (Millipred) 1.5 mg/kg/day PO for 2 weeks, then 0.75 mg/kg/day PO for 1 week (week 3), then 0.5 mg/kg/d PO for 1 week (week 4), followed by gradual reduction over the next 4-8 weeks to the lowest dose that was effective in maintaining a normal hemoglobin level.
• Rituximab (Rituxan) 375 mg/m² IV once weekly for 4 weeks

Supportive care

• Folic acid 5 mg/day PO

References

1. Birgens H, Frederiksen H, Hasselbalch HC, Rasmussen IH, Nielsen OJ, Kjeldsen L, Larsen H, Mourits-Andersen T, Plesner T, Rønnov-Jessen D, Vestergaard H, Klausen TW, Schöllkopf C. A phase III randomized trial comparing glucocorticoid monotherapy versus glucocorticoid and rituximab in patients with autoimmune haemolytic anaemia. Br J Haematol. 2013 Nov;163(3):393-9. Epub 2013 Aug 24. link to original article PubMed

Prednisone & Rituximab

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Regimen

Study	Evidence	Comparator	Efficacy
Michel et al. 2016 (RAIHA)	Phase III (E-esc)	Prednisone	Seems to have superior ORR

Immunosuppressive therapy

• Prednisone (Sterapred)
• Rituximab (Rituxan)

References

1. RAIHA: Michel M, Terriou L, Roudot-Thoraval F, Hamidou M, Ebbo M, Le Guenno G, Galicier L, Audia S, Royer B, Morin AS, Marie Michot J, Jaccard A, Frenzel L, Khellaf M, Godeau B. A randomized and double-blind controlled trial evaluating the safety and efficacy of rituximab for warm auto-immune hemolytic anemia in adults (the RAIHA study). Am J Hematol. 2017 Jan;92(1):23-27. Epub 2016 Nov 10. link to original article PubMed