Warm autoimmune hemolytic anemia

Section editor
	Benjamin Tillman, MD Vanderbilt University Nashville, TN, USA LinkedIn

• We have moved How I Treat articles to a dedicated page.

4 regimens on this page 3 variants on this page

Guidelines

Given the rapid change in evidence in many areas of hematology/oncology, readers are encouraged to consider any guideline published 5+ years ago to be for historical purposes, only.

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

All lines of therapy

Prednisolone monotherapy

Regimen

Study	Evidence	Comparator	Efficacy
Birgens et al. 2013	Phase 3 (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/day PO for 1 week (week 4), then a 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

Regimen

Study	Evidence	Comparator	Efficacy
Michel et al. 2016 (RAIHA)	Phase 3 (C)	Prednisone & Rituximab	Seems to have inferior ORR

Immunosuppressive therapy

• Prednisone (Sterapred) 1 mg/kg PO once per day on days 1 to 14, then tapered "according to a pre-defined recommended reduction scheme"

One course

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 contains dosing details in abstract PubMed NCT01181154

Prednisolone & Rituximab

Regimen

Study	Evidence	Comparator	Efficacy
Birgens et al. 2013	Phase 3 (E-esc)	Prednisolone	Seems to have superior RFS (secondary endpoint)

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/day PO for 1 week (week 4), then a gradual reduction over the next 4 to 8 weeks to the lowest dose that was effective in maintaining a normal hemoglobin level.
• Rituximab (Rituxan) 375 mg/m² IV once per week for 4 weeks

Supportive therapy

• 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

Regimen

Study	Evidence	Comparator	Efficacy
Michel et al. 2016 (RAIHA)	Phase 3 (E-esc)	Prednisone	Seems to have superior ORR (primary endpoint)

Immunosuppressive therapy

• Prednisone (Sterapred) 1 mg/kg PO once per day on days 1 to 14, then tapered "according to a pre-defined recommended reduction scheme"
• Rituximab (Rituxan) 1000 mg IV once per day on days 1 & 15

One course

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 contains dosing details in abstract PubMed NCT01181154