This handout is for haemolytic anaemia (aiha / g6pd / hereditary spherocytosis — chronic + acute crisis). Your care team identified this based on: anaemia + reticulocytosis (haemolytic pattern) (first intl consensus aiha 2020 pmid 31839434).
Other reasons your team may use this plan: ↑ldh + ↓haptoglobin + ↑indirect bilirubin → haemolysis screen (bsh 2017 pmid 28005293); jaundice / dark urine (haemoglobinuria — intravascular) (luzzatto nejm 2018 pmid 29298156); acute haemolysis after oxidant drug / fava / infection → g6pd (luzzatto nejm 2018 pmid 29298156).
Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.
| Medication | Starting dose | How | When | What it does |
|---|---|---|---|---|
| prednisolone | 1 mg/kg/day | PO | once daily | Warm AIHA first-line; ~70-80% initial response, slow taper over months (First Intl Consensus AIHA 2020 PMID 31839434; BSH 2017 PMID 28005293) |
| prednisone | 1 mg/kg/day | PO | once daily | Interchangeable with prednisolone for warm AIHA induction (First Intl Consensus AIHA 2020 PMID 31839434) |
| methylprednisolone | 250-1000 mg/day x 1-3 days | IV | once daily pulse | IV pulse for severe/fulminant warm AIHA before oral taper (First Intl Consensus AIHA 2020 PMID 31839434) |
| rituximab | 375 mg/m2 weekly x4 (or 1000 mg x2) | IV | weekly x4 | Birgens RCT — rituximab+prednisolone 75% vs 36% 12-mo response, better relapse-free survival, PMID 23981017; first-line in CAD (First Intl Consensus AIHA 2020 PMID 31839434) |
| sutimlimab | 6.5 g (<75 kg) or 7.5 g (≥75 kg) wk0, wk1, then q2w | IV | q2 weeks | CARDINAL — 54% composite Hb response, 71% transfusion-free wk5-26, PMID 33826820; CADENZA RCT PMID 35687757; meningococcal vaccination required |
| fostamatinib | 100 mg BID, escalate to 150 mg BID | PO | BID | FORWARD phase 3 — durable Hb response benefit in N.America/Australia/W.Europe subgroup, PMID 37929318 (refractory warm AIHA) |
| azathioprine | 1-2 mg/kg/day | PO | once daily | Steroid-sparing maintenance in warm AIHA (BSH 2017 PMID 28005293); avoid with allopurinol / dose-adjust |
| mycophenolate | 500-1000 mg BID | PO | BID | Steroid-sparing / refractory warm AIHA, especially secondary to SLE (First Intl Consensus AIHA 2020 PMID 31839434) |
| cyclophosphamide | oral or pulse IV per protocol | PO/IV | per protocol | Refractory AIHA after steroid + rituximab + splenectomy failure (First Intl Consensus AIHA 2020 PMID 31839434) |
| danazol | 200 mg BID-TID | PO | BID-TID | Steroid-sparing adjunct in warm AIHA (BSH 2017 PMID 28005293) |
| intravenous_immunoglobulin | — | — | — | Temporising in severe/paediatric warm AIHA; modest, transient effect (First Intl Consensus AIHA 2020 PMID 31839434) |
| splenectomy | — | — | — | Second/third-line for warm AIHA (NOT effective in cold agglutinin disease); pre-splenectomy vaccination mandatory (First Intl Consensus AIHA 2020 PMID 31839434) |
| plasma_exchange | — | — | — | Temporising in fulminant CAD / bridge; definitive only when routed to TTP (protocol.ttp) |
| least_incompatible_warmed_transfusion | — | — | — | Do NOT delay transfusion for life-threatening anaemia in AIHA; least-incompatible units, blood-warmer mandatory for CAD (BSH 2017 PMID 28005293) |
| cold_avoidance | — | — | — | Cornerstone non-pharmacologic management of cold agglutinin disease (Röth NEJM 2021 PMID 33826820) |
Plan: AIHA immunosuppression (warm AIHA + cold agglutinin disease)
Contact your care team if any of the following happen:
Call 911 or go to the nearest emergency room right away if you have:
Chronic haemolysis maintenance: lifelong folic acid; gallstone surveillance + iron-overload surveillance; post-splenectomy lifelong pneumococcal/meningococcal/Hib vaccination + penicillin prophylaxis + asplenia education; G6PD drug-avoidance card; relapse precautions for AIHA/CAD; periodic re-evaluation for secondary cause emergence (CLL/lymphoma); pregnancy planning + drug-safety review (BSH 2017 PMID 28005293)
Guideline: First International Consensus Meeting — Diagnosis & Treatment of AIHA in Adults (Jäger/Barcellini, Blood Reviews 2020) + BSH 2017 primary AIHA + BSH 2017 drug-induced/secondary AIHA + WHO 2022 G6PD classification + BSH 2011 hereditary spherocytosis