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Patient handout

Aplastic Anaemia / Acquired Bone-Marrow Failure (SAA / vSAA — chronic + subacute)

PRODUCTION

1. Your condition

This handout is for aplastic anaemia / acquired bone-marrow failure (saa / vsaa — chronic + subacute). Your care team identified this based on: pancytopenia (≥2 lineages cytopenic) on cbc — bone-marrow failure differential (bsh 2024 pmid 38247114).

Other reasons your team may use this plan: isolated or bilineage cytopenia progressing to pancytopenia (dezern blood adv 2021 pmid 34156438); petechiae / mucosal bleeding from severe thrombocytopenia (bsh 2024 pmid 38247114); recurrent / severe infection from neutropenia (bsh 2024 pmid 38247114).

2. Your medications

Take these medications exactly as prescribed. Do not stop or change a dose without talking to your provider.

MedicationStarting doseHowWhenWhat it does
horse antithymocyte globulin (lymphocyte immune globulin, ATGAM)40 mg/kg/day IV × 4 consecutive daysIVdaily × 4 daysHorse-ATG backbone of first-line IST — superior to rabbit-ATG (Scheinberg NEJM 2011 PMID 21812672: 6-mo ORR 68% vs 37%, 3-y survival 96% vs 76%). Premedicate (steroid/antihistamine); monitor serum sickness d7-14 (BSH 2024 PMID 38247114).
ciclosporin (cyclosporine)5 mg/kg/day PO divided BID (trough-adjusted ~200-400 ng/mL)POBIDCalcineurin inhibitor — second pillar of IST with horse-ATG; continue ≥12 mo then taper SLOWLY to limit relapse (BSH 2024 PMID 38247114; RACE PMID 34986284). Monitor trough, renal function, BP.
eltrombopag150 mg PO once daily (75 mg East-Asian ancestry); from day 1POonce dailyTPO-RA added UPFRONT to hATG + CsA — RACE NEJM 2022 PMID 34986284: 3-mo CR 22% vs 10%, 6-mo ORR 68% vs 41%, faster response (median 3.0 vs 8.8 mo); Townsley NEJM 2017 PMID 28423296 day-1 cohort 6-mo CR 58%/ORR 94%. Monitor LFTs + clonal evolution (BSH 2024 PMID 38247114).
rabbit antithymocyte globulin (Thymoglobulin)3.5 mg/kg/day IV × 5 days (HSCT conditioning) or salvage ISTIVdaily × 5 daysInferior to horse-ATG as first-line IST (Scheinberg PMID 21812672) — reserved for salvage IST or as serotherapy within HSCT conditioning where rabbit-ATG is preferred (ASTCT 2024 PMID 39307421).
romiplostim10 mcg/kg SC weekly, titrateSCweeklyAlternative TPO-RA in refractory AA where eltrombopag is not tolerated/available; monitor for clonal evolution (BSH 2024 PMID 38247114).
prednisolone1 mg/kg/day PO (ATG cover; serum-sickness short course)POdaily, taperedShort corticosteroid course with ATG to attenuate cytokine-release and serum sickness (NOT disease-modifying monotherapy) (BSH 2024 PMID 38247114).
filgrastim (G-CSF)5 mcg/kg/day SC (short course)SCdailyAdjunct in severe infection/sepsis only — does NOT improve survival or response and routine use discouraged (clonal-evolution concern); no substitute for definitive therapy (BSH 2024 PMID 38247114).
danazol200 mg PO BID-TIDPOBID-TIDAndrogen — historical / supportive role; rational in telomere-biology disorders (dyskeratosis congenita) and where IST/HSCT not feasible; monitor LFTs, virilisation (BSH 2024 PMID 38247114; DeZern PMID 34156438).
oxymetholone1-5 mg/kg/day POPOdailyAnabolic androgen — supportive option where IST/HSCT unavailable; hepatotoxicity / virilisation monitoring (BSH 2024 PMID 38247114).
allogeneic haematopoietic stem-cell transplantation (HLA-matched sibling / matched-unrelated / haploidentical)Curative for vSAA/SAA — first-line in young patients with HLA-matched sibling (5-y OS >90%); bone-marrow graft + rabbit-ATG conditioning preferred; Fanconi/IBMFS REQUIRES alkylator/irradiation DOSE-REDUCTION to avoid fatal toxicity (ASTCT 2024 PMID 39307421; BSH paeds PMID 29285764).
irradiated leucodepleted CMV-safe blood products (pRBC / platelets)Restrictive thresholds; irradiated + leucodepleted + CMV-safe if potential HSCT candidate; AVOID family-donor products pre-HSCT (alloimmunisation/graft-rejection risk) (BSH 2024 PMID 38247114; ASTCT 2024 PMID 39307421).
iron-chelation therapy (transfusional iron overload)Manage transfusional iron overload in transfusion-dependent AA; weigh cytopenia/marrow effects of chelator choice (BSH 2024 PMID 38247114).

Plan: Aplastic Anaemia Definitive Therapy (IST triplet + TPO-RA + supportive)

3. When to call your provider

Contact your care team if any of the following happen:

  • Fever / suspected neutropenic sepsis → ED + empiric antipseudomonal within 60 min (BSH 2024 PMID 38247114)
  • Platelets <10×10⁹/L or any significant bleeding → urgent platelet transfusion (BSH 2024 PMID 38247114)
  • Progression to SAA/vSAA (Camitta) → inpatient IST induction or HSCT (Camitta PMID 2893118)
  • New circulating blasts / evolving dysplasia → clonal-evolution route (heme.acute-leukemia.core.v1)

4. When to seek emergency care

Call 911 or go to the nearest emergency room right away if you have:

  • Very-severe AA: hypocellular marrow + ANC <0.2×10⁹/L (plus SAA criteria) (Camitta PMID 2893118)(life-threatening)
  • Temperature ≥38.0°C with ANC <0.5×10⁹/L in SAA/vSAA (BSH 2024 PMID 38247114)(life-threatening)
  • Platelets <10×10⁹/L (or <20×10⁹/L with bleeding/fever) (BSH 2024 PMID 38247114)
  • New circulating blasts / emerging dysplasia / acquired cytogenetic clone on surveillance (DeZern PMID 34156438)
  • Rapidly progressive marrow failure with antecedent/concurrent seronegative hepatitis ± hepatic failure (hep-AA series PMID 32925550)(life-threatening)
  • Expanding GPI-deficient PNH clone with intravascular haemolysis / thrombosis in an AA patient (DeZern PMID 34156438)

5. Follow-up

Long-term clonal-evolution surveillance (MDS/AML/PNH — periodic marrow + cytogenetics + flow); response reassessment at 3/6/12 mo (re-treat or escalate non-responders); taper ciclosporin slowly to limit relapse; vaccination/fertility counselling; transition paediatric → adult care; lifelong haematology follow-up given late clonal/relapse risk (BSH 2024 PMID 38247114; ASTCT 2024 PMID 39307421)

6. Sources

Guideline: BSH 2024 adult aplastic anaemia guideline (supersedes BSH 2016 Killick) + BSH paediatric amendment + ASTCT 2024 allo-HCT guideline + RACE NEJM 2022 + Camitta criteria

  1. pubmed.ncbi.nlm.nih.gov/38247114
  2. pubmed.ncbi.nlm.nih.gov/26568159
  3. pubmed.ncbi.nlm.nih.gov/29285764