Anaemia, Aplastic

Description

Diagnostic criteria

General and supportive measures

Medicine treatment

Referral

---

ICD-10 code: D61.0

DESCRIPTION

Pancytopenia caused by bone marrow failure with a hypocellular bone marrow without infiltration or fibrosis. May be acquired or inherited. Inherited bone marrow failure syndromes include Fanconi anaemia, which has specific associated phenotypic features and chromosomal abnormalities.

DIAGNOSTIC CRITERIA

Clinical

• Pallor, petechiae, purpura, bleeding, with frequent and/or severe infections.
• Phenotypic features of Fanconi anaemia include:
  • Café-au-lait spots (skin pigmentary changes),
  • short stature and dysmorphic faces,
  • hypoplasia/absence of radius, fingerised thumb,
  • microcephaly, small eyes, hyperreflexia,
  • renal tract and cardiac abnormalities,
  • hypogonadism.

Investigations

• Full blood count shows anaemia (may be macrocytic), leucopenia and thrombocytopenia.
• Hypoplastic bone marrow.

GENERAL AND SUPPORTIVE MEASURES

Limit the liberal use of blood products as the patient may become sensitised and jeopardise future bone marrow transplant prospects. Avoid contact sport.

MEDICINE TREATMENT

For symptomatic anaemia (usually Hb < 7 g/dL):

• Packed red cells, IV, 4 x weight x deficit in haemoglobin.
  • Use leukocyte depleted products.

For active bleeding:

• Platelets, IV, 20 mL/kg, administered immediately and rapidly over 15–30 minutes through a platelet giving set.
  • If transplant is a possibility, use single donor, apheresis platelets rather than pooled, random donor platelets; preferably group specific.
  • Use the whole unit, unless the volume compromises cardiovascular status, (particularly in neonates). Apheresis platelets are available in paediatric volumes

For fever (T > 38 ºC), manage as febrile neutropenia in discussion with appropriate specialist/subspecialist with broad-spectrum antibiotics.
Take blood cultures first.

For febrile neutropenia:

Within 48 hours of admission:

• Ceftriaxone, IV, 50 mg/kg twice daily.

PLUS

• Gentamicin, IV, 6 mg/kg daily.

After 48 hours from admission:

Consider local antimicrobial resistance patterns when treating empirically, however, if this is not known then:

• Piperacillin-tazobactam, IV, 100 mg/kg 8 hourly.

Antibiotic adjustment based on microbiology results.

REFERRAL

• All cases of suspected aplastic anaemia.
• Stabilise patient before transport with blood and/or platelet transfusions, if necessary, after consultation with a paediatrician or paediatric haematologist.
• All cases for consideration for bone marrow transplant or immunosuppressive therapy in the case of acquired aplastic anaemia.

Click here to access the pdf.

---

Standard Treatment Guidelines and Essential Medicines List for South Africa Paediatric Hospital_2023 edition_Updated February 2025

Chapter 3: Blood and Blood-Forming Organs, 3.1 Anaemia, Aplastic