Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

ACUTE

acute haemolysis

1st line – supportive care plus folic acid

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ACUTE
|
acute haemolysis
1st line – 

supportive care plus folic acid

Affected patients should be encouraged to maintain a good fluid intake and to eat a light diet, as nausea is common.

A haematology consultation is warranted once haemolytic anaemia is diagnosed.

Folic acid is required to supply increased RBC production.

Primary options

folic acid: 1-5 mg orally once daily for 14-21 days

Plus – blood transfusion

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ACUTE
|
acute haemolysis
|
severe anaemia (haemoglobin <70 g/L [<7 g/dL]) with no renal impairment
Plus – 

blood transfusion

Treatment recommended for ALL patients in selected patient group

A haematology consultation is warranted once haemolytic anaemia is diagnosed.

Packed RBC transfusion is recommended in those with severe or symptomatic anaemia.

Absolute haemoglobin threshold for transfusion differs based on age and comorbidities. Blood from G6PD-deficient donors should not be used for neonatal transfusions and should certainly not be used when transfusing neonates with G6PD deficiency.[43]

Plus – blood transfusion and renal support

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ACUTE
|
acute haemolysis
|
severe anaemia (haemoglobin <70 g/L [<7 g/dL]) with renal impairment
Plus – 

blood transfusion and renal support

Treatment recommended for ALL patients in selected patient group

Erythropoietin can potentially assist in patients with inadequate endogenous erythropoietin levels, such as patients with severe kidney disease.

A haematology consultation is warranted once haemolytic anaemia is diagnosed.

Packed RBC transfusion is recommended in those with severe or symptomatic anaemia.

Blood from G6PD-deficient donors should not be used for neonatal transfusions and should certainly not be used when transfusing neonates with G6PD deficiency.[43]

In patients with impaired renal function receiving blood transfusions, additional measures may be required.

Haemoglobinuria can cause acute renal damage.

Dialysis may be required to support the patient until renal function recovers. As the renal injury is acute, dialysis is not likely to be required long-term.

Primary options

epoetin alfa: refer to consultant for guidance on dose

neonates with prolonged indirect hyperbilirubinaemia

1st line – phototherapy

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ACUTE
|
neonates with prolonged indirect hyperbilirubinaemia
1st line – 

phototherapy

Neonatal jaundice should be managed by paediatricians familiar with the appropriate guidelines.[46]

Phototherapy uses light energy to cause photochemical reactions to transform bilirubin into isomers that are less lipophilic and more easily excretable, making breakdown products that do not require conjugation in the liver. The most effective wavelengths are from 425 to 490 nm.[17]

Double-light phototherapy is more effective than single-light or fibre-optic phototherapy.[47]

Fibre-optic phototherapy is an alternative to conventional phototherapy in term neonates with physiological jaundice.[48] [ Cochrane Clinical Answers logo ]

Plus – exchange transfusion

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ACUTE
|
neonates with prolonged indirect hyperbilirubinaemia
|
with ongoing haemolyis or markedly elevated bilirubin levels
Plus – 

exchange transfusion

Treatment recommended for ALL patients in selected patient group

In the face of acute ongoing haemolysis or markedly elevated bilirubin levels, exchange transfusion should be considered early on.

Neonatal jaundice should be managed by paediatricians familiar with the appropriate guidelines.

Phototherapy is continued while waiting to start the procedure, stopped while doing the transfusion, and restarted as soon as the exchange transfusion is completed.

The rationale is to remove the unconjugated bilirubin by doing a double-volume exchange transfusion, which should allow the bilirubin to move out of the brain tissue and hence decrease the risk of neurological toxicities.

There is insufficient evidence to support or refute the use of single-volume exchange transfusion, as opposed to double-volume exchange transfusion, in jaundiced newborns.[49]

Some consultants recommend the use of 5% albumin before the exchange transfusion in an attempt to bind unconjugated (free) bilirubin.

The major potential complications of the procedure include electrolyte disturbances, bleeding, infection, cardiac arrhythmias, thrombosis with embolisation, necrotising enterocolitis, and graft-versus-host disease.

ONGOING

chronic non-spherocytic haemolytic anaemia

1st line – supportive care plus folic acid

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ONGOING
|
chronic non-spherocytic haemolytic anaemia
1st line – 

supportive care plus folic acid

A haematology consultation is warranted once haemolytic anaemia is diagnosed.

Packed RBC transfusion is restricted to symptomatic anaemia.

Folic acid is required to supply increased RBC production.

Primary options

folic acid: 1-5 mg orally once daily

Plus – blood transfusion

Back
ONGOING
|
chronic non-spherocytic haemolytic anaemia
|
severe anaemia (haemoglobin <70 g/L [<7 g/dL]) with no splenomegaly
Plus – 

blood transfusion

Treatment recommended for ALL patients in selected patient group

A haematology consultation is warranted once haemolytic anaemia is diagnosed.

Packed RBC transfusion is restricted to symptomatic anaemia.

Absolute haemoglobin threshold for transfusion differs based on age and comorbidities. Blood from G6PD-deficient donors should not be used for neonatal transfusions and should certainly not be used when transfusing neonates with G6PD deficiency.[43]

Consider – splenectomy

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ONGOING
|
chronic non-spherocytic haemolytic anaemia
|
severe anaemia (haemoglobin <70 g/L [<7 g/dL]) with splenomegaly
Consider – 

splenectomy

Additional treatment recommended for SOME patients in selected patient group

Splenectomy may be considered for those with significant extravascular haemolysis, marked splenomegaly, or persistent severe anaemia that is interfering with growth, development, or normal activity. This may result in a significant decrease in haemolysis.

Cholecystectomy may be considered at the same time if gallstones are present.

Patients who are about to undergo splenectomy should receive appropriate immunisations (against pneumococcus, meningococcus, and Haemophilus influenzae) and start long-term antibiotic prophylaxis to protect against infection by encapsulated bacterial organisms.[50] CDC: ACIP vaccine recommendations and guidelines Opens in new window UK Health Security Agency: Guidance of immunisation of individuals with underlying medical conditions: the green book, chapter 7 Opens in new window

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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