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 hemolysis

1st line – supportive care plus folic acid

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ACUTE
|
acute hemolysis
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 hematology consult is warranted once hemolytic anemia is diagnosed.

Folic acid is required to supply increased RBC production.

Primary options

folic acid (vitamin B9): 1-5 mg orally once daily for 14-21 days

Plus – blood transfusion

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ACUTE
|
acute hemolysis
|
severe anemia (Hb <7 g/dL) with no renal impairment
Plus – 

blood transfusion

Treatment recommended for ALL patients in selected patient group

A hematology consult is warranted once hemolytic anemia is diagnosed.

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

Absolute Hb 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.[39]

Plus – blood transfusion and renal support

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ACUTE
|
acute hemolysis
|
severe anemia (Hb <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 hematology consult is warranted once hemolytic anemia is diagnosed.

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

Blood from G6PD-deficient donors should not be used for neonatal transfusions and should certainly not be used when transfusing neonates with G6PD deficiency.[39]

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

Hemoglobinuria 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: consult specialist for guidance on dose

neonates with prolonged indirect hyperbilirubinemia

1st line – phototherapy

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ACUTE
|
neonates with prolonged indirect hyperbilirubinemia
1st line – 

phototherapy

Neonatal jaundice should be managed by pediatricians familiar with the appropriate guidelines.[42]​

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.[16]

Double-light phototherapy is more effective than single-light or fiberoptic phototherapy.[43]

Fiberoptic phototherapy is an alternative to conventional phototherapy in term neonates with physiologic jaundice.[44] [ Cochrane Clinical Answers logo ]

Plus – exchange transfusion

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ACUTE
|
neonates with prolonged indirect hyperbilirubinemia
|
with ongoing hemolysis or markedly elevated bilirubin levels
Plus – 

exchange transfusion

Treatment recommended for ALL patients in selected patient group

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

Neonatal jaundice should be managed by pediatricians 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 neurologic 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.[45]

Some specialists 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 embolization, necrotizing enterocolitis, and graft-versus-host disease.

ONGOING

chronic nonspherocytic hemolytic anemia

1st line – supportive care plus folic acid

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ONGOING
|
chronic nonspherocytic hemolytic anemia
1st line – 

supportive care plus folic acid

A hematology consult is warranted once hemolytic anemia is diagnosed.

Packed RBC transfusion is restricted to symptomatic anemia.

Folic acid is required to supply increased RBC production.

Primary options

folic acid (vitamin B9): 1-5 mg orally once daily

Plus – blood transfusion

Back
ONGOING
|
chronic nonspherocytic hemolytic anemia
|
severe anemia (Hb <7 g/dL) with no splenomegaly
Plus – 

blood transfusion

Treatment recommended for ALL patients in selected patient group

A hematology consult is warranted once hemolytic anemia is diagnosed.

Packed RBC transfusion is restricted to symptomatic anemia.

Absolute Hb 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.[39]

Consider – splenectomy

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ONGOING
|
chronic nonspherocytic hemolytic anemia
|
severe anemia (Hb <7 g/dL) with splenomegaly
Consider – 

splenectomy

Treatment recommended for SOME patients in selected patient group

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

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

Patients who are about to undergo splenectomy should receive appropriate immunizations (against pneumococcus, meningococcus, and Haemophilus influenzae) and start long-term antibiotic prophylaxis to protect against infection by encapsulated bacterial organisms.[46] CDC: ACIP vaccine recommendations and guidelines 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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