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

neonates (<28 days old)

1st line – supportive care ± red blood cell transfusions

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ACUTE
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neonates (<28 days old)
1st line – 

supportive care ± red blood cell transfusions

When HS is diagnosed early, up to 76% of affected newborns require one or more transfusions during the first 6 to 12 months of life, despite often having normal haemoglobin values immediately following birth.[27]

The transfusion requirement early in life does not appear to predict the severity of the disease or the need for continued regular transfusions beyond the first year of life.[27]

Consider – folic acid supplementation

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ACUTE
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neonates (<28 days old)
Consider – 

folic acid supplementation

Additional treatment recommended for SOME patients in selected patient group

In addition to other therapy, patients with significant haemolysis may benefit from folic acid supplementation.[5]

Primary options

folic acid: neonates: consult specialist for guidance on dose

Consider – phototherapy or exchange transfusion

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ACUTE
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neonates (<28 days old)
|
with jaundice
Consider – 

phototherapy or exchange transfusion

Additional treatment recommended for SOME patients in selected patient group

Neonatal jaundice occurs in about 50% of patients with HS. Jaundice typically occurs within the first 24 hours of life and bilirubin levels may reach levels at which treatment with phototherapy and/or exchange transfusion is indicated. Current guidelines for neonatal jaundice should be followed to determine appropriateness of therapy.[28]​​

infants (>28 days old), children, and adults: severe HS

1st line – supportive care + red blood cell transfusions for symptomatic anaemia

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ACUTE
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infants (>28 days old), children, and adults: severe HS
1st line – 

supportive care + red blood cell transfusions for symptomatic anaemia

Splenectomy is generally considered the treatment of choice in patients with severe HS.[5][29][30]However, patients should be managed with transfusions for symptomatic anaemia until a time when splenectomy is deemed appropriate.

Transfusion may become necessary during an infection with parvovirus B19 that results in aplastic crisis or during episodes of hyperhaemolytic crisis.

It is best to avoid surgical splenectomy until at least 6 years of age to reduce the risk of post-splenectomy sepsis. However, patients with the most severe anaemia requiring regular transfusions may be candidates for splenectomy at a younger age (generally not prior to 2 years of age).

Beyond the neonatal period most patients can tolerate a Hb value as low as 60 g/L (6 g/dL) without the need for regular transfusions.

Plus – folic acid supplementation

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ACUTE
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infants (>28 days old), children, and adults: severe HS
Plus – 

folic acid supplementation

Treatment recommended for ALL patients in selected patient group

Patients with significant haemolysis (e.g., with a reticulocyte count of >5%) may benefit from folic acid supplementation to prevent megaloblastic anaemia. There are no studies to establish best practice.

Primary options

folic acid: 2-5 mg orally once daily

2nd line – splenectomy with preoperative vaccination regimen

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ACUTE
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infants (>28 days old), children, and adults: severe HS
2nd line – 

splenectomy with preoperative vaccination regimen

Splenectomy is considered the treatment of choice in patients with severe HS.[5][29]

It is best to avoid surgical splenectomy until at least 6 years of age to reduce the risk of post-splenectomy sepsis. Transfusions for symptomatic anaemia may be required until then, although patients with the most severe anaemia may undergo splenectomy at a younger age (generally not prior to 2 years of age).

Partial splenectomy is an option for some children. This is because it preserves some immune functions of the spleen while reducing haemolysis.[41] This has some limitations, and in the absence of convincing comparative studies remains an investigative procedure.[41]

Pre-splenectomy vaccination and post-splenectomy antibiotics reduce the risk of post-splenectomy sepsis, but they do not eliminate it.[30][35]

Patients undergoing splenectomy should be immunised with vaccines against Streptococcus pneumoniae, Haemophilus influenzae type b, and Neisseria meningitidis.[35][36]​​ ​​ CDC: ACIP vaccine recommendations and guidelines Opens in new window[45]

Three vaccines against pneumococcal disease are recommended: two pneumococcal conjugate vaccines (PCV15 and PCV20) and a pneumococcal polysaccharide vaccine (PPSV23). Choice of pneumococcal vaccine depends upon the age of the patient (minimum age: 6 weeks [PCV15], [PCV 20]; 2 years [PPSV23]) and medical status.[38][39]​ Consult local schedules for pneumococcal vaccination recommendations (and catch up guidance) in patients undergoing splenectomy.​[38][39][40]​​ UK HSA: UK immunisation schedule: the green book, chapter 11 Opens in new window​​

Children with anatomical or functional asplenia should be vaccinated with the quadrivalent meningococcal conjugate vaccine; those aged ≥10 years should receive the meningococcal serogroup B vaccine.

For elective splenectomy, immunisations should be given at least 2 weeks in advance of surgery if feasible.[36]​ However, they are also effective when given after splenectomy.​

Vaccines should be administered according to recommended vaccination schedules and preoperatively as required. CDC: Immunization schedules Opens in new window​​ UK HSA: UK immunisation schedule: the green book, chapter 11 Opens in new window​​

Folic acid is not required post-splenectomy.

Consider – cholecystectomy or cholecystostomy

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ACUTE
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infants (>28 days old), children, and adults: severe HS
Consider – 

cholecystectomy or cholecystostomy

Additional treatment recommended for SOME patients in selected patient group

Gallstones are common in HS and may be present in the first decade, increasing with age to up to 50% by 50 years of age.[43]

Ultrasound of the gallbladder should be performed prior to splenectomy. If there are symptomatic stones at the time of splenectomy, the gallbladder is removed simultaneously (cholecystectomy).

If asymptomatic gallstones are detected, options include splenectomy alone, removal of stones leaving the gallbladder (cholecystotomy) with splenectomy, or cholecystectomy with splenectomy.

Plus – post-splenectomy antibiotic pneumococcal prophylaxis

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ACUTE
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infants (>28 days old), children, and adults: severe HS
Plus – 

post-splenectomy antibiotic pneumococcal prophylaxis

Treatment recommended for ALL patients in selected patient group

Prophylactic penicillin should be administered for at least 3 years following splenectomy, and some practitioners advocate lifelong penicillin prophylaxis.[35] Guidelines vary and there is no clear evidence to guide practice.[16]

Post-splenectomy risk of overwhelming infection varies and those at highest risk (>50 years of age, documented inadequate response to vaccination, history of previous invasive pneumococcal disease or underlying haematological malignancy, particularly if immunosuppression is ongoing) should be offered lifelong antibiotic prophylaxis. Patients should carry a supply of appropriate antibiotics for emergency use.

If penicillin is not used (e.g., in areas with documented resistant strains), an alternative antibiotic to protect against pneumococcal infection may be appropriate. Amoxicillin has been recommended, and people who are allergic to penicillin may use erythromycin instead.[35]

Primary options

phenoxymethylpenicillin: children <5 years: 125 mg orally twice daily; children ≥5 years and adults: 250 mg orally twice daily

Secondary options

amoxicillin: children: 20 mg/kg orally once daily, maximum 250 mg/day; adults: 250 mg orally once daily

OR

erythromycin base: children: 7.5 mg/kg orally once daily, maximum 250 mg/day; adults: 250 mg orally once daily

infants (>28 days old), children, and adults: mild-to-moderate HS

1st line – supportive care + red blood cell transfusions for symptomatic anaemia

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ACUTE
|
infants (>28 days old), children, and adults: mild-to-moderate HS
1st line – 

supportive care + red blood cell transfusions for symptomatic anaemia

The management of mild-to-moderate HS is generally supportive, at least during early childhood. Red-cell transfusions may be required. Transfusion may become necessary during an infection with parvovirus B19 that results in aplastic crisis or during any episodes of hyperhaemolytic crises.

Consider – folic acid supplementation

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ACUTE
|
infants (>28 days old), children, and adults: mild-to-moderate HS
Consider – 

folic acid supplementation

Additional treatment recommended for SOME patients in selected patient group

Patients with significant haemolysis (e.g., with a reticulocyte count of >5%) may benefit from folic acid supplementation to prevent megaloblastic anaemia. There are no studies to establish best practice.

It is likely not to be necessary in mild disease as many foods are now supplemented with folic acid and deficiency is very rare in developed countries.

Primary options

folic acid: 2-5 mg orally once daily

2nd line – splenectomy with preoperative vaccination regimen

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ACUTE
|
infants (>28 days old), children, and adults: mild-to-moderate HS
2nd line – 

splenectomy with preoperative vaccination regimen

Patients likely to benefit from splenectomy include those with moderate, symptomatic anaemia; those who have had recurrent hyperhaemolytic crises; and those requiring multiple transfusions.

In patients with milder disease, the risks and benefits of splenectomy must be carefully weighed on an individual basis.[29] Splenectomy may be warranted in mild HS for issues related to reduced quality of life, such as bothersome jaundice, fatigue, poor growth, or poor school performance.

Pre-splenectomy vaccination schedules are the same as those described for patients with severe HS.[35]​​ CDC: ACIP vaccine recommendations and guidelines Opens in new window[45]

Check local vaccine recommendations. Vaccines should be administered according to recommended vaccination schedules and preoperatively as required. CDC: Immunization schedules Opens in new window UK HSA: UK immunisation schedule: the green book, chapter 11 Opens in new window

Folic acid is not required post-splenectomy.

Consider – cholecystectomy or cholecystostomy

Back
ACUTE
|
infants (>28 days old), children, and adults: mild-to-moderate HS
Consider – 

cholecystectomy or cholecystostomy

Additional treatment recommended for SOME patients in selected patient group

Gallstones are common in HS and may be present in the first decade, increasing with age to up to 50% by 50 years of age.[43]

Ultrasound of the gallbladder should be performed prior to splenectomy. If there are symptomatic stones at the time of splenectomy, the gallbladder is removed simultaneously (cholecystectomy). If asymptomatic gallstones are detected, options include splenectomy alone, removal of stones leaving the gallbladder (cholecystotomy) with splenectomy, or cholecystectomy with splenectomy.

There is some evidence that it is not always necessary to remove the spleen at the same time as performing surgery for symptomatic gallstones; each case should be assessed on its own merits.[44]

Plus – post-splenectomy antibiotic pneumococcal prophylaxis

Back
ACUTE
|
infants (>28 days old), children, and adults: mild-to-moderate HS
Plus – 

post-splenectomy antibiotic pneumococcal prophylaxis

Treatment recommended for ALL patients in selected patient group

Prophylactic penicillin should be administered for at least 3 years following splenectomy, and some practitioners advocate lifelong penicillin prophylaxis.[35] Guidelines vary and there is no clear evidence to guide practice.[16]

Post-splenectomy risk of overwhelming infection varies and those at highest risk (>50 years of age, documented inadequate response to vaccination, history of previous invasive pneumococcal disease or underlying haematological malignancy, particularly if immunosuppression is ongoing) should be offered lifelong antibiotic prophylaxis. Patients should carry a supply of appropriate antibiotics for emergency use.

If penicillin is not used (e.g., in areas with documented resistant strains), an alternative antibiotic to protect against pneumococcal infection may be appropriate. Amoxicillin has been recommended, and people who are allergic to penicillin may use erythromycin instead.[35]

Primary options

phenoxymethylpenicillin: children <5 years: 125 mg orally twice daily; children ≥5 years and adults: 250 mg orally twice daily

Secondary options

amoxicillin: children: 20 mg/kg orally once daily, maximum 250 mg/day; adults: 250 mg orally once daily

OR

erythromycin base: children: 7.5 mg/kg orally once daily, maximum 250 mg/day; adults: 250 mg orally once daily

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Choose a patient group to see our recommendations

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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