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 hereditary spherocytosis is diagnosed in the neonatal period it is likely to be more severe, with up to 76% of affected newborns requiring one or more transfusions during the first 6 to 12 months of life, despite often having normal hemoglobin values immediately following birth.[43]

The transfusion requirement early in life does not appear to predict the severity of the disease or the need for continued regular transfusions beyond of the first year of life.[43]

Consider – folic acid supplementation

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ACUTE
|
neonates (<28 days old)
Consider – 

folic acid supplementation

Treatment recommended for SOME patients in selected patient group

Patients with significant hemolysis may benefit from folic acid supplementation.[7]

Primary options

folic acid (vitamin B9): 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

Treatment recommended for SOME patients in selected patient group

Neonatal jaundice is common in hereditary spherocytosis.[7][23]​​ 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.[44]​​ See Neonatal jaundice.

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

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

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

supportive care + red blood cell transfusions for symptomatic anemia

Patients should be managed with transfusions for symptomatic anemia until splenectomy is deemed appropriate.

Beyond the neonatal period, most patients can tolerate a hemoglobin value as low as 6 g/dL without the need for regular transfusions. Supportive care with red-cell transfusions may be required, particularly if infection with parvovirus B19 results in aplastic crisis or during episodes of hyperhemolytic crises.

It is best to avoid surgical splenectomy in children below ages 5-6 years to reduce the risk of postsplenectomy sepsis.[7][46]​​ However, patients with the most severe anemia requiring regular transfusions may be candidates for splenectomy at a younger age (generally not prior to 2 years of age).

Plus – folic acid supplementation

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

folic acid supplementation

Treatment recommended for ALL patients in selected patient group

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

Primary options

folic acid (vitamin B9): 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 hereditary spherocytosis (HS)
2nd line – 

splenectomy with preoperative vaccination regimen

Splenectomy is generally considered the treatment of choice in patients with severe HS.[7][45][46]

It is best to avoid surgical splenectomy in children below ages 5-6 years to reduce the risk of postsplenectomy sepsis.[7][46]​​​ Transfusions for symptomatic anemia may be required until then, although patients with the most severe anemia may undergo splenectomy at a younger age (generally not prior to 2 years of age).

Splenectomy is best performed laparoscopically as it is associated with less pain and a quicker recovery than laparotomy, but this will depend upon local expertise and equipment.[7][46]

An alternative to full splenectomy is to remove part of the spleen (partial splenectomy) with the intention of leaving enough splenic tissue behind to preserve immune function.[52] Partial splenectomy may, theoretically, reduce the risk for postsplenectomy sepsis, but completion splenectomy may be required to address recurrence of hematologic problems or clinically significant abdominal pain.[46][53][54][55]​​​ Formal clinical trials with long-term follow-up are required.[45][46][57]

Presplenectomy vaccination and postsplenectomy antibiotics reduce the risk of postsplenectomy sepsis, but they do not eliminate it.[46][47] CDC: ACIP vaccine recommendations and guidelines Opens in new window​​

Patients undergoing splenectomy should be immunized with vaccines against Streptococcus pneumoniae, Haemophilus influenzae type b, and Neisseria meningitidis.[47][48]​​​​​ CDC: ACIP vaccine recommendations and guidelines Opens in new window​ Two vaccines against pneumococcal disease are recommended: a pneumococcal conjugate vaccine (PCV15, PCV20, or PCV21), or a pneumococcal polysaccharide vaccine (PPSV23). Choice of pneumococcal vaccine depends upon the age of the patient and medical status.[50][51]​​

Asplenic (anatomic or functional) children should be vaccinated with the quadrivalent meningococcal conjugate vaccine (MenACWY-CRM or MenACWY-TT); those ages ≥10 years should receive the meningococcal serogroup B vaccine (MenB-4C or MenB-FHbp).[50] Children ages 10 years or older may receive a dose of the pentavalent meningococcal vaccine (MenACWY-TT/MenB-FHbp) as an alternative to separate administration of the quadrivalent meningococcal vaccine and the meningococcal serogroup B vaccine when both vaccines would be given on the same clinic day.[50] Vaccines should be administered according to recommended vaccination schedules and preoperatively as required. CDC: Immunization schedules Opens in new window​​

Folic acid is not required postsplenectomy.

Consider – cholecystectomy or cholecystostomy

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

cholecystectomy or cholecystostomy

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.[14]

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).[7][46]​​

If asymptomatic gallstones are detected, options include splenectomy alone, removal of stones leaving the gallbladder (cholecystotomy) with splenectomy, or cholecystectomy with splenectomy.[7]

Plus – postsplenectomy antibiotic pneumococcal prophylaxis

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

postsplenectomy antibiotic pneumococcal prophylaxis

Treatment recommended for ALL patients in selected patient group

Prophylactic penicillin should be administered for at least 3 years following splenectomy; some practitioners advocate lifelong penicillin prophylaxis.[47] Guidelines vary and there is no clear evidence to guide practice.[24]

Lifelong antibiotic prophylaxis should be offered to the following patients who are at high risk for overwhelming infection postsplenectomy: over 50 years of age; with a documented inadequate response to vaccination; with a history of previous invasive pneumococcal disease; with an underlying hematologic malignancy, particularly if immunosuppression is ongoing.

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; people who are allergic to penicillin may use erythromycin.[47]

Primary options

penicillin V potassium: 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 hereditary spherocytosis (HS)

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

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

supportive care + red blood cell transfusions for symptomatic anemia

The management of mild-to-moderate HS is generally supportive, at least during early childhood.

Red-cell transfusions may be required, particularly if infection with parvovirus B19 results in aplastic crisis or during episodes of hyperhemolytic crises.

Consider – folic acid supplementation

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

folic acid supplementation

Treatment recommended for SOME patients in selected patient group

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

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

Primary options

folic acid (vitamin B9): 2-5 mg orally once daily

2nd line – splenectomy with preoperative vaccination regimen

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

splenectomy with preoperative vaccination regimen

Patients likely to benefit from splenectomy include those with moderate, symptomatic anemia; those who have had recurrent hyperhemolytic 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.[45] 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.

It is best to avoid surgical splenectomy in children below ages 5-6 years to reduce the risk of postsplenectomy sepsis.[7][46] ​Transfusions for symptomatic anemia may be required until then, although patients with the most severe anemia may undergo splenectomy at a younger age (generally not prior to 2 years of age).

Splenectomy is best performed laparoscopically as it is associated with less pain and a quicker recovery than laparotomy, but this will depend upon local expertise and equipment.[7][46]

An alternative to full splenectomy is to remove part of the spleen (partial splenectomy) with the intention of leaving enough splenic tissue behind to preserve immune function.[52] ​Partial splenectomy may, theoretically, reduce the risk for postsplenectomy sepsis, but completion splenectomy may be required to address recurrence of hematologic problems or clinically significant abdominal pain.[46][53]​​[54][55] Formal clinical trials with long-term follow-up are required.[45][46][57]

Patients undergoing splenectomy should be immunized with vaccines against Streptococcus pneumoniae, Haemophilus influenzae type b, and Neisseria meningitidis.[47] CDC: ACIP vaccine recommendations and guidelines Opens in new window Two vaccines against pneumococcal disease are recommended: a pneumococcal conjugate vaccine (PCV15, PCV20, or PCV21), or a pneumococcal polysaccharide vaccine (PPSV23). Choice of pneumococcal vaccine depends upon the age of the patient and medical status.[50][51]

Asplenic (anatomic or functional) children should be vaccinated with the quadrivalent meningococcal conjugate vaccine (MenACWY-CRM or MenACWY-TT); those ages ≥10 years should receive the meningococcal serogroup B vaccine (MenB-4C or MenB-FHbp).[50] Children ages 10 years or older may receive a dose of the pentavalent meningococcal vaccine (MenACWY-TT/MenB-FHbp) as an alternative to separate administration of the quadrivalent meningococcal vaccine and the meningococcal serogroup B vaccine when both vaccines would be given on the same clinic day.[50]​ Vaccines should be administered according to recommended vaccination schedules and preoperatively as required. CDC: Immunization schedules Opens in new window

Consider – cholecystectomy or cholecystostomy

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

cholecystectomy or cholecystostomy

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.[14]

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).[7][46]​​ If asymptomatic gallstones are detected, options include splenectomy alone, removal of stones leaving the gallbladder (cholecystotomy) with splenectomy, or cholecystectomy with splenectomy.[7]

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.[58]

Plus – postsplenectomy antibiotic pneumococcal prophylaxis

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

postsplenectomy antibiotic pneumococcal prophylaxis

Treatment recommended for ALL patients in selected patient group

Prophylactic penicillin should be administered for at least 3 years following splenectomy; some practitioners advocate lifelong penicillin prophylaxis.[47] Guidelines vary and there is no clear evidence to guide practice.[24]

Lifelong antibiotic prophylaxis should be offered to the following patients who are at high risk for overwhelming infection postsplenectomy: over 50 years of age; with a documented inadequate response to vaccination; with a history of previous invasive pneumococcal disease; with an underlying hematologic malignancy, particularly if immunosuppression is ongoing.

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; people who are allergic to penicillin may use erythromycin.[47]

Primary options

penicillin V potassium: 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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