Asplenia, Hyposplenia and Complement Deficiency Vaccination and Prophylaxis

Disclaimer

These guidelines have been produced to guide clinical decision making for the medical, nursing and allied health staff of Perth Children’s Hospital. They are not strict protocols, and they do not replace the judgement of a senior clinician. Clinical common sense should be applied at all times. These clinical guidelines should never be relied on as a substitute for proper assessment with respect to the particular circumstances of each case and the needs of each patient. Clinicians should also consider the local skill level available and their local area policies before following any guideline. 

Read the full PCH Emergency Department disclaimer.

Aim

To provide guidance to PCH clinical staff on the vaccination schedule and prophylactic antibiotic therapy for children who have no spleen (asplenia), or a non-functioning spleen (hyposplenia), or who have a deficiency of the classical or alternative complement pathway (complement deficiency).

Background1,2,4 

Patients with asplenia or hyposplenia are at life-long risk of severe infection with encapsulated bacteria (Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae type b) and influenza virus. Patients with complement deficiency are at variable risk of invasive encapsulated bacterial infection, depending on the nature of the complement pathway defect. Vaccination and empiric antibiotic management is warranted to decrease morbidity and mortality. The risk of infection is greatest within 3 years of splenectomy or, in the setting of congenital asplenia, the first three years of life. These patients include, but are not limited to:
  • Post-traumatic or elective splenectomy
  • Congenital asplenia, including hyposplenia or polysplenia
  • Cancer-related asplenia
  • Complement deficiency
  • Splenic infarction and or sequestration e.g. sickle cell disease.

Definitions

  • 13vPCV – 13 valent pneumococcal conjugate vaccine (Prevenar 13®)
  • 23vPPV – 23 valent pneumococcal polysaccharide vaccine (Pneumovax 23®)
  • MenACWY – Meningococcal quadrivalent vaccine covering serogroups A, C, W-135 and Y
  • MenB – Meningococcal vaccine covering serogroup B

Assessing splenic function

There is no consensus regarding the best method for assessing splenic function and if there is concern regarding hyposplenism, a cautious approach and adherence to this guideline is recommended. Some investigations that may be useful to assist with identifying patients with hyposplenism include:
  • Ultrasound+/- Doppler to assess spleen size and blood flow
  • Howell Jolly bodies (intracellular nuclear remnants in erythrocytes) on a peripheral blood film, which must be specifically requested on the pathology form.
  • Other imaging modalities such as magnetic resonance imaging (MRI) or computerised tomography (CT) can further delineate anatomy in heterotaxy syndrome with polysplenia and technetium 99m sulphur colloid scintigraphy may be considered.

Assessing complement function

Investigation of patients with suspected complement deficiency (e.g. due to invasive encapsulated bacterial infection or family history) should be performed by or in consultation with a clinical immunologist to optimise test selection, timing of collection and interpretation of results.

Key Points1,2

1. Education:

  • All patients and families must be educated regarding:
    • The need for early investigation and management of any febrile illness or acute illness where the patient is unwell.
    • The symptoms and signs of bacterial infection and when to give standby antibiotics (see below).
  • All patients and families should be provided with written information, including a management plan and health fact sheet. This should also be provided to the patient’s general practitioner (GP) and any other care providers.
  • The patient should be strongly encouraged to wear a Medic Alert bracelet.
  • Further resources are available for patients and families on the Spleen Australia website at https://spleen.org.au/ (registration not currently available for patients in WA).
  • All patients should be referred to the Specialist Immunisation Clinic, Department of Infectious Diseases, Perth Children’s Hospital, for further education, and optimisation of antibiotic prophylaxis and immunisations.

2. Management of suspected infection:

  • Urgent presentation to hospital when febrile (temperature > 38°C) or with rigors, or when acutely unwell, is essential in patients with asplenia / hyposplenia / complement deficiency. In this situation, blood cultures should be taken and broad spectrum empiric antibiotics commenced as per local guidelines (Refer to the Sepsis and Bacteraemia - ChAMP Guideline).

3. Prophylactic antibiotics:

  • Should be administered at a minimum until patients are at least 5 years of age and for at least two years following splenectomy (see below). Lifelong antibiotic prophylaxis should be considered given patients are at lifelong risk of severe sepsis; this should be addressed on a case-by-case basis.

4. Vaccinations:

  • Individuals should importantly should be up to date with routine vaccinations (see online WA immunisation schedule), particularly Haemophilus influenzae B (Hib), and require additional pneumococcal, meningococcal, and annual influenza vaccination, due to their increased risk of overwhelming sepsis.
  • For elective splenectomy, vaccination should be administered at least 2 weeks before surgery is performed. However, if this is not possible, start vaccinations approximately 1 week after surgery, prior to discharge, with a vaccination plan in place. Obtain an inpatient consult from the Specialist Immunisation Service, Department of Infectious Diseases.
  • If a child with asplenia / hyposplenia / complement deficiency has also had a solid organ or haematopoietic stem cell transplant or is receiving chemotherapy or other immunosuppressive therapy; their immunisation requirements will differ, refer to the relevant guideline and refer to the Specialist Immunisation Clinic, Department of Infectious Diseases.
  • Household and close contacts of individuals should be up to date with age-appropriate recommended vaccinations, including annual influenza vaccination. The use of live vaccines in contacts of immunocompromised persons is safe, and strongly recommended.
  • There is no clear evidence to suggest that patients with asplenia / hyposplenia / complement deficiency are at increased risk of severe disease due to COVID-19 infection. COVID-19 vaccination is recommended for all who are in an eligible age group
    • The response to vaccination may be reduced in these patients, but it is expected that they will develop at least a partial response.

5. Clinical Alert

  • A PCH clinical alert must be in place for all patients with asplenia / hyposplenia or complement deficiency.
  • The treating team should complete a ‘Clinical Alert notification form’ (internal WA Health only) for entry onto webPAS by Clinical Coding. Refer to the Clinical Alerts Policy (internal WA Health only) in the PCH Operational Manual.

Recommended vaccination schedule for children with asplenia / hyposplenia

1. Streptococcus pneumoniae (Pneumococcus)

Due to higher disease burden and the potential for poorer antibody responses, all patients are recommended to complete a 4-dose primary course of 13vPCV (additional 4th dose to be given at 6 months of age or at diagnosis if older). Give the 1st dose of 23vPPV at 4 years of age and the 2nd dose of 23vPPV 5 years later. Pneumococcal catch-up schedules for children are outlined in Table 1.

Table 1: Pneumococcal vaccines3

Current age  Number of 13vPCV doses previously received Age at presentation   Age at 1st PCV dose Number of 13vPCV doses required #+ Number of 23vPPC doses required 
< 5 years None
< 12 months  N/A  4 2 lifetime doses **
12 - 59 months
 N/A
 2
 1
< 12 months  N/A
 3
12 - 59 months
 < 12 months  2
 > 12 months  1
 2  < 12 months  N/A  2
12 - 59 months  < 12 months  2 (if 2nd dose received at age > 12 months, 1 further 13vPCV dose is required)
 > 12 months  -
 3  < 12 months  N/A  1
 12-59 months  < 12 months  1 (if 2nd and 3rd doses received at age > 12 months, no further 13vPCV doses are required)
 4  N/A  N/A  - 
5 to 17 years 0 to ≤ 3 (excluding Prevenar7 and Prevenar10)  N/A  N/A  1 2 lifetime doses **

# π13vPCV (Prevenar13)/ 23vPPV (Pneumovax23); route IM, dose 0.5mL. The minimum interval between 13vPCV dose(s) is 1 month if aged <12 months, and 2 months if aged ≥12 months. The minimum interval between 13vPCV (recommended to be given first) and 23vPPV is 2 months. If 23vPPV is given first, a minimum interval of 12 months is recommended before 13vPCV can be given.

** The first 23vPPV dose should be given at age 4-5 years & at a minimum of 2 months after the last 13vPCV dose. The second dose of 23vPPV is recommended 5 years after the first and at a minimum of 2 months after 13vPCV.  

+ Menactra (MenACWY) should not be co-administered with 13vPCV as Menactra may interfere with the immune response against some pneumococcal serotypes. If co-administered, the 13vPCV dose should be repeated a minimum of 8 weeks later. Menveo and Nimenrix can be co-administered with 13vPCV.

2. Neisseria meningitidis (Meningococcus)

All patients require additional vaccines against N. meningitidis, which are hospital funded, including vaccinations against meningococcal ACWY (MenACWY) and meningococcal B (MenB).

Table 2: Meningococcal ACWY vaccines3

Age at commencement  Recommended brand(s)# Number of primary MenACWY doses# Recommended interval between primary doses  Timing of booster MenACWY doses
6 weeks to 5 months  Nimenrix OR Menveo  4 8 weeks; 4th dose at 12 months of age or 8 weeks after 3rd dose, whichever is later 3 years after completing primary schedule, then every 5 years thereafter
6 - 8 months Nimenrix OR Menveo
 3 8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
3 years after completing primary schedule, then every 5 years thereafter
9 -11 months Nimenrix OR Menveo
 3 8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
3 years after completing primary schedule, then every 5 years thereafter
> 12 months Nimenrix OR Menveo
 2 8 weeks If primary course completed at ≤ 6 years of age - 3 years after completing primary schedule, then every 5 years thereafter. If primary course completed at ≥ 7 years of age – every 5 years after completing primary schedule.

# Menveo/Niminrix; route IM, dose 0.5mL. MenACWY and MenB vaccines can be given at the same time

Menveo or Niminrix are the preferred brands over Menactra. Menactra should not be co-administered with 13vPCV as Menactra may interfere with the immune response against some pneumococcal serotypes. If co-administered, the 13vPCV dose should be repeated a minimum of 8 weeks later. Menveo and Nimenrix can be co-administered with 13vPCV.

Table 3: Meningococcal B vaccine3

Age at commencement  Recommended brand Number of primary MenB doses+ Interval between doses 
6 weeks to 5 months   Bexsero  4 8 weeks; 4th dose at 12 months of age or 8 weeks after 3rd dose, whichever is later
6 - 11 months  Bexsero

 3 8 weeks; 3rd dose at 12 months of age or 8 weeks after 2nd dose, whichever is later
12 months to 9 years  Bexsero

 2 8 weeks
> 10 years  Bexsero

 2 8 weeks

+All MenB vaccines (Bexsero) route IM, dose 0.5mL. MenACWY and MenB vaccines can be given at the same time. Prophylactic use of paracetamol is recommended when MenB is administered to children < 2 years of age. There is an increased risk of fever when MenB is co-administered with other routine vaccines, however this is not a contraindication.

3. Influenza

Table 4: Influenza vaccine 3

Age £ Influenza vaccine brand Route Dose Number of doses required if receiving the Influenza vaccine for the first time Number of doses required if previously received one or more doses of influenza vaccine
6 months to <9 years Refer to the WA immunisation schedule seasonal influenza vaccine recommendations IM 0.5mL 2 doses, minimum 4 weeks apart 1 dose#
9 years and over Refer to the WA immunisation schedule seasonal influenza vaccine recommendations IM 0.5mL 1 dose* 1 dose

£ All children with asplenia require annual influenza vaccine at the start of each influenza season, given their increased risk of severe infection.

# One dose is required in subsequent years even if only one dose was given in the first year of influenza immunisation. 

4. Haemophilus influenzae B vaccine (Hib)

All patients require review of their Hib vaccination status. Catch up schedules are outlined in Table 5.

Table 5: Haemophilus influenzae B vaccine3

Age at diagnosis Vaccine history Number of further primary doses required# Number of booster doses required at age > 18 months of age, or 2 months after the last dose, whichever is later
  Number of previous Hib doses Current age (months Age at time of 1st Hib vaccine (months
6 weeks to < 5 years No previous Hib doses  < 7 months  N/A  3  1
 7 - 11 months  N/A  2  1
 12 - 17 months  N/A  1  1
 18 - 59 months  N/A  1  -
1 previous Hib dose  < 12 months  < 7 months  2  1
 7-11 months  1  1
 12-17 months  < 12 months  1  1
 > 12 months  -  1
 18-59 months  < 18 months  -  1
 > 18 months  -  -
2 previous Hib doses  < 12 months  < 7 months  1  1
 < 12 months  7-11 months  -   1
12 - 17 months  > 12 months  -   -
12 - 17 months  > 12 months  -  -
 18 - 59 months  < 12 months  -  1
 > 12 months  -   - 
3 Hib doses received previously  All 3 doses at < 12 months
  
 -  1
 > 1 dose at 12-17 months  -  -
 > 5 years Primary course complete

 -   - 
Never received a dose of Hib-containing vaccine or primary course incomplete  -   1

For all Hib containing vaccines; route IM, dose 0.5mL. The minimum interval between primary doses for Hib is 4 weeks. Hib age appropriate combination vaccines are acceptable, e.g DTPa- HepB-IPV-Hib (Infanrix-hexa) on schedule at 6 weeks, 4 and 6 months. Monovalent Hib vaccines (Hiberix & Act-HIB) are registered for use in infants and children from age 2 months – 5 years.

5. Antibiotic prophylaxis 5,6

Prophylactic antibiotics should be administered until patients are at least 5 years of age and for at least 2 years following splenectomy. Lifelong prophylaxis may be warranted and must be addressed on a case by case basis.

See the Asplenia/ Hyposplenia health fact sheet. Refer to Infectious Diseases, Specialist Immunisation Clinic for ongoing management plan.

 

Age Prophylactic antibiotic
 < 1 year Amoxicillin 20mg/kg/dose (up to 250mg) once daily OR
Phenoxymethylpenicillin (Penicillin V) 62.5mg twice daily
 1 - 5 years Amoxicillin 20mg/kg/dose (up to 250mg) once daily OR
Phenoxymethylpenicillin (Penicillin V) 125mg twice daily
 > 5 years Amoxicillin 20mg/kg/dose (up to 250mg) once daily OR
Phenoxymethylpenicillin (Penicillin V) 250mg twice daily
 If low risk allergy to penicillins Discuss options with Infectious Diseases team
 If high risk allergy to penicillins Desensitisation may be an option if adherence to prophylaxis is likely. 

Standby antibiotic supply 5,6

A prescription for standby antibiotics should be supplied in case immediate access to medical care is not available (e.g. whilst on holiday or if living in remote areas). In this circumstance if a patient develops a fever (temp > 38°C) or rigors or is acutely unwell, they should commence the standby antibiotic immediately and seek urgent hospital/medical review.

Age at diagnosis  Standby antibiotic
 All ages Amoxicillin/Clavulanate 25mg/kg/dose of amoxicillin component (to a maximum of 875mg) twice daily (use DUO forte or DUO 400 preparations) until medical review
If low risk allergy to penicillins For children 3 months or older:
Cefuroxime 15mg/kg/dose (to a maximum of 500mg) twice daily until medical review
For children less than 3 months of age, discuss options with Infectious Diseases team
If high risk allergy to penicillins  Discuss options with Infectious Diseases team

6. Travel

Patients are at increased risk of severe disease and complications if they contract Plasmodium falciparum malaria, Salmonella infection and Babesiosis (rare tick infection). If travelling to endemic areas, Infectious Diseases advice should be sought prior to travel on antimalarial prophylaxis, mosquito avoidance and additional travel vaccines required.

7. Animal bites

There is an increased risk of severe sepsis following dog, cat or other animal bites/severe scratches (e.g. due to Capnocytophaga canimorsus, streptococcal sp, anaerobic bacteria). Early medical attention and antibiotic prophylactic treatment with amoxicillin/clavulanic acid is recommended (refer to Skin and Soft Tissue Infections - ChAMP Guidelines).

References

  1. Spelman D, Buttery J, Daley A et al. Guidelines for the prevention of sepsis in asplenic and hyposplenic patients. Internal Medicine Journal 2008; 38:349-356.
  2. Davies, JM, Lewis, PN et al. Review of guidelines for the prevention and treatment of infection in patients with an absent or dysfunctional spleen. British Journal of Haematology 2012. 155:308-317.
  3. Australian Technical Advisory Group on Immunisation (ATAGI). Australian Immunisation Handbook, Australian Government Department of Health, Canberra, 2018. Accessed from: Australian Immunisation Handbook
  4. Skattum L, van Deuren M, van der Poll T, Truedsson L. Complement deficiency states and associated infections. Molecular Immunology. 2011/08/01/ 2011;48(14):1643-1655. doi:https://doi.org/10.1016/j.molimm.2011.05.001
  5. Spleen Australia. Medical Guidelines for Individuals with Asplenia or Hyposplenism. February 2016 January 2019, V2.
  6. eTG complete: Antibiotic. Prevention of infection in patients with asplenia or hyposplenism. March 2021

Approved by: Medication Safety Committee Date: Jan 2022
Endorsed by: Drugs and Therapeutics Committee Date: Feb 2022


Review date:  Dec 2024


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