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 CAHS clinical 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).

Background^1,2,4 

• 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.

• 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.

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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