Outpatient Clinics

Tasmanian Health Services
STATEWIDE CLINICAL GUIDELINE

Note: The electronic version of this document is the version currently in use.  Any printed version can not be assumed to be current.  Please remember to read our disclaimer.

• Background
• Objective
• Definitions
• Diagnosis
• Principles of Management
• Follow-up
• References
• Acknowledgements
• Appendix 1 - Vaccine access pathway flowchart

Background

The patient who has undergone a splenectomy (asplenia) or who has reduced splenic function (hyposplenism) is at increased risk of death due to infection.

Splenectomy has been estimated to carry a lifetime risk of overwhelming infection of up to 5%. The highest risk is within the first two years following splenectomy but one third of overwhelming infections occur at least five years later. Fulminant infections have been reported more than 20 years after splenectomy. The majority of serious infections are due to Streptococcus pneumoniae, Neisseria meningitidis, Haemophilus influenzae, Capnocytophagia canimorsus and malaria.

Patients who undergo splenectomy are easily identified but there are many other medical conditions that may be associated with hyposplenism. These include haematologic diseases, neoplasias, splenic irradiation and high dose steroid therapy. Coeliac disease and severe liver disease are the commonest non-haematologic causes of functional hyposplenism.

Objective

This guideline has been developed to assist in the management of patients with asplenia or hyposplenism patients in Tasmania.

Definitions

Asplenia: Absence of the spleen due to either surgical removal of the spleen or a congenital absence of the spleen.

Hyposplenism: Spleen is present but has impaired function.

Diagnosis

Asplenia

A history of splenectomy is usually sufficient for the diagnosis of asplenia.

If residual splenic tissue is present, it may indicate a lower risk for the development of infective complications but further advice should be sought from the Infectious Diseases Service in relation to ongoing management of these patients.

Asplenia may also be due to a congenital absence of the spleen.

Hyposplenism

Hyposplenism may be suggested by the following:

• Red cell changes on the blood film suggestive of hyposplenism including Howell-Jolly bodies, target cells, acanthocytes and Pappenheimer bodies (in the absence of other associated conditions such as myelodysplasia, megaloblastic anaemia and haemolysis)
• Low IgM memory B cells or impaired generation of IgM memory B cells

If hyposplenism is suspected with no clinically apparent cause, further investigation is warranted and referral to a Clinical Haematologist may be appropriate. Conditions associated with hyposplenism include the following:

• Gastrointestinal and hepatic diseases
  • Coeliac disease
  • Dermatitis herpetiformis
  • Inflammatory bowel disease
  • Cirrhosis
• Autoimmune disorders
  • Systemic lupus erythematosus
  • Rheumatoid arthritis
  • Vasculitis
  • Sarcoidosis
• Haematologic and neoplastic disorders
  • Sickle haemoglobinopathies
  • Graft-versus-host disease
  • Chronic lymphocytic leukaemia
  • Lymphoma
  • Amyloidosis
• Miscellaneous
  • Malaria
  • Disseminated meningococcemia
  • Previous radiotherapy to spleen

Principles of Management

1. Registration onto Spleen Registry

All asplenic or hyposplenic patients should be offered registration onto the Spleen Australia registry. Registration can be done on line via the Spleen Australia website by the treating doctor once patient consent has been obtained. Verbal consent is sufficient and the person responsible for registering the patient is required to indicate this on the on-line registration page. The treating doctor undertaking the registration could include the patient’s General Practitioner (GP), their medical specialist or their treating doctor whilst an inpatient.

Once the patient has been registered, the following will occur:

• A letter will be sent to the patient’s GP from Spleen Australia  outlining their condition and the management plan including administered and planned vaccination dates.
• Patient information will be provided to the patient via Spleen Australia directly.

2. Antibiotics (Prophylaxis And Emergency Supply) And Vaccinations

Antibiotic prophylaxis should be commenced according to the Spleen Australia website.

Emergency antibiotic supply should also be considered and the patient should be appropriately educated about its use. Please refer to Spleen Australia website for further information.

3. Vaccinations and their Access through THS Pharmacy Departments

Vaccination should be commenced according to the Spleen Australia website. Please also refer to the current version of the Australian Immunisation Handbook for further detailed information in relation to recommendation vaccinations and dosage schedules, particularly for additional doses recommended for paediatric patients who will have pneumococcal, meningococcal and HiB vaccines included in their vaccination schedule.

Given the cost associated with vaccines for this group of patients, GPs are able to access state government subsidised vaccinations directly through THS Pharmacy Departments. For information on how to access this program, please refer to the vaccination access pathway outlined in Appendix 1. Completion of a Referral and Prescription Form, accessible from the ID Outpatient Clinics page should be undertaken by the referring GP, and sent to the THS Pharmacy as outlined in Appendix 1.

Follow-up

Follow-up of the patients will be with their LMO or GP.

Information will also be sent to the LMO/GP by the Spleen Australia Service (as above).

Additional support will be provided by the Spleen Australia Service, Infectious Diseases Service or Haematology Service as required.

References

1. Therapeutic Guidelines: Antibiotic, version 15, 2014. Therapeutic Guidelines Limited.
2. Guidelines for the prevention of sepsis in asplenic and hyposplenic patients. D. Spelman, J. Buttery, A. Daley, D. Isaacs, I. Jennens, A. Kakakios, R. Lawrence, S. Roberts, A. Torda, D.A.R. Watson, I. Woolley, T. Anderson, A. Street. Internal Medicine Journal 2008; 38(5): 349-356
3. Australian Immunisation Handbook, 10th Edition, 2013
4. Marshall Lichtman. Williams Haematology 8^th Edition 2010.

Key Stakeholders

• Spleen Australia – a clinical service and registry for people with a non-functioning spleen
• HOD, Microbiology and Infectious Diseases, RHH (also providing ID support to the NWRH and MCH)
• Head of Infectious Diseases Service, LGH
• ID Pharmacist, RHH
• Statewide Pharmacy Director
• HOD, Medical Oncology and Haematology, RHH
• HOD, General Surgery, RHH
• HOD, Paediatrics, RHH
• GP Liaison Officer

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