Home' Australian Pharmacist : Australian Pharmacist September 2016 Contents Australian Pharmacist September 2016 I © Pharmaceutical Society of Australia Ltd.
along with the occasional presence of
subcutaneous nodules.2,7,11,13 Within
synovial joints, the synovium and the
tidemark region of articular cartilage
are particularly susceptible to iron
deposition. This makes the cartilage
matrix stiffer and more predisposed to
fragmentation. In addition, iron salts
promote the formation and precipitation
of calcium pyrophosphate dihydrate
crystals within the joint, contributing to
subsequent joint degeneration.2
Individuals with more severe iron overload
may develop liver disease with fibrosis or
cirrhosis. Hepatocellular carcinoma has
been reported to develop in about 30%
of individuals with untreated cirrhosis
caused by hereditary haemochromatosis.
Diabetes also occurs more frequently in
hereditary haemochromatosis, especially in
advanced disease; overall, 20–50% of those
with symptomatic disease have diabetes.
Treatment of hereditary
haemochromatosis is through removal of
iron, so that total body iron stores return
to normal. Usually, this can be achieved
most efficiently by venesection of 500
mL blood (which depletes about 250 mg
of iron), initially every week or fortnight
until the serum ferritin concentration
is reduced to 50–100 microg/L.
Maintenance phlebotomy may then be
scheduled to maintain haemoglobin
concentrations above 11–12 g/dL
and the serum ferritin concentration
between 50–100 microg/L,
most patients requiring phlebotomy
every two to four months.3,5,7,10,14
venesection should be preceded by
measurement of haemoglobin and
haematocrit. Venesection should only
go ahead if the haematocrit is within
20% of the previous measurement
and/or haemoglobin is greater than
Venesection treatment is
performed through the Australian
Red Cross Blood Service, many public
hospitals and some private pathology
providers; if the individual has no
contraindications, the blood can be used
for donation purposes.
While the evidence is not definitive,
chronic use of proton pump inhibitors
by individuals with hemochromatosis
may decrease iron absorption,
diminishing the severity of overload
that would otherwise occur as the
result of excess absorption of iron from
dietary sources, or reduce requirements
for maintenance phlebotomy.
Conversely, vitamin C increases
iron mobilisation; individuals with
hemochromatosis should limit their intake
of supplemental vitamin C to 500 mg
7,15 Patients with haemochromatosis
should not consume raw shellfish, owing
to an increased susceptibility to infection
with Vibrio vulnificus.
While phlebotomy is very effective,
there are potential adverse effects,
including fatigue, fainting, pain at the
venous access site, haematomas, and
Negative experiences such
as travel, loss of time, and discomfort
by the procedure itself have been
reported by between one-third and
one-half of patients, and there tends
to be a constant rate of decline in the
percentage of patients who comply
with maintenance therapy.
surprisingly, most patients would
prefer to take medication rather than
Some patients with hereditary
haemochromatosis are intolerant
of phlebotomy because of baseline
anaemia, poor venous access (e.g.
with severe heart failure), intolerance
to phlebotomy, or coexisting
haematological disorders. These patients
should be considered for iron chelation
therapy, such as deferasirox.
expensive alternative to phlebotomy
is erythrocytapheresis, or the removal
of erythrocytes only rather than whole
blood. Here, blood is centrifuged,
separating the erythrocytes from
plasma. The red cells are removed while
the plasma is returned to the treated
individual. In addition saline can be
infused during the process to minimise
the risk of vasovagal symptoms.
than twice as much iron can be removed
per session compared with phlebotomy.
In addition, side effects are reduced and
it's preferred by patients.
it's likely that venesection will remain
the mainstay of treatment because of its
efficacy, tolerance, and low cost.
Useful resources for pharmacists and
consumers are available from the website
of Haemochromatosis Australia:
1. Axford JS, Bomford AB, Revell P, Watt I, Hamilton ED. A case of
haemochromatosis arthritis. Br J Rheumatol 1992;31(8):547–
2. Davies MB, Saxby T. Ankle arthropathy of hemochromatosis:
a case series and review of the literature. Foot Ankle Int
3. Mohamed M, Phillips J. Hereditary haemochromatosis. BMJ
4. Emery J, Rose P. Hereditary haemochromatosis: never seen a
case? Br J Gen Pract 2001;51(466):347–8 .
5. Salgia RJ, Brown K. Diagnosis and management of hereditary
hemochromatosis. Clin Liver Dis 2015;19(1):187–98.
6. Bentley P, Bell B, Olynyk J. Therapeutic venesection at
the Australian Red Cross Blood Service: impact of the
High Ferritin Application on management of hereditary
haemochromatosis. Aust Fam Physician 2015;44(8):589–92.
7. Delatycki M, Allen K. Hereditary haemochromatosis.
Australian Doctor 2013; Apr 12:25–32.
8. Ogilvie C, Gaffney D, Murray H, Kerry A, Haig C, Spooner R, et
al. Improved detection of hereditary haemochromatosis. J
Clin Pathol 2015;68(3):218–21.
9. Goot K, Hazeldine S, Bentley P, et al. Elevated serum
ferritin – what should GPs know? Aust Fam Physician
10. Ekanayake D, Roddick C, Powell LW. Recent advances
in hemochromatosis: a 2015 update : a summary of
proceedings of the 2014 conference held under the auspices
of Hemochromatosis Australia. Hepatol Int 2015;9(2):174–82.
11. Powell LW, Seckington RC, Deugnier Y. Haemochromatosis.
Lancet 2016. DOI: 10.1016/S0140-6736(15)01315-X
12. Borch-Iohnsen B, Hauge A. Should iron preparations be
available only by prescription?. Tidsskr Nor Laegeforen
13. Barbosa FB, Callegari A, Sarinho JC, Lucena J, Casagrande R, de
Souza BD. Hemochromatosis simulating rheumatoid arthritis:
a case report. Rev Bras Reumatol 2014;54(1):62–4 .
14. Genetic haemochromatosis. Therapeutic Guidelines Ltd (eTG
March 2016 edition).
15. Adams PC, Barton JC. How I treat hemochromatosis. Blood
16. Hutchinson C, Geissler CA, Powell JJ, Bomford A. Proton pump
inhibitors suppress absorption of dietary non-haem iron in
hereditary haemochromatosis. Gut 2007;56(9):1291–5.
17. Rombout-Sestrienkova E, Winkens B, Essers BA, Nieman
FH, Noord PA, Janssen MC, et al. Erythrocytapheresis
versus phlebotomy in the maintenance treatment of HFE
hemochromatosis patients: results from a randomized
crossover trial. Transfusion 2016;56(1):261–70.
18. Hicken BL, Tucker DC, Barton JC. Patient compliance with
phlebotomy therapy for iron overload associated with
hemochromatosis. Am J Gastroenterol 2003;98(9):2072–7 .
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