CARDIOVASCULAR JOURNAL OF AFRICA • Vol 23, No 6, July 2012
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Letter to the Editor
Hermansky–Pudlak/Chediak–Higashi syndromes
The common denominator in both of these conditions is
albinism. Hermansky–Pudlak syndrome affects the platelets and
patients have a tendency to bleed.
1
Chediak–Higashi syndrome affects the leukocytes, results in
immune disorders and causes intracytoplasmic inclusions. These
latter patients are prone to malignant lymphomas as the immune
system is involved.
Hermansky–Pudlak symptoms occur due to defects in the
melanosomes and the disease affects the lysosomal organelles in
the cells, especially the platelet-dense granules. For this reason
these patients have a haemorrhagic tendency.
Patients with Chediak–Higashi syndrome usually die at an
early age. The disease also affects the lysosomal organelles.
2
Chediak–Higashi syndrome is an autosomal recessive
disorder, as is Hermansky–Pudlak syndrome.
3
Subtypes of
Hermansky–Pudlak disease exist. Chromosomes 3, 5 and 10 are
involved. Hermansky–Pudlak is seen predominantly in Puerto
Ricans but is also found in the Swiss Alps.
In Chediak–Higashi disease, eight known gene allele defects
are found, natural killer cells are deficient and the immune
system is involved, predisposing patients to lymphomas. In
both disorders hair, skin and eye colour are deficient, making
albinism the common factor.
3
It can therefore be concluded that both Hermansky–Pudlak
and Chediak–Higashi syndromes affect the platelets and white
cells, namely the haematological system.
Hilary Denis Solomons, MB BCh, MMed (Haem) Path (Wits)
Johnnesburg, South Africa
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based on a heritable subcellular structural defect – the Chediak–Higashi
syndrome.
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3.
Oetting WS,
et al.
The clinical spectrum of albinism in humans.
Mol
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