Sichelzellkrankheiten sind seit den 1950er-Jahren aus dem Mittelmeerraum, aus Afrika
und dem Mittleren Osten nach Deutschland gekommen und beschäftigten anfangs hauptsächlich
die Pädiater. Seit den 70er-Jahren ist die Lebenserwartung der Betroffenen deutlich
angestiegen, ca. 95 % werden heute erwachsen. Deshalb sollten Allgemeinmediziner und
Internisten die verschiedenen Formen der Sichelzellkrankheiten, v. a. die HbSC-Krankheit
(ca. 20 %), kennen.
Abstract
Sickle cell disease has come to Germany from the Mediterranean region, Africa and
the Middle East since the 1950 s and initially mainly concerned paediatricians. Since
the 1970 s, the life expectancy of those affected has risen significantly, and about
95 % now live to adulthood. Therefore, general practitioners and internists should
be familiar with the different forms of sickle cell disease, especially HbSC disease
(approx. 20 %).
A precise diagnosis of sickle cell disease (exact phenotype) is essential; the term
„sickle cell anaemia“ must be avoided. In patients of African origin with microcytosis,
slightly elevated reticulocytes and pain symptomatology, the possibility of HbSC disease
should be considered – even with age-appropriate haemoglobin values. Annual retinoscopy
is recommended for HbSC patients from the age of 7, and for all other sickle cell
patients from the age of 10. If a hearing loss occurs in an HbSC patient, phlebotomy
should be performed immediately. In all sickle cell patients with dizziness or pain
and an Hb > 10 g/dl, phlebotomy is indicated.
Schlüsselwörter
Sichelzellkrankheit - HbSC-Krankheit - proliferative Retinopathie - Hörsturz - Aderlass
Key words
sickle cell disease - HbSC-disease - proliferative retinopathy - hearing loss - phlebotomy
