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Dtsch. Med. Wochenschr. · Sep 2022
[The broad spectrum of sickle cell disease: HbSC in everyday clinical practice].
- Roswitha Dickerhoff.
- Dtsch. Med. Wochenschr. 2022 Sep 1; 147 (19): 1259-1266.
AbstractSickle 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.Thieme. All rights reserved.
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