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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
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[Diamond-Blackfan anemia with extremely low hemoglobin]

U M Andersen1

  • 1Randers Centralsygehus, paediatrisk afdeling.

Ugeskrift for Laeger
|July 12, 1993
PubMed

Diamond-Blackfan anaemia (DBA) has been described in approximately 300 cases world wide. It is characterised by a normochromic-macrocytic anaemia in early childhood, reticulocytopenia and a normocellular marrow with a selective deficiency of red cell precursors. Foetal haemoglobin is usually increased, and DBA is associated with increased expression of "i" antigen. DBA is often associated with multiple congenital anomalies, which are described. Transient erythroblastopenia is an important differential diagnosis and the distinctive characteristics are discussed. A case of DBA in an 8-week-old boy presenting with extremely low haemoglobin (0.9 mmol/l) is presented. The literature is reviewed.

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