ABSTRACT
25 consecutive episodes of acute chest syndrome in 13 adult patients with sickle-cell disease were studied retrospectively. Chest symptoms were present on admission in 23 of 25 episodes. Abnormal chest signs and an abnormal chest X-ray were present on admission in only 11 and 9 episodes, respectively, but developed later in the remainder. The 9 episodes with bilateral radiological changes were associated with higher pulse rates, longer-lasting fever, more profound arterial hypoxaemia, and greater falls in haemoglobin than the 16 unilateral episodes. 2 patients with bilateral disease died; both had platelet counts less than 100 X 10(9)/litre. In 12 episodes (6 bilateral, 6 unilateral) exchange transfusion was required and produced striking improvement in 11. Despite intensive microbiological investigation, infection was found in only 2 episodes--1 mycoplasma and 1 evidence of Escherichia coli. Pulmonary intravascular sickling may account for much of the clinical picture.
Subject(s)
Anemia, Sickle Cell/complications , Pain/complications , Thorax , Acute Disease , Adolescent , Adult , Anemia, Sickle Cell/physiopathology , Anemia, Sickle Cell/therapy , Exchange Transfusion, Whole Blood , Female , Hemoglobin, Sickle/analysis , Humans , Leukocytosis/physiopathology , Leukocytosis/therapy , Male , Middle Aged , Pain/physiopathology , Pulse , Radiography, Thoracic , Retrospective Studies , SyndromeABSTRACT
The iron status of 31 patients with sickle cell anaemia (Hb SS) and balanced globin chain synthesis was studied. Twelve patients (group I) had never been transfused; 14 had received up to 4 units of blood in the past (group II) and five had been hypertransfused for 6 months to 2 years (group III). The hypertransfused group had significantly higher MCV and MCH than the nontransfused one, and significantly lower total iron binding capacity and higher serum ferritin concentration than either groups I or II. The serum ferritin concentration was lower than normal in eight patients (five in group I and three in group II), and higher than normal in seven patients (four in group III and three in group II). The remaining 16 patients had normal serum ferritin concentrations. Our results indicate that iron overload is uncommon in adults with Hb SS who have not been transfused, and that a proportion of patients have lower than normal serum ferritin concentrations. Transfused patients, even 6 months after the last transfusion, show increased haemoglobin content of red cells, which may have an adverse effect on the frequency and severity of sickle crises.
