Parameters of host defence were investigated in 30 patients with sickle cell disease (SCD). A newly devised perfusion system was used to study the kinetics in whole blood of leucocyte adherence, phagocytosis, killing and solubilization of a mixture of Staph. aureus and Str. pneumoniae, and secretion of lactoferrin. A skin window technique was used to examine the accumulation of leucocytes at inflammatory foci and their subsequent rate of movement through a filter. Serum concentrations of C3, C4, total haemolytic complement and immunoglobulins were also measured. The rate of neutrophil migration into filters was slightly reduced in patients with SCD. The proportion of monocytes that emigrated from the skin windows and their rate of migration were markedly diminished. The adhesion of neutrophils and their ability to kill staphylococci were also reduced, particularly in patients of the haemoglobin (Hb) SS and Hb S-beta-thalassaemia genotypes. Neutrophil function was mostly impaired in patients with the greatest frequency of bacterial infection. The rate of clearance of pneumococci was related to the concentration of type specific immunoglobulin G but not M. Serum concentrations of immunoglobulins and complement were normal. We were unable to define a defect of host defence of sufficient magnitude to explain the susceptibility of these patients to severe infection.

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Patients and methodsTwenty-four patients with active SLE who fulfilled four or more of the criteria of the revised American Rheumatism Association criteria for classification of the disease" were studied. All were female aged between 22 and 59 years (mean 26). Disease activity was graded as severely active, moderately active, or inactive at the time of study according to a described classification. 12 The effect of MPPT (1 g of sodium succinate salt in 500 ml of saline intravenously over four hours on each of three successive days) on a wide range of phagocyte functions was tested on 15 patients on maintenance therapy with relatively small doses of prednisolone (5-15 mg/day). Six were studied with the perfusion chamber, skin windows were performed on four, and both techniques were used in the remaining five. Control studies on the perfusion chamber and skin window technique were performed on 18 healthy subjects matched for age (mean ± SD, 24 ± 5 years) and sex.The kinetics of lactoferrin secretion were determined in the perfusion chamber in four patients on maintenance doses of prednisolone of 20-40 mg-day before and after MPPT, in five patients with active 541 on 12 May 2018 by guest. Protected by copyright.

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