Investigations of host defence in patients with sickle cell disease

Abstract

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.

Serum immunoglobulin (G, A and M) classes and IgG subclasses in sickle cell anaemia

“…Respiratory tract infections occurred in some of the HbSS subjects in this study and may have contributed to the elevation of serum IgA. In contrast to some reports of normal or decreased IgM levels in SCA (3,5,19,22), subjects with moderate and severe disease in this study had significantly high values of this immunoglobulin class. Other workers have found raised IgM levels only in a small proportion of HbSS individuals (9,28).…”

Genetic Syndromes with Evidence of Immune Deficiency

“…231 IgM levels are often normal, or may be decreased. 231,232 Many patients with sickle cell disease do not generate an adequate antibody response following immunization for S. pneumoniae or H. influenzae type b. 233 Immunoglobulin synthesis in response to both mitogen and antigen may be decreased.…”

“…240À243 However, other investigators have not found defects in complement activation. 232,244 Management Penicillin prophylaxis should be initiated by 2 months of age, or at the time of diagnosis if over 2 months, for patients with HbSS and sickle-β-thalassemia disease. 245 A routine immunization schedule, including Haemophilus influenzae type B and hepatitis B immunization, should be followed.…”

Sickle Cell Disease in the Sudan: Clinical and Biochemical Aspects

“…Other investigators, including ourselves, have not identified any defect in the complement activation (77,120,140,141). Complement proteins C3, C4 and factor B as well as the immunoglobulins IgG, IgA and IgM, have been reported to be normal and even high in patients with sickle cell disease (27,49,140,142,143).…”

“…Complement proteins C3, C4 and factor B as well as the immunoglobulins IgG, IgA and IgM, have been reported to be normal and even high in patients with sickle cell disease (27,49,140,142,143).…”