Presence of bone pain and anemia in elderly patients should alert the clinician to investigate along the lines of MM. Majority of patients have osteolytic lesions on X-ray and pathological fractures, and benefit from melphalan based combinations in situations where facilities for transplant are not available.

Background and study objectivesBlood transfusion is central in the prevention and treatment of certain chronic complications of sickle cell disease. It is indispensible in correcting anaemias as well as in the practice of exchange blood transfusion. These gains are largely limited by formation of allo-antibodies. Several studies demonstrated varying frequencies of allo-immunization in various patient groups. The effect of the racial differences between the donor and recipient pool, which has been subsumed in this study, has continuously created a confounding effect on the results of previous studies.AimThis study was aimed at determining the pattern and frequency of allo-immunization in multiply transfused sickle cell patients, in a racially matched donor and recipient population.Patients and methodsThis was a cross-sectional case-controlled study involving 80 Nigerian sickle cell disease patients who had received three or more units of packed red cells in the within 4 weeks of the study and 40 controls (who were SCD that had not been transfused in their life time). Antibody screening and identification was done using the Diamed microtyping system.ResultsFrequency of allo-immunization was determined to be 18.7 % (15/80) among the previously transfused and 5 % (15/120) in all sickle cell disease patients. Auto-antibodies were detected in 1.25 % of the study group and 2.5 % of the control, and all reacted with the Kell and Lutheran blood group antigens. The pattern of allo-antibodies found showed; 46.7 % Rhesus, 40 % Kell, while Lutheran and Duffy 13.3 %, each.ConclusionSickle cell disease patients are particularly susceptible to development of allo-antibodies despite racial similarities between the donor and recipient population. The most common allo-antibodies are Rhesus, Kell and Lutheran and Duffy respectively in order of decreasing frequency. Development of auto-antibodies seems to be independent of blood transfusion in sickle cell disease with possibly different pathogenetic mechanism. Policy on extended red cell phenotyping for common antigens will reduce allo-immunization among multiply transfused patients.

Relatively lower arterial blood pressure is a significant finding in patients with sickle cell anemia. Hematocrit, frequency of crisis, body mass index, and body surface area are significant determinants of blood pressure indices in sickle cell anemia.

Leukocyte adhesion to vascular endothelium contributes to vaso-occlusion and widespread organ damage in sickle cell disease (SCD). Previously, we found high expression of the adhesion molecules alphaMbeta2 integrin and L-selectin in HbSS individuals with severe disease. Since membrane n-6 and n-3 polyunsaturated fatty acids modulate cell adhesion, inflammation, aggregation and vascular tone, we investigated the fatty acid composition of mononuclear cells (MNC) and platelets of HbSS patients in steady state (n=28) and racially matched, healthy HbAA controls with similar age and sex distribution living in the same environment (n=13). MNC phospholipids of the patients had lower levels of docosahexaenoic acid (DHA, p<0.01) and increased arachidonic acid (AA, p<0.005) relative to HbAA controls. Similarly, platelets from HbSS patients had less eicosapentaenoic acid (EPA, p<0.05) and more AA (p<0.05) in choline phosphoglycerides (CPG), with reduced DHA (p<0.05) in ethanolamine phosphoglycerides. Platelet CPG had lower DHA levels in SCD patients with complications compared to those without (p<0.05). Reduced cell content of EPA and DHA relative to AA favours the production of aggregatory and proinflammatory eicosanoids that activate leukocytes and platelets. This facilitates inflammation, leukocyte adhesion, platelet aggregation and vaso-occlusion in SCD.

There is a need for adequate counseling of pregnant women on the importance of Rh D negative factor during the antenatal period in order to prevent hemolytic disease of the newborn.