Hemoglobin C acquired via a blood transfusion. Detection by automated blood counter.

Hemoglobin C was acquired by a 52-year-old man via a blood transfusion. The presence of nonlysis of erythrocytes on the Technicon H6000 (Tarrytown, NY) scattergram after blood transfusions and the absence of this process in the admission peripheral blood count confirmed that this abnormal hemoglobin was acquired and not endogenous to the patient. Homozygous hemoglobin C disease was confirmed in one of the donors of blood received by the patient.

Automated detection of red cell antibodies in donor sera using an automated technology.

The evaluation of two automated (AutoGrouper-16C) antibody detection techniques, designed for routine use is reported. Three channels on the instrument were modified for low-ionic-strength or enzyme methods. The incidence of antibody detection was 90.5 percent, and all antibodies that were not detected had titers of 4 or less and would be unlikely to cause adverse clinical reactions if transfused. The adaptation of this automated instrument for donor antibody screening resulted in substantial time and cost savings.

Transfusion support for haemoglobinopathies.

The indications and management of blood transfusion in the haemoglobinopathies have been reviewed. The sickle cell diseases that require transfusion support are sickle cell anaemia, sickle haemoglobin-C and -D diseases and sickle beta-thalassaemia. Homozygous beta-thalassaemia (Cooley's anaemia) is the major problem among the thalassaemias. The pathophysiology of the sickle cell disorders is largely based on the secondary effects of increased blood viscosity, whereas in the thalassaemias the defect is ineffective haematopoiesis. In the former the major problems occur as manifestations of vaso-occlusive crises with disseminated bone and abdominal pain, priapism, stroke and leg ulcers. Bone infarction and aseptic necrosis occur but the widespread bone changes, underdevelopment and haemochromatosis that complicate the thalassaemia are not prominent. Transfusion therapy in the sickle cell diseases is mainly episodic and is guided by the frequency of crises and the severity of vaso-occlusive complications. Partial exchange transfusion and the maintenance of haemoglobin A concentrations at 40 to 50 per cent is frequently indicated. In the thalassaemias, maintenance of haemoglobin levels is essential for normal growth and development. The problem of haemochromatosis is very serious. With hypertransfusion regimens the haemoglobin and haemotocrit are maintained above 12-13 g/dl and 35 per cent. The resulting benefit appears to be reduced blood volume, less iron turnover, and less intestinal iron absorption. The splenomegaly in these disorders is frequently associated with hypersplenism requiring well-timed splenectomy. Chronic and intensive chelation is necessary to prevent the ravages of iron overload. The availability of automated equipment for in vivo and ex vivo blood cell separation has brought new possibilities for improving the management of these haemoglobinopathies. It is feasible, but not as yet practical, to offer transfusions of neocytes (red cells with a mean age of 30 days) which have a 50 per cent longer survival than routine red cell preparations (mean age of 60 days). Neocytes can be prepared ex vivo from fresh routine blood donations using blood cell separator devices. The result is reduced transfusion requirements. A more recent suggestion for using the new technology is to remove the patient's oldest and most abnormal corpuscles on the basis of buoyant density and replacing them with neocytes . Thus the short-lived abnormal red cells would be removed before they could unload their iron. With automation it is possible to perform these procedures on an outpatient basis.

The I, i, and HI blood group antigens in extracts of human erythrocytes.

Extracts of stromata of human adult and newborn (cord) erythrocytes were prepared with n-butanol. The aqueous phase and butanol phase extracts were examined for the presence of blood group I, i, HI and H substances in inhibition of agglutination experiments. The recovery of HI activity in both the aqueous and butanol phase extracts of adult red cells but only in the aqueous phase of cord cells is reported heare for the first time. The i specificity was present in the aqueous phase but not in the butanol phase of cord erythrocytes, also not previously reported. Although the recovery of I but not i substance in aqueous phase extracts of adult cells had been shown by other workers, examination of the butanol phase extracts for I and i substances had not been documented. In the present study, Ii substances were not demonstrable in any of the butanol phase extracts of adult and cord erythrocytes.

The A, M, N, and P1 blood group substances in butanol extracts of human erythrocytes.

Aqueous phase and butanol phase extracts of group A1, O, M, N, P1 and P2 human erythrocytes perpared by extraction of red cell stromata with n-butanol were examined for the presence of blood group substances in inhibition of agglutination experiments using antisera and test cells of human origin. Group A substance was recovered in both aqueous phase and butanol phase extracts, M and N substance in aqueous phase extracts only, and P1 substance only in butanol phase extracts. Use of high concentrations of extract resulted in detection of P1 activity in aqueous phase extracts and of N specific inhibition in the aqueous phase extracts of group M erythrocytes. Distilled water appeared to be superior to digitonin and hypotonic phosphate buffer solutions for preparation of stromata for the recovery of group A and M substances in the butanol extracts but not for group N and P1 substances.