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Post-transfusion alloimmunization in patients with sickle cell disease.

The transfusion histories over a 33-month period of 50 patients with sickle cell disease were reviewed to determine the frequency of alloimmunization to red cell antigens following transfusion in these patients. There were 30 females and 20 males, aged 19--49 years. Eighteen (36%) were immunized of which thirteen were females. Five of the patients have formed only one antibody so far, while the other 13 have formed two or more. Thirty-six antibodies were identified: 16 against various Rh antigens, 12 anti-Lewis, 5 anti-Kell and one each of anti-Jka, -Fya and -M. The immunized patients received, on the average, more transfusions although there was a considerable degree of overlap between the immunized and nonimmunized groups. An approach to the hemotherapy of patients with sickle cell disease (SCD) is discussed.

Adult

Genetic studies of the Macushi and Wapishana Indians. II. Data on 12 genetic polymorphisms of the red cell and serum proteins: gene flor between the tribes.

Blood samples from 509 Macushi (3 villages) and 623 Wapishana (11 villages) of Northern Brasil and Southern Guyana have been analyzed with respect to the phenotype and gene frequencies at the following 12 polymorphic loci: ABO, Kell-Cellano, MNSs, Rh, P, Duffy, Kidd, Diego, Lewis, Group-specific component, and the immunoglobulin allotypes of the Gm and Inv systems. The data suggest that 5-6% of the Wapishana gene pool is derived from non-Indians but only 1-2% of the Macushi. Inter- and intratribal genetic distances between villages are calculated for these data in an effort to understand gene flow between the tribes and to account for the unusual distribution of a newly-discovered genetic polymorphism of erythrocyte esterase A thus far limited to these 2 tribes (Neel et al., 1977). The data are puzzling and consistent with the possibility that both the Carib-speaking Macushi and the Arawak-speaking Wapishana have derived the esterase A allele in question from some third group now extinct or thus far undiscovered. Intertribal genetic distances based on gene frequencies at 6 loci are derived for 20 Amerindian tribes (including these 2); the "central" position of these 2 tribes can in part be explained by the active migration matrix connecting them.

ABO Blood-Group System

Blood group alloantibodies: an assessment of some laboratory practices.

Over a 20-year period, the number of blood donors and transfusion recipients previously immunized by blood group antigens was determined in a large blood center. During that period, the proportion of patients found to have anti-D declined, while those with other antibodies in the Rh system, as well as in the Kell, Duffy, and Kidd systems, increased sharply. As expected, the rate of sensitization was much lower among blood donors than among recipients. In both groups, sensitized females greatly outnumbered sensitized males. Some of the findings summarized here raise questions about current practices in transfusion laboratories and emphasize the responsibilities of blood bank professionals to donors as well as to patients.

Adolescent

[Problematic blood transfusion (author's transl)].

Two patients were admitted with signs of haemolysis due to severe transfusion reactions. Serological investigations showed the presence of the antibodies anti-IKb and anti-Fya. After a short period of haemodialysis the patient with Kidd antibodies died. A further patient with triple antibodies (anti-c, -K, -Lub) was so pre-immunised that with an antigen frequency of over 99.9% no compatible blood could be found in Europe. By means of information from the European Central Index in Amsterdam compatible blood for the operation could be obtained from Australia. In the fourth case the antibody anti-Cob was found which had not previously been described in Germany and with which the phenotype could be determined in 1405 unselected persons in the German population. The frequency of Cob is 7.2%.

Adult

Production of plasma with high anti-D concentration in Rh-negative volunteers.

63 Rh-negative male volunteers were immunized with D-positive red cells. 28 (44%) produced anti-D. To provide sustained levels of high concentration of anti-D, 18 of the 28 male volunteers have received repeated antigenic stimuli at individual intervals of 2-9 months. In all but 1 case the level of anti-D rose to between 30 and 300 microgram/ml and persisted at this level for several years. There was no evidence of a decrease in antibody response with an increase in the number of stimuli. 24 Rh-negative women immunized by pregnancy were restimulated. 16 achieved anti-D concentrations over 21 microgram/ml. The levels achieved were not influenced by the different D-positive cells injected, nor were larger antigenic doses of any benefit. No untoward clinical reactions occurred as a result of the repeated injections of antigenic material or of long-term plasmapheresis. 2 volunteers developed unwanted antibodies outside the Rh-system. Beginning in 1968, the programme to obtain anti-D plasma has yielded more than 70,000 doses of 250 microgram each, permitting the treatment of all Rh-negative women at risk.

Antibody Formation

Association of blood groups with essential and secondary hypertension. A possible association of the MNS system.

Persons participating in a 5-day diagnostic protocol were routinely typed for ABO, Rh, MNS, Kell, Kidd, Duffy, P, Haptoglobin, phosphoglucomutase-1 (PGM-1), and acid phosphatase (AcP). The study population was composed of 164 normotensive whites, 34 normotensive blacks, 161 whites and 43 blacks with essential hypertension, and 52 whites with secondary forms of hypertension (18 atherosclerotic renovascular hypertensives, 17 patients with fibromuscular disease, and 17 patients with primary aldosteronism). There were no significant differences in phenotype frequencies in ABO, Rh, Kidd, Kell, Duffy, P, Haptoglobin, PGM-1 or AcP in any of the comparisons. However, there was a significantly different distribution of MNS phenotypes in comparisons of essential and atherosclerotic renovascular hypertensives with normotensive controls. Essential hypertensives had a lower frequency of the S gene and a higher frequency of s in whites (X2 = 12.21, p less than 0.005). Atherosclerotic renovascular hypertensives differed from the normotensive population in the frequencies of both MN (X 2 = 4.34, p less than 0.05) and Ss (X2 = 4.21, p less than 0.05). The finding of disease-blood group associations supports the hypothesis that there may be significant physiological differences between individuals of different blood types.

ABO Blood-Group System

Sero-genetic studies on the San of South West Africa.

The San, a physically, culturally and linguistically distinctive people, have been shown by archaeological records anciently to have inhabited the whole of Eastern and Southern Africa. They, in common with the Khoi, the other members of the Khoisan race, are confined now to Southern Africa and principally to Botswana and South West Africa, though a number are also found in Angola. Sero-genetic data concerning seven South West African groups are presented in this study, and confirm a shared overall genetic profile characteristic of the San in general, slightly different from that of the Khoi and in significant contrast with that of the Negroes.

ABO Blood-Group System

Associations between atopic diseases and the polymorphic systems ABO, Kidd, Inv and red cell acid phosphatase.

In 239 German patients with atopic conditions (atopic dermatitis, hay fever, allergic rhinitis, bronchial asthma, and acute urticaria) the phenotype and gene distribution of 15 genetic blood polymorphisms (ABO, MNSs, rhesus, P, Kell, Duffy, Kidd, Hp, Gc, Gm, Inv, aP, PGM1, EsD, and 6-PGD) were analyzed and compared with those in 151 selected controls (individuals clinically free of allergic conditions and without allergy in the family history). The incidence of blood group antigens A and B was somewhat higher in patients than in controls. These observations are in accordance with the results of previous studies in other populations. In addition, our observations favor the hypothesis that there are also associations between the phenotypes Jk (a-b+), Inv(1) and red cell acid phosphatase aP A and aP AP on the one hand and atopic disposition on the other. The possible reasons for these associations are discussed.

ABO Blood-Group System

Further evidence of heterogeneity within the Kidd blood group system.

Serological studies on blood of selected individuals show that red blood cells from individuals genetically JkaJk and JkbJk give single dose agglutination reactions with anti-Jka and anti-Jkb respectively, but react as strongly with anti-JkaJkb as do cells from random individuals. A Jk(a+b-) Oriental person has been found whose red blood cells give a single dose reaction with anti-Jka, but react more weakly than do cells from random people in tests with anti-JkaJkb. The results support a conclusion that the antigen recognized by anti-JkaJkb is a distinct and separate antigen of the Kidd blood group system.

Alleles

[Hemolytic transfusion accident due to the blood group antibody anti-Jka].

A haemolytic transfusion incident caused by anti-Jka is reported about. In a 56 year old patient suffering from chronic iron deficiency anaemia an immunization in the Kidd system has taken place within 9 days. In the course of this immunization the fourth incompatible blood transfusion caused a severe transfusion incident. The necessity of serological compatibility testing by the indirect antiglobulin or enzyme test and the significance of making a strict indication for blood transfusions are referred to.

Anemia, Hypochromic

[Determination of biological individuality by blood group studies].

Author deals with the possibility of determination of the biological individuality by serological methods. Determination of HL-A tissue-compatibility antigens and system of MN/Ss and Rh seen to be the most suitable tools. Alloantigens, serum proteins and group of enzymes characterize the biological individual i. e. serological methods can be used as a tool of the identification of a person. Author deals with the possiblity of determination of various razes. For this purpose investigation of the following systems are necessary: ABO-, Rh-, MN/Ss-, Kell-, Duffy-, Lutheran, Kidd, Lewis and Diego. Nevertheless, this problem is not yet solved.

ABO Blood-Group System

On the incidence of blood group O and Gm(-1) phenotypes in patients with malignant melanoma.

Fifteen polymorphic systems of the blood (ABO, MNSs, Rhesus, P, Kell, Duffy, Kidd, Hp, Gc, Gm, Inv, aP, PGM1, EsD, and 6-PGD) were examined in 191 unrelated male and female patients suffering from malignant melanoma. These polymorphic systems were compared with the corresponding phenotype and gene frequencies of controls from the same geographical area (Rhineland-Palatinate). The only associations discovered were the ABO and Gm polymorphisms: The incidence of O and Gm(-1) phenotypes in patients is obviously higher than in controls. These observations agree with the findings in other population samples from Germany and Bulgaria.

ABO Blood-Group System

[Genetic analysis of a population with abnormalities of gene frequencies].

Nineteen out of the 53 blood donors of french village with 241 inhabitants (Cezay Loire) are Rh negative (D--). This discrepancy in the distribution is analysed. 1.--The study of the genetic erythrocyte markers (ABO and Rh system for 158 inhabitants, Kell, Rautenberg, Duffy, Kidd, MNSs, P1 Lutheran, PGM1, PGM2, 6 PGD, AK, ADA, Acid phosphatase systems for 104 inhabitants) show significant abnormal gene frequencies (No. 10%) compared with a control population from Saint-Etienne, for A1, Ms, r, P1 alleles; conversely rare alleles do not seem to exist. HLA system was not tested. 2.--The genetic study led to: a) a demographic study which implied 7840 registrar's certificates and the building up of 1364 families to which the 5096 subjects belonged identified and having lived in Cezay since 1607 (this date corresponds to the earliest registrar's certificate). b) it also led to the analysis of the origin and evolution of the genetic inheritance throughout the 13 generations of known inhabitants. The calculation of the chances of each generation having passed on its genetic material to following generations shows that: Cezay has an integrated population; 30% of the genes are renewed for each generation the average value of each founder can vary according to the various generations but there seems to exist a "founder effect" of the Rh--(D--) having been and lived in the village before 1860. Although they represent 68% of the total population, the tested samples can be contested for certain systems, in its constitution (formation, choice) which prevents from ascertaining the foundation effect observed. The authors underligne the contribution of immunogenetics to the genetics of populations, and show the incidence of the choice of samples in the method used.

Alleles