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1.
Transfusion ; 49(11): 2406-11, 2009 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-19624489

RESUMO

BACKGROUND: ceAR (RHCE ceAR) is a rare RH allele encountered in people of African/Caribbean ancestry, known to encode a partial e antigen. The homozygous ceAR/ceAR genotype encodes the rare blood group Hr-. This study describes alloanti-c/ce in a ceAR/Ce patient, suggesting that ceAR also encodes a partial c antigen. CASE REPORT: A 21-year-old patient suffering from intermediate beta-thalassemia, with transfusion history, was hospitalized for severe anemia. Blood samples were referred to the National Reference Laboratory for suspicion of a mixture of alloantibodies or an alloantibody to a high-prevalence antigen. MATERIALS AND METHODS: Standard hemagglutination methods were performed to investigate the patient's RBCs and serum. A molecular analysis of RHD and RHCE was carried out by allele-specific polymerase chain reaction and DNA sequencing. RESULTS: Blood type performed by the referring laboratory was B, D+C+E-c+e+, K-. Several antibodies were identified: anti-c/ce, anti-Fy(b), anti-Jk(a), and anti-S. Full serologic investigations showed that anti-c/ce could be very likely considered as an alloantibody. The patient's genotype was ceAR/Ce. Anti-c/ce reacted with ceAR/ceEK, ceEK/ceEK, and ceAR/ceBI but not with ceAR/ceAR, ceMO/ceMO, and ce(s)(340)/ce(s)(340) RBCs. CONCLUSION: This is the first case of alloanti-c/ce related to ceAR, suggesting that this rare RHCE allele encodes a partial c antigen. The presence of the C antigen in the patient allowed for the partial expression of the c antigen encoded by ceAR. The c antigen encoded by ceAR appeared to be different than that encoded by ceEK and ceBI and may share common lacking epitopes with the c antigens encoded by ceMO and ce(s)(340).


Assuntos
Isoanticorpos/imunologia , Sistema do Grupo Sanguíneo Rh-Hr/genética , Sistema do Grupo Sanguíneo Rh-Hr/imunologia , Alelos , Testes de Hemaglutinação , Humanos , Masculino , Reação em Cadeia da Polimerase , Adulto Jovem
2.
Transfusion ; 49(9): 1785-92, 2009 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-19413729

RESUMO

BACKGROUND: Delayed hemolytic transfusion reaction (DHTR) is a life-threatening complication in sickle cell disease (SCD) characterized by recurrence of disease complications, recipient red blood cell (RBC) destruction, and frequently no detectable antibody. Phosphatidylserine (PS) exposure signs suicidal RBC death or eryptosis and is involved in vasoocclusive crisis (VOC). STUDY DESIGN AND METHODS: Transfusion was monitored in 48 SCD patients for up to 20 days. PS exposure was evaluated in vivo on patient RBCs (PS-RBCs) at five time points and in vitro after incubation of donor RBCs with pretransfusion plasma. RESULTS: Three VOC patients displayed DHTR with recurrent SCD features and no detectable antibody in two cases. In vitro, PS-RBC percentage was significantly increased by incubating donor RBCs with pretransfusion plasma samples from DHTR patients with no detectable antibody. No such increase was observed with samples from other patients. This result indicates that donor RBCs may be damaged by the environment of SCD patients, increasing the physiologic clearance of apoptotic RBCs. In vivo, PS-RBC percentage increased in all three cases after destruction of transfused RBCs, indicating that DHTR induces PS-RBCs and, possibly, subsequent VOC and autologous RBC destruction. CONCLUSION: This study clearly demonstrates that DHTR can occur in the absence of detectable antibody. In these cases, a mechanism of excessive eryptosis is proposed.


Assuntos
Anemia Falciforme/terapia , Eritrócitos/citologia , Hemólise/imunologia , Reação Transfusional , Adulto , Anemia Falciforme/sangue , Anemia Falciforme/imunologia , Morte Celular , Eritrócitos/imunologia , Feminino , Citometria de Fluxo , Humanos , Masculino , Fosfatidilserinas/metabolismo , Síndrome , Adulto Jovem
3.
Transfusion ; 49(7): 1329-34, 2009 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19320865

RESUMO

BACKGROUND: In the Rh blood group system, published observations showed that the c antigen has the fewest variant forms of the principal antigens in this system. The partial nature of the c antigen was only reported in c+ Rh:-26 persons and to be associated with the ce(s)(340) allele. This study reports the first case of alloanti-c related to a (C)ce(s) haplotype. STUDY DESIGN AND METHODS: Serologic and genetic studies were performed on blood samples of a multitransfused 40-year-old African patient with sickle cell disease displaying a DCcee phenotype. RESULTS: Red blood cells (RBCs) of the patient displayed normal expression of C, c, e, ce antigens either with routine reagents or with monoclonal antibodies. Analyses of DNA and Rh transcripts showed that the patient carried a (C)ce(s)/DCe genotype. The patient's serum contained anti-D, anti-c, anti-E, anti-e, anti-V, anti-Js(a), and anti-S. Anti-c was isolated from the mixture of antibodies by using absorption and adsorption-elution techniques. Anti-c provided consistent reactions with c+ RBCs. Reactions were stronger with c+ ce+ RBCs than with c+ ce- RBCs. No agglutination of RBCs from individuals carrying a homozygous (C)ce(s) genotype was observed. CONCLUSION: These data provide the evidence that anti-c in our patient was an alloanti-c and, consequently, that (C)ce(s) haplotype encodes a partial c antigen. The clinical significance of anti-c related to this haplotype should be evaluated in the future.


Assuntos
Haplótipos/genética , Sistema do Grupo Sanguíneo Rh-Hr/genética , Adulto , Éxons/genética , Feminino , Humanos , Reação em Cadeia da Polimerase , Testes Sorológicos
4.
Transfusion ; 47(9): 1616-20, 2007 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-17725725

RESUMO

BACKGROUND: Weak D Types 1, 2, and 3 recipients cannot be immunized when exposed to D antigen. Molecular biology is very efficient to type weak D variants but rarely implemented in daily practice. The serologic typing practice of weak D in a Caucasian patient population was analyzed and a transfusion strategy is proposed. STUDY DESIGN AND METHODS: Samples typed either ddCcee or ddccEe in routine laboratories were tested with the indirect antiglobulin test (D(u) test). D(u)-positive samples were screened for weak D alleles Types 1, 2, and 3 and further tested with immunoglobulin M (IgM) anti-D reagents, used in a fully automated device. RESULTS: A total of 468 of 55,162 samples were found to be ddCcee or ddccEe. Ninety-three expressed weak D after the D(u) test leading to D+ assignment for transfusion. Seventy-three percent of D(u)-positive samples were weak D alleles Type 1, 2, or 3. Almost all weak D Types 1, 2, and 3 were positive with IgM reagents in gel matrix with an automated device. Other variants that could be potentially associated with anti-D alloimmunization, however, were also positive. CONCLUSION: Serology is very sensitive to detect weak D Types 1, 2, and 3, but there is no cutoff to distinguish variants of clinical significance. When molecular analysis is not available, it is proposed that a D+ status for blood recipients found to be weak D with a sensitive method be assigned, except for women of childbearing age or younger, because of the remaining possibility to be partial D or other rare weak D who can be immunized.


Assuntos
Tipagem e Reações Cruzadas Sanguíneas/métodos , Tipagem e Reações Cruzadas Sanguíneas/normas , Transfusão de Sangue/métodos , Transfusão de Sangue/normas , Sistema do Grupo Sanguíneo Rh-Hr/análise , Alelos , Automação , Mapeamento de Epitopos , Humanos , Imunoglobulina M/imunologia , Indicadores e Reagentes , Fenótipo , Sistema do Grupo Sanguíneo Rh-Hr/genética , Sistema do Grupo Sanguíneo Rh-Hr/imunologia , Sensibilidade e Especificidade , Testes Sorológicos
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