Enfermedad hemolítica del feto y del recién nacido por aloanticuerpos contra el antígeno M / Hemolytic disease in fetuses and newborns due to antibodies against the M-antigen

Resumen | Hay pocos reportes de enfermedad hemolítica del feto y del recién nacido causada por aloanticuerpos contra el sistema de antígenos MNS, especialmente, porque los anticuerpos que se generan contra estos antígenos son del tipo IgM, los cuales tienen reactividad a temperaturas inferiores a los 37 °C, y, por lo tanto, no son de importancia clínica. A pesar de ello, se han reportado casos con presencia de anticuerpos anti-M de tipo IgG causantes de la enfermedad hemolítica del recién nacido e, incluso, casos de muerte intrauterina por incompatibilidad materno-fetal en el sistema MNS. El proceso hemolítico se asemeja al causado por los anticuerpos anti-Kell, con anemia progresiva por supresión hematopoyética que induce la destrucción de precursores hematopoyéticos en la médula ósea y ausencia de reticulocitos en la periferia. Se reporta el caso de una mujer con 38,5 semanas de gestación, que presentó discrepancia en la hemoclasificación directa y en la inversa. Como resultado, el recién nacido fue positivo en la prueba de Coombs directa sin que existiera incompatibilidad ABO con la madre. La correlación de estos resultados llevó a la detección de un anticuerpo anti-M en el suero materno. El diagnóstico definitivo fue posible gracias a la discrepancia en la hemoclasificación de la sangre materna. A pesar de que los anticuerpos anti-M usualmente no desempeñan un papel importante en la enfermedad hemolítica perinatal, este caso resalta la importancia de determinar la presencia de diferentes anticuerpos que pueden ser de vital interés a la hora de prevenir resultados graves asociados con dicha condición. Además, abre la puerta a nuevas recomendaciones relacionadas con la tamización y el tratamiento temprano de la hemólisis en los recién nacidos.

Abstract | There are few case reports of hemolytic disease in fetuses and newborns (HDFN) caused by alloantibodies against the MNS blood group system. The reason for this dearth is that antibodies toward these antigens are usually IgM, which not only cannot cross the placental circulation but also react at temperatures below 37°C. They are, therefore, of minimal clinical importance. Nevertheless, cases have been reported in which the presence of anti-M IgG antibodies caused severe HDFN and even intrauterine death in the presence of maternal-fetal MNS incompatibility indicating that they could have a high clinical impact. The hemolytic pattern observed in these cases is similar to that caused by anti-Kell antibodies. Progressive anemia is mediated and developed through hematopoietic suppression inducing the destruction of bone marrow precursor cells with the resulting absence of reticulocytes in peripheral blood. This occurred in the case of a woman at 38.5 weeks of gestation who showed a discrepancy between direct and reverse blood type determination. A direct Coombs test was performed on the newborn's blood, which was positive in the absence of maternal-fetal ABO incompatibility. Further tests were performed and anti-M antibodies were found in the maternal serum screening. Our final diagnosis was largely due to discrepancy issues in maternal blood. Although anti-M antibodies do not usually play a significant role in HDFN, this case stresses the importance of identifying the presence of antibodies that can be crucial in preventing HDFN and lead to new recommendations for the screening and prompt treatment of hemolysis in newborns.

Enfermedad por aglutininas frías: proceso diagnóstico, tratamiento y seguimiento de un paciente / Cold agglutinin disease: diagnosis, treatment and follow-up of a patient

La enfermedad por crioaglutininas es una anemia hemolítica autoinmune que se caracteriza, en la gran mayoría de los casos, por la hemólisis mediada por autoanticuerpos de tipo IgM y complemento C3d, contra los antígenos de la membrana del eritrocito, que conduce a hemólisis extravascular con propensión a la trombosis, y que afecta principalmente al sexo femenino y personas mayores. Su diagnóstico se realiza con la prueba de Coombs directo y fraccionado, y la titulación de aglutininas frías >1:64 a 4 °C. Se describe el caso clínico de una mujer de 89 años con un síndrome constitucional y una anemia de 3 años de evolución, en quien se determinó el diagnóstico de enfermedad por aglutininas frías. Asimismo, se describe el abordaje diagnóstico, el tratamiento instaurado, y se hace una breve revisión de la literatura publicada

Cold agglutinin disease (CAD) is an autoimmune hemolytic anemia characterized in the vast majority of cases by hemolysis mediated by IgM autoantibodies and complement C3d against erythrocyte membrane antigens, leading to extravascular hemolysis with propensity to thrombosis, affecting mainly females and older individuals. It is diagnosed by direct and fractionated Coombs test and a cold agglutinin titer >1:64 at 4 °C. We describe the case of an 89-year-old woman with a constitutional syndrome and a 3-year history of anemia, who was diagnosed with cold agglutinin disease. Also, we include the diagnostic and treatment approach, and a brief review of the literature

Investigation and Analysis of Non-ABO Hemolytic Disease of the Newborn / 中国实验血液学杂志

OBJECTIVE@#To study the serological detection characteristics and antibody specific distribution of hemolytic disease of the newborn (HDN) caused by irregular antibodies through retrospective case analysis.@*METHODS@#A total of 3 047 suspected cases of HDN were submitted by the Neonatal Department of our hospital from January 2014 to December 2019. Non ABO-HDN cases confirmed in our laboratory were taken as the research objects, while some cases of ABO-HDN were randomly selected as control. Disease-causing antibody specificity, serological detection characteristics, total bilirubin change trend and gender ratio of non ABO-HDN patients were explored.@*RESULTS@#Sixty-seven cases of non ABO-HDN were confirmed from the suspected cases of HDN, Among which 45 males and 22 females were detected with the positive rate 1.48% and 0.72%, respectively. The mothers of 65 cases had two or more pregnancies. The detected irregular antibodies were mainly involved with Rh system, MNS system, Kidd system and Lewis system, among which Rh system accounted for 88.07% of the total antibody detection rate. Compared with that of ABO-HDN patients, the total bilirubin of non ABO-HDN patients developed more rapidly with a higher peak and a longer duration (P<0.001). In terms of serological detection, the positive rate of non ABO-HDN direct antibody test was 97.01%, which was higher than 47.00% of ABO-HDN (P<0.001), and the agglutination strength was often ≥ 2+, but there were still weak positive or negative cases of direct antibody test.@*CONCLUSION@#Non ABO-HDN caused by irregular antibodies mostly occurs in fetuses whose mothers experience multiple pregnancies, and the number of males is more than females. The irregular antibodies detected are mainly attributed to Rh system. The peak value of bilirubin in non ABO-HDN patients is higher and lasts longer than that in ABO-HDN patients. Direct antiglobulin test may be used to roughly distinguish ABO-HDN from non ABO-HDN.

Hemolytic anemia as the initial presentation of Wilson's disease. Regarding a rare disease with an infrequent presentation / Anemia hemolítica como presentación inicial de la enfermedad de Wilson. A propósito de una enfermedad rara con una presentación infrecuente

Abstract Wilson's disease is a rare genetic disorder that affects the excretion capacity of copper. Its distribution is worldwide, with an estimated prevalence in 30 cases per million habitants. Although the most frequent symptoms are those of hepatic and neuropsychiatric origin, hemolytic anemia with negative Coombs may be the only manifestation of the disease and its presentation usually precedes for months to clinically evident liver disease or neurological manifestations. The case of a young patient with negative Coombs hemolytic anemia and an alkaline phosphatase / total bilirubin ratio <4 and AST / ALT> 2.2 is presented, establishing Wilson's disease as a diagnosis. (Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1459).

Resumen La enfermedad de Wilson es un raro trastorno genético que afecta la capacidad de excreción del cobre. Su distribución es mundial, con una prevalencia estimada en 30 casos por millón de habitantes. Aunque los síntomas más frecuentes son los de origen hepático y neuropsiquiatricos, la anemia hemolítica con Coombs negativo puede ser la única manifestación de la enfermedad y su presentación suele preceder por meses a la enfermedad hepática clínicamente evidente o las manifestaciones neurológicas. Se presenta el caso de una paciente joven con anemia hemolítica Coombs negativo y relación fosfatasa alcalina/bilirrubina total <4 y AST/ALT >2,2, en quien terminó por establecerse como diagnóstico una enfermedad de Wilson.(Acta Med Colomb 2020; 45. DOI:https://doi.org/10.36104/amc.2020.1459).

Transfusion-related acute lung injury in a parturient diagnosed with myelodysplastic syndrome: A case report

Transfusion-related acute lung injury (TRALI) is defined as a new episode of acute lung injury that occurs during or within 6 hours of a completed transfusion, which is one of the leading causes of transfusion-related morbidity and mortality. We present a case of TRALI in a 29-year-old parturient with myelodysplastic syndrome scheduled for cesarean section. The parturient developed hypoxemia and dyspnea after preoperative transfusion of platelets following apheresis to eliminate a unit of leucocyte in order to correct thrombocytopenia. She underwent emergent caesarean section for fetal distress. After surgery, the chest radiograph showed diffuse haziness of both lung fields. Direct and indirect antiglobulin tests were negative, and hemolytic transfusion reaction was ruled out. Pro-BNP 347.3 pg/ml also excluded transfusion-associated circulatory overload. The parturient completely recovered after oxygen support for 2 days. It is important to recognize TRALI as soon as possible to minimize perioperative morbidity and mortality.

Avaliação da validação da pesquisa de anticorpos irregulares no Hemocentro Regional de Montes Claros / Validation assessment of irregular antibodies investigation at the Regional Blood Center in Montes Claros City / Evaluación de la validación de la búsqueda de anticuerpos irregulares em la Región Hemocentro de Montes Claros

Objetivo: Analizar lavalidación de la detección de anticuerpos irregulares (PAI) mediante el uso del reactivo de control de Coombs en muestras de sangre tomadas de Febrero 2015 a Agosto 2016. Métodos: Estudio de naturaleza observacional, retrospectivo y prospectivo, con los procedimientos técnicos de carácter documental, que se sucederá em Laboratorio de Inmunohematología del Hemocentro Regional de Montes Claros - MG. Resultados: Se observó durante la encuesta que después de la no validación de algunos testes y sucedida la repetición del mismos individuos, no se ha encontrado la validación, por lo tanto requeiendo otra repetición hasta que la validación de la muestra. Esto plantea la posibilidad de interferencia que no sea el conocido y discutido, ya que la repetición se realiza aisladamente el análisis crítico de todos los pasos del proceso. Conclusión: El bajo porcentaje de resultados no validados ratifica la prueba de validación antiglobulínico es un buen método para confirmar el resultado de la búsqueda de anticuerpos irregulares

Objetivo: Analisar a validação da pesquisa de anticorpos irregulares (PAI) através da utilização do reagente Controle de Coombs em amostras sanguíneas coletadas de Fevereiro de 2015 à Agosto de 2016. Métodos: Estudo de natureza observacional, retrospectiva e prospectiva, apresentando procedimentos técnicos de caráter documental, a ser realizado no Laboratório de Imunohematologia do Hemocentro Regional de Montes Claros - MG. Resultados: Foi observado durante a pesquisa que após a não validação de alguns testes e realizada a repetição dos mesmos isoladamente, não foi constatado a validação sendo necessário outra repetição até que essa amostra validasse. Esse fato levanta a possibilidade de outras interferências além das conhecidas e discutidas, uma vez que a repetição foi realizada isoladamente analisando criticamente todas as etapas do processo. Conclusão: O baixo percentual de resultados não validados ratifica que o teste de validação antiglobulínico é um bom método para confirmar o resultado da pesquisa de anticorpos irregulares

Objective: The study's purpose has been to assess the validation of irregular antibodies investigation using the Coombs control reagent in blood samples collected over the period from February 2015 to August 2016. Methods: It is a observational, retrospective and prospective study, which presents technical procedures bearing a documentary character, and that was performed at the Laboratory of Immunohematology from the Regional Blood Center in Montes Claros-MG. Results: During the research, it was observed that after the non-validation of some tests and its repetition was then performed alone; the validation was not verified and once again a repetition was necessary until this sample was defined as validated. This fact raises the possibility of other interferences beyond those both known and discussed; bearing in mind that the repetition was carried out in isolation and also all stages of the process were performed under scrutiny. Conclusion: The low percentage of non-validated results ratifies that the antiglobulin validation test is a good method to confirm the result of the search for irregular antibodies

Cost-effectiveness analysis of diagnostic-therapeutic strategies for visceral leishmaniasis in Brazil

Abstract INTRODUCTION: Visceral leishmaniasis (VL) is fatal if not diagnosed and treated. This study aimed to estimate the cost-effectiveness of diagnostic-therapeutic alternatives for VL in Brazil. METHODS: A decision model estimated the life expectancy and costs of six diagnostic-therapeutic strategies. RESULTS: IT LEISH + liposomal amphotericin B emerged the best option, presenting lower costs and higher effectiveness. DAT-LPC + liposomal amphotericin B showed an incremental cost-effectiveness ratio of US$ 326.31 per life year. CONCLUSIONS: These findings indicate the feasibility of incorporating DAT and designating liposomal amphotericin B as the first-line drug for VL in Brazil.

Annual Report of the Korean Association of External Quality Assessment Service on Transfusion Medicine (2018) / 임상검사와정도관리

Herein, we report the results of the 2018 survey on the external quality assessment (EQA) scheme for the Transfusion Medicine Program (TMP) in Korea. The proficiency testing specimens were prepared at Ajou University Hospital and were sent to the participants biannually. The average accuracy rates for ten different test items on the regular survey were as follows: ABO typing, 99.5%–99.8% (N=841); RhD typing, 99.8%–100.0% (N=827); crossmatching, 89.4%–99.6% (N=708); ABO subtyping, 94.2% and 94.4% (N=53); Rh CcEe antigen testing, all 100.0% (N=51); weak D test, 80.9% (N=207) for the first trial and not graded for the second trial; antibody screening, 99.7%–100.0% (N=304); direct antiglobulin test (DAT) using a polyspecific reagent, 98.9%–100.0% (N=264); DAT using an immunoglobulin-G monospecific reagent, all 100.0% (N=66); DAT using a C3d monospecific reagent, 97.0%–100.0% (N=67); antibody identification, 98.4%–100.0% (N=127); and ABO antibody titration, 84.6%–100.0% (N=73). There were approximately 10.5% more participants in the 2018 EQA scheme than that in 2017. Excellent survey results were obtained in the 2018 EQA scheme compared with those in 2017, except for the weak D test. The 2018 EQA scheme for the TMP should be helpful for improving the quality of the participating laboratories.

Analysis of Screening Results in 501 Newborns with Hemolytic Disease / 中国实验血液学杂志

OBJECTIVE@#To explore the relationship between the serological detection of neonatal hemolytic disease (HDN) and related factors, and to observe the detection rate and specificity of the antibodies against the blood group in the newborn hemolytic disease.@*METHODS@#Maternal-neonatal blood type was detected firstly, and then the direct antiglobulin test(DAT), the free antibody test and the antibody release test were used to detect the occurrence of HDN; For those suspected hemolytic disease except ABO or direct DAT result over 2+, the indirect antiglobulin test with irregular antibody were used for screening cells and the plasma of the patient and mother, and then to detezmine whether there is a corresponding antigen in the red blood cells of the patient to confirm whether hemolytic disease of the other blood type system exists or not. The analysis was carried out by SPSS 22 software. The statistical analysis of classified data was tested by χ test. P<0.05 was considered as statistically significance.@*RESULTS@#A total of 501 cases of hyperbilirubinemia were collected. Among them 250 cases of HDN were diagnosed as HDN, and the detection rate was 49.90%.The detection rate of the male was 45.14%, and that of the female was 56.34%(χ =6.143, P<0.05). The average day-age of patients was 3.97±2.81 days. The analysis of relatianship between the detected rate of HDN and the day-age of HDN chilren showed that the day-age of HDN chilren affected the detected rate of HDN(χ =63.489, P<0.05). The analysis of positive rate of 3 test in HDN childen of every group found that the day-age had an infuence on the detected rate of direct antiglobulin test(χ=18.976，P<0.01) and also had an influence on the detected rate of the free antibody test(χ=9.650，P<0.05). The positive rate of the release test in HDN patients was highest（100%）. 244 cases suffered from ABO hemolysis, including 1 case of ABO hemolysis combined with Rh system (anti -E) hemolysis, 4 cases of Rh system (anti -D), 2 cases of MN system (1 case was caused by anti -M, 1 case was caused by low frequency anti -Mur). ABO HDN caused by anti-A or anti-B were not statisticaly significant.@*CONCLUSION@#Hemolytic disease of the newborn is a common cause of neonatal hyperbilirubinemia. The positive rate of HDN has a certain relations with the sexual distinction and the day-age. But there is no significant difference between anti-A and anti-B type. At the same time, screening and identification of irregular antibodies should be carried out to avoid diagnostic errors caused by undetected antibody when necessary.

Red blood cell alloimmunization among hospitalized patients: transfusion reactions and low alloantibody identification rate

ABSTRACT Background: Unexpected red blood cell alloantibodies can cause hemolytic transfusion reactions. In this study, the prevalence of alloimmunization, the rate of identification of alloantibodies and the rate of blood transfusion reactions among transfused patients were identified in a clinical emergency hospital in Brazil. Methods: Transfusions and clinical records of patients who had a positive indirect antiglobulin test between January and December 2013 were analyzed. Results: Of 1169 patients who received blood transfusions, 28 had positive indirect antiglobulin tests, with one patient having two positive tests at different times, resulting in 29 positive tests during the period of this study. Alloantibodies were identified in 58.6% (17/29) of the cases. In 27.5% (8/29), identification was inconclusive and it was not possible to confirm alloimmunization. The rate of red blood cell alloimmunization was 1.71% (21/1169). Of 21 cases of alloimmunization, four (19%) were unidentified due to an unusual agglutination profile. All identified alloantibodies were clinically significant (10/17 anti-Rh, 5/17 anti-Kell and 2/17 anti-MNS). In two patients who had positive indirect antiglobulin tests, one had an unidentified alloantibody, and the other had an inconclusive test and developed a hemolytic transfusion reaction. Conclusion: The prevalence of clinically important red blood cell alloantibodies and hemolytic transfusion reactions among patients with unidentified alloantibodies suggests that specific laboratory techniques should be performed to identify alloantibodies in cases of pan-reactivity or autoantibodies to improve transfusion safety.

Transfusion management for patients taking an anti-CD38 monoclonal antibody

Abstract Introduction: Pre-transfusion tests, essential for the release of blood components, may be affected by drugs. Monoclonal antibodies represent a class of medications increasingly used in the clinical practice, with anti-CD38 monoclonal antibodies (daratumumab) being a promising resource in the treatment of refractory myeloma. This monoclonal antibody recognizes CD38 in myeloma cells and interferes with pre-transfusion tests by causing panreactivity in indirect antiglobulin tests thereby clinically masking alloantibodies. Dithiothreitol is a reagent that breaks disulfide bonds and effectively destroys antigenic sites for CD38 on red blood cells. This study reports the immunohematological findings of pre-transfusion tests of patients with multiple myeloma receiving daratumumab and on solutions to prevent the interference of this monoclonal antibody. Methods: Serum samples from five patients on anti-CD38 monoclonal antibody treatment were evaluated. Tests performed included ABO/RhD typing, indirect antiglobulin test, direct antiglobulin test and eluate test. A daily evaluation was performed to determine the shelf life of dithiothreitol-treated red blood cells when stored in Alsever's solution. Results: No interference in the ABO/RhD typing results was noted but in all samples, a panreactivity was observed in indirect antiglobulin tests. Regarding the direct antiglobulin test, two samples presented positive results but negative eluates. In all samples, treatment of reagent red blood cells with 0.2 M dithiothreitol offset interference by anti-CD38 monoclonal antibodies. Dithiothreitol-treated red blood cells stored in Alsever's solution were stable for up to 15 days. Conclusion: Treatment of reagent red blood cells with dithiothreitol can be efficient and accessible to offset the interference of the anti-CD38 drug in pre-transfusion tests. The number of costly serological workups can be reduced by having stored dithiothreitol red blood cells with this proving to be a useful reagent for investigating anti-CD38.

Cytomegalovirus-Associated Severe Direct Antiglobulin Test Negative Hemolytic Anemia: A Case Report / 임상소아혈액종양

Cytomegalovirus is a common virus that is mostly asymptomatic when infected, but rarely causes life-threatening hemolysis especially in immunocompromised children. We report a case of antiglobulin test negative severe hemolytic anemia caused by cytomegalovirus infection developed in an immune competent 9-year-old girl. The patient's hemoglobin level was 4.8 g/dL on the day of admission. The diagnosis was achieved by exclusion of other causes of hemolytic anemia and serological evidence of recent CMV infection. The patient was successfully treated with anti-viral agents and steroids resulting in recovery from anemia. Clinicians should consider cytomegalovirus infection in the differential diagnosis of hemolytic anemia in pediatric patients.

Annual Report on the External Quality Assessment Scheme for Transfusion Medicine in Korea (2017)

Here, we have reported results of the surveys on the external quality assessment scheme (EQA) of the Transfusion Medicine Program (TMP) in Korea that were carried out in 2017. The proficiency testing specimens were prepared at Ajou University Hospital, and sent to the participants biannually. The average accuracy rates (N=the number of participants) for ten different test items on the regular survey were as follows: ABO typing, 99.1%–99.9% (N=714); RhD typing, 99.3%–100.0% (N=695); crossmatching, 88.9%–98.5% (N=618); ABO subtyping, 80.7% and 96.0% (N=51); Rh CcEe antigen testing, 98.8%–100.0% (N=51); weak D test, 99.3% and 100.0 (N=150); antibody screening, 98.6%–100.0% (N=295); direct antiglobulin test (DAT) using a poly-specific reagent, 99.2%–100.0 (N=256); DAT using an immunoglobulin-G monospecific reagent, all 100.0% (N=68); DAT using a C3d-monospecific reagent, 83.6%–100.0% (N=72); antibody identification, 88.7%–99.2% (N=123); and ABO Ab titration, 84.6%–100.0% (N=73). The number of participants for the EQA for TMP in 2017 was much higher than that in 2016. Except for the case of ABO subtyping, excellent survey results for the 2017 EQA for TMP were obtained, compared to those in 2016. Thus, the EQA for TMP in 2017 should be helpful for improving the quality of the participating laboratories.

Warm autoimmune hemolytic anemia: experience from a single referral center in Mexico City

BACKGROUND: Autoimmune hemolytic anemia (AIHA) is characterized by an autoimmune-mediated destruction of red blood cells. Warm AIHA (wAIHA) represents 60% of AIHA cases and is associated with the positive detection of IgG and C3d in the direct antiglobulin test (DAT). This study aimed to assess the clinical and laboratorial differences between primary and secondary wAIHA patients from a referral center in Mexico City. METHODS: All patients diagnosed with wAIHA in our institution from January 1992 to December 2015 were included and received corticosteroids as the first-line treatment. We analyzed the response to the first-line treatment, relapse-free survival, and time to splenectomy. RESULTS: Eighty-nine patients were included. Secondary wAIHA represented 55.1% of the cases. At diagnosis, secondary wAIHA patients showed a DAT mixed pattern more frequently than primary wAIHA patients (36.7 vs. 17.5%, P<0.001). In the survival analysis, patients with secondary wAIHA had a lower time to response (18 vs. 37 days, P=0.05), median disease-free survival (28.51 vs. 50.95 weeks, P=0.018), and time to splenectomy (43.5 vs. 61 wks, P=0.029) than those with primary wAIHA. Due to economic constraints, rituximab was considered as the third-line treatment in only two patients. CONCLUSION: Secondary wAIHA may benefit from a longer low-dose steroid maintenance period mainly due to its shorter time to relapse and time to splenectomy than primary wAIHA.

Anti-LW Showing Anti-D Mimicking Specificity: A Case Report / 대한수혈학회지

LW antigens are expressed in higher intensities in D-positive blood cells than D-negative cells, which can result in false identification of anti-D in pretransfusion testing. Although several cases of anti-LW have been reported abroad, to the best of our knowledge, none have been reported in Korea. Herein, we report a case of anti-LW in a 58 year-old RhD positive patient with non-Hodgkin's lymphoma with a positive direct Coombs test and a suspicion of the presence of passive anti-D antibodies because of a history of intravenous immunoglobulin administration. However, during a 5-month follow up, the antibody was confirmed as anti-LW on grounds that it showed weakened reaction in dithiothreitol treated cells and enforced reaction in cord O+ cells when compared to the results from antibody identification panel cells.

Revisiting the Pre-transfusion Test: A Case of Acute Hemolytic Transfusion Reaction due to Multiple Alloantibodies of Anti-E, Anti-c, Anti-Jk(b) / 대한수혈학회지

We report a case of acute hemolytic transfusion reaction due to multiple alloantibodies. A 41-year-old male with multiple histories of transfusion was admitted for jaundice and oliguria after receiving two units of red blood cells in a local clinic. He showed acute renal failure and disseminated intravascular coagulation. Direct Coombs test was negative and antibody screening test showed strong positive results. Anti-E, anti-c, and anti-Jk(b) antibodies were identified in two panels of unexpected antibody assays. Acute hemolytic transfusion was diagnosed, and he was discharged after 1 month of supportive treatment. Unexpected antibody detection tests, including the antiglobulin phase, should be performed to prevent adverse transfusion reactions by unexpected antibodies. Better precision and quality control are necessary when performing pre-transfusion tests.

Neonatal indirect hyperbilirubinemia and glucose-6-phosphate dehydrogenase deficiency / 소아과

PURPOSE: This study aimed to determine the prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency among infants with neonatal indirect hyperbilirubinemia (NIH); compare G6PD-deficient and G6PD-normal patients regarding hyperbilirubinemia and need for exchange transfusions (ET); and assess risk factors for ET and kernicterus. METHODS: This is a case-control retrospective study. Medical records of NIH patients admitted to the Pediatric Department, Salmaniya Medical Complex, Bahrain, between January 2007 and June 2010 were reviewed. Data on sex, age at presentation, hospitalization duration, need for ET, hemoglobin (Hb) level, reticulocyte count, direct Coombs test, serum total and indirect bilirubin levels, thyroid function, blood and urine cultures, G6PD status, and blood groups were collected and compared between the G6PD-deficent and G6PD-normal patients. RESULTS: Of 1,159 NIH patients admitted, 1,129 were included, of whom 646 (57%) were male. Among 1,046 patients tested, 442 (42%) were G6PD deficient, 49 (4%) needed ET, and 11 (1%) had suspected Kernicterus. The G6PD-deficient patients were mainly male (P<0.0001), and had lower Hb levels (P<0.0001) and higher maximum bilirubin levels (P=0.001). More G6PD-deficient patients needed ET (P<0.0001). G6PD deficiency (P=0.006), lower Hb level (P=0.002), lower hematocrit count (P=0.02), higher bilirubin level (P<0.0001), higher maximal bilirubin level (P<0.0001), and positive blood culture result (P<0.0001) were significant risk factors for ET. Maximal bilirubin level was a significant risk factor for kernicterus (P=0.021) and independently related to ET (P=0.03). CONCLUSION: G6PD deficiency is an important risk factor for severe NIH. In G6PD-deficent neonates, management of NIH should be hastened to avoid irreversible neurological complications.

The Clinical Significance of Implementing Concurrent Direct Antiglobulin Test and Antibody Elution Test in Diagnosing Hemolytic Disease of the Fetus and Newborn / 대한수혈학회지

BACKGROUND: Hemolytic disease of the fetus and newborn (HDFN) is a condition in which immune hemolytic anemia occurs in fetuses or newborns as a result of maternal alloimunized antibodies transfer. Antibody elution test and direct antiglobulin test (DAT) can be performed to diagnose HDFN; maternal originated antibodies cannot be confirmed if DAT is utilized alone. In this study, we analyzed the clinical significance of implementing concurrent DAT and antibody elution test in diagnosing HDFN. METHODS: We retrospectively analyzed the DATs and antibody elution tests that were simultaneously conducted in a period of 11 years, between 2005 and 2015, in newborns that received hemoglobin, reticulocyte, and total bilirubin tests. According to the results of these tests, the number of newborns diagnosed with HDFN was measured. Furthermore, the sensitivity and specificity of DAT and antibody elution test were compared. RESULTS: Among 325 newborns, the results of DATs and antibody elution tests were both negative in 208 (64.0%), negative and positive, respectively, in 80 (24.6%), positive and negative in 10 (3.1%), both positive in 27 (8.3%). When this was compared to the clinical diagnosis of HDFN, more sensitive and specific diagnoses were possible when implementing DAT and antibody elution test together (sensitivity of 76.9% for antibody elution test and specificity of 90.3% for DAT). Twenty-six (8.0%) newborns suspected for HDFN showed clinically significant hemolytic anemia. CONCLUSION: It is necessary to conduct both DAT and antibody elution test when HDFN is suspected. The severity of hemolysis in HDFN can be indirectly anticipated using an antibody elution test confirming maternal originated alloantibodies.

Annual Report on the External Quality Assessment Scheme for Immunohematology Testing in Korea (2016)

We report here the 2016 results of surveys on an external quality assessment (EQA) for immunohematology testing in Korea carried out in 2016. The proficiency testing specimens were prepared at Ajou University Hospital, and the specimens were sent to participants biannually. The average accuracy rates (N=the number of participants) for ten different test items on the regular survey were as follows: ABO typing, 98.9%–99.7% (N=627); RhD typing, 98.6%–99.8% (N=607); crossmatching, 90.1%–98.1% (N=454); ABO subtyping, 92.7%–93.0% (N=42); Rh CcEe antigen testing, 97.8%–100.0% (N=46); weak D test, 70.8%–73.6% (N=91); antibody screening, 96.6%–100.0% (N=267); direct antiglobulin test (DAT) using a polyspecific reagent, 92.2%–99.6% (N=49); DAT using an immunoglobulin G monospecific reagent, 84.1%–100.0% (N=68); DAT using a C3d monospecific reagent, 80.0%–100.0% (N=68); antibody identification, 96.7%–99.1% (N=118); and ABO antibody titration, 82.4%–100.0% (N=42). Excellent results for the 2016 EQA for immunohematology testing were obtained, except for those of the weak D test. The EQA for immunohematology testing in 2016 should be helpful for improving the quality of participating laboratories.

Algorithm for Differential Diagnosis of in Vitro and in Vivo Hemolysis in Anticoagulated Blood Specimens

BACKGROUND: Hemolytic specimens contain components that interfere with clinical laboratory results. We evaluated previously published hemolysis indices (HI) and developed an algorithm for differentiating between mechanical hemolysis and immune-mediated hemolysis based on complete blood count (CBC). METHODS: Sixty-three residual EDTA (ethylenediamine tetraacetic acid)-anticoagulated blood specimens were obtained during regular health check-ups, and each specimen was divided into 3 aliquots (A control, B, and C group). Aliquots B and C were mechanically hemolysed by 2 and 5 aspirations, respectively, using a 25-gauge needle before testing; aliquot A was analysed immediately without hemolysis. Additionally, we collected 36 specimens from patients suspected of having immune-mediated hemolysis after thorough reviewing their various laboratory results including direct antiglobulin test. We compared CBC parameters between the groups (A, B, C, D [B+C], and E [immune-mediated hemolysis group]). RESULTS: Our HI scoring system using the sum of red blood cell ghosts, measured hemoglobin-calculated hemoglobin, mean corpuscular hemoglobin concentration-corpuscular hemoglobin concentration mean, and mean platelet volume rather than mean corpuscular hemoglobin, effectively identified mechanical hemolysis; the results were similar to those of previous studies. Furthermore, the HI score using the sum of mean corpuscular volume, red cell distribution width, hemoglobin distribution width, polymorphonuclear %, and neutrophil % differentiated mechanical hemolysis from immune-mediated hemolysis (cut-off, 9; sensitivity, 91.7%; specificity, 92.9%; area under the receiver operating characteristic curve, 0.965 [95% confidence interval, 0.924–0.988]). CONCLUSIONS: The newly developed algorithm may provide effective screening for detecting hemolysis and differential diagnosis of mechanical hemolysis and immune-mediated hemolysis based on CBC results.