Reacción hemolítica transfusional tardía en un paciente con anemia de células falciformes: reporte de un caso / Delayed hemolytic reaction to transfusion in sickle cell anemia: report of one case

Sickle cell anemia was a rare disease in Chile, especially in adults, however the recent immigration wave from Haiti is changing this scenario. We report a 29 year old black female from Haiti with a non-disclosed history of sickle cell anemia. She was transfused with two units of red blood cells, found unconscious and with jaundice five days later and admitted to the hospital. On admission she had a hemoglobin of 3.3 g/dL, a total bilirubin of 5.08 mg/dL, a LDH of 1,306 Ui/L. She was transfused again, worsening her condition. An alloimmunization and delayed hemolytic reaction was suspected. A direct Coombs test was positive. She was treated with steroids and her serum hemoglobin rose progressively.

A Case of Sickle Cell Anemia with a Lack of High Frequency Red Blood Cell Antigen / 대한수혈학회지

Patients with sickle cell anemia are chronically transfused. Therefore, it is important to prevent the alloimmunization of RBC antigens. The authors identified a high frequency antigen-negative blood group in patients with sickle cell anemia. As the number of foreigners residing in Korea is increasing, it is necessary to know what to consider when transfusing blood to sickle cell anemia patients. Patients with sickle cell anemia should be informed of the exact blood group type using extended RBC typing to confirm the ABO, Rh, Kell, and Duffy blood types at diagnosis or before the first blood transfusion. Extended matched blood transfusion can reduce the risk of alloimmunization of RBC antigens.

Clinical analysis of seven cases of rare hemolytic disease of the newborn / 中华儿科杂志

Objective@#To summarize the clinical features of 7 rare cases of hemolytic disease of newborn (HDN), and to improve the understanding of rare HDN.@*Methods@#Data of clinical information, laboratory findings, treatments and outcomes were collected and analyzed for four cases with HDN due to anti-M, two cases due to anti-Kidd, and one case due to anti-Duffy. All of them were admitted to the Department of Neonatology, Beijing Children's Hospital Affiliated to Capital Medial University from July 2007 to June 2017.@*Results@#Among the four MN hemolytic babies, two were males and two were females. Jaundice was found in three cases. Two cases had hyperbilirubinemia, one of them had severe hyperbilirubinemia. All the four cases developed anemia, including severe anemia in three cases. Two cases of Kidd hemolytic disease and 1 case of Duffy hemolytic disease had jaundice and anemia, but did not reach the level of severe hyperbilirubinemia and severe anemia. MN hemolytic disease babies got negative results in direct antiglobulin test, whereas the Kidd and Duffy hemolytic disease babies had positive findings in direct antiglobulin test. None of the babies had blood transfusion, and they were discharged from the hospital.@*Conclusions@#Without maternal and fetal blood group incompatibility (ABO or Rh blood-group system), for early onset of jaundice, severe jaundice or anemia, antiglobulin test to mother and child earlier should be administered, and MN, Kidd, Duffy and other rare hemolytic disease of the newborn should be pay attention to.

Case report and literature review of neonatal hemolysis induced by anti-E and anti-Ec combined with anti-Fyb / 天津医药

Hemolytic diseases of newborn (HDN) can cause miscarriage, premature birth, fetal edema, fetal intrauterine anemia and even fetal death in early pregnancy. Neonatus with HDN can have jaundice, anemia, hepatosplenomegaly, edema and nuclear jaundice sequelae. This article reviewed the diagnosis and treatment of two patients with HDN caused by anti-E and anti-Ec combined with anti-Fyb, and reviewed the relevant literature on the epidemilogy, the diagnosis and treatment of HDN in order to improve the understanding of the disease.

Expresión del fenotipo Duffy negativo en mujeres afrodescendientes y su relación con la preeclampsia / Expression of the Duffy negative phenotype in women of African descent and their relation to pre-eclampsia

La preeclampsia (PE) es una complicación del embarazo que trae consigo algunas consecuencias negativas para la madre y el feto: en la madre provoca principalmente hipertensión y proteinuria, mientras que en el feto puede presentarse trombocitopenia, alteración en el desarrollo del sistema nervioso central y circulatorio, y restricción del crecimiento intrauterino, lo cual se considera el factor de riesgo principal de muerte fetal en nacimientos producto de una PE severa. En la preeclampsia se presenta una disfunción endotelial relacionada con placentación anormal, estado de estrés oxidativo y proceso inflamatorio sistémico, que lleva a la activación de neutrófilos y monocitos. Se ha considerado a la interleucina-8 (IL-8) como un posible candidato desencadenante por ser quimioatrayente y activador de leucocitos; en la circulación sanguínea, la IL-8 se une a un receptor de quimiocina multiespecífico de alta afinidad denominado DARC, que es idéntico al antígeno del grupo sanguíneo Duffy. Este receptor regula los niveles plasmáticos de IL-8, uniéndose a esta quimiocina, pero cuando hay una mutación en la región promotora del gen se altera la expresión de DARC, lo que conlleva a que la IL-8 de los factores genéticos involucrados en la activación de los neutrófilos y de los monocitos, y por ende, en la disfunción endotelial presentada durante este síndrome hipertensivo, especialmente en la población afrodescendiente.

Preeclampsia (PE) is a complication of pregnancy that brings some negative consequences for both mother and fetus. It specially causes hypertension and proteinuria in mothers; while in fetuses it causes thrombocytopenia, development alterations of the central nervous and circulatory system; also intrauterine growth restriction may occur. This last factor is regarded as the main risk factor for fetal death in births as a result of severe PE. There is endothelial dysfunction in preeclampsia related to abnormal placentation, state of oxidative stress and systemic inflammatory process that leads to the activation of neutrophils and monocytes. Interleukin-8 (IL-8) is considered as a possible trigger candidate, since this chemokine is a chemoattractant and leukocyte activator. In the bloodstream, interleukin-8 binds to a high affinity multispecific-chemokine receptor called DARC, which is identical to the Duffy blood group antigen. This receptor regulates plasma levels of IL-8 by binding to chemokine. But, when there is a mutation in the gene promoter region, DARC expression is altered, and IL-8 inefficiently binds to receptor. This mutation results in Duffy negative phenotype, which is present in most of African descendants. This literature review is intended to address the role of IL-8 as neutrophil chemo-attractant, the importance of Duffy blood system and the possible association between ethnicity and preeclampsia.

Actividad del cortisol plasmático en ratas en condiciones de estrés crónico suplementadas con resveratrol / Plasma cortisol activity in rats under conditions of chronic stress supplemented with resveratrol

Objective: Determine the activity of cortisol in rats treated with exogenous adrenocorticotropic hormone (ACTH) and a resveratrol supplement. Methodology:Forty-eight adult male rats and 16 adult female rats (Rattus norvegicus) three months old with a body weight of 200 to 250g and 300 to 350g for both male and female were used and kept in controlled environmental conditions, temperature of 20±2°C and light-dark cycles of 14 and 10 hours. They were fed with balanced food and had free access to water. The rats were randomly divided into four groups: group 1, was treated with 5 µg/kg of ACTH i.p. every twelve hours; group 2, received the same treatment with ACTH plus a grape extract supplementation of 40 mg/kg; group 3, only received grape extract and group 4,served as control and only received saline solution (0.9%) i.p. The experimental was designed as a 2×2 factorial with two ACTH levels and two extract grape levels. Results:Significant differences were not found in cortisol concentrations for day, gender or treatment effects (0.75ug/dL ± 0.11; p<0.001).

Objetivo: Determinar la actividad de cortisol en ratas tratadas con hormona adrecorticotropa (ACTH) exógena y un suplemento de resveratrol.Metodología:Se utilizaron 48 ratas hembras adultas y 16machos de la cepa wistar (Rattus norvegicus) de tres meses de edad y con un peso corporal de 200-250g y 300-350 g, para hembras y machos, respectivamente, que permanecieron en condiciones ambientales controladas, temperatura 20±2°C de ciclos de luz-oscuridad de 14 y 10 horas, respectivamente.Se les proporcionó alimento balanceado con libre acceso a agua. Las ratas fueron divididas en cuatro grupos al azarasí: grupo 1, fue tratado con 5 µg/kg de ACTH i.p. cada 12 horas; grupo 2, recibió el mismo tratamiento con ACTH además de una suplementación oral de 40 mg/kg de extracto deshidratado de uva (resveratrol); grupo 3, solo recibió extracto de uva y el grupo 4,recibió solución salina y sirvió como control y (0,9%) i.p. y oral. El diseño experimental fue en factorial 2×2, con dos niveles de ACTH y dos niveles de polifenol. Resultados: No se encontraron diferencias significativas del cortisol sanguíneo, con respecto al día y sexo, entre los tratamientos (0,75ug/dL ± 0,11; p<0,001). Conclusión: Los resultados sugieren que el estrés crónico y el consumo de resveratrol no alteran directamente los niveles plasmáticos de cortisol, en ratas estresadas y no estresadas. De la misma manera que, posiblemente la dosis utilizada de ACTH no produjo estimulación de la glándula suprarrenal en las ratas.

Duffy blood group system and the malaria adaptation process in humans

Malaria is an acute infectious disease caused by the protozoa of the genus Plasmodium. The antigens of the Duffy Blood Group System, in addition to incompatibilities in transfusions and hemolytic disease of the newborn, are of great interest in medicine due to their association with the invasion of red blood cells by the parasite Plasmodium vivax. For invasions to occur an interaction between the parasites and antigens of the Duffy Blood Group System is necessary. In Caucasians six antigens are produced by the Duffy locus (Fya, Fyb, F3, F4, F5 and F6). It has been observed that Fy(a-b-) individuals are resistant to Plasmodium knowlesi and P. vivax infection, because the invasion requires at least one of these antigens. The P. vivax Duffy Binding Protein (PvDBP) is functionally important in the invasion process of these parasites in Duffy / DARC positive humans. The proteins or fractions may be considered, therefore, an important and potential inoculum to be used in immunization against malaria.

Chagas' disease and Duffy antigen/receptor for chemokine (DARC): a mini-review

Duffy gene (FY) codifies the transmembrane glycoprotein Duffy (gp-Fy) of 35 to 43 kDa which is moderately immunogenic. This glycoprotein is polymorphic, and constitutes the antigens of the Duffy histo-blood system which were designated receptors for chemokines and denominated DARC (Duffy antigen/receptor for chemokine). This receptor has an important role in the regulation of chemokine levels in the circulation, as it binds and adsorbs them on the surface of red cells as a reservoir. It plays a "sink" role, which can contribute to homeostasis by removing inflammatory chemokines from circulation as well as maintaining them in plasmatic levels. Chronic Chagas' cardiopathy (CCC) is the most frequent form of the disease. It is an inflammatory disease, in which infiltrated inflammatory cells play an important role in the development and progress of the infection. High chemokine levels in the plasma have been associated with the disease severity in patients with heart failure. In this context, the profile of DARC expression could play an important function as a receptor for chemokines in Chagas' disease, in patients with CCC, as it can modulate damage from this inflammatory disease.