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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This article has presented an overview of developments in the treatment of HDN. The number of cases requiring treatment that have occurred in the last decade has dropped because of the development and implementation of Rh immunoprophylaxis, although this treatment still appears to be underutilized in the United States. Failure to recognize the need for immunoprophylaxis in certain situations (including unrecognized abortion) has led to a small residual population of alloimmunized mothers who will require comprehensive treatment during subsequent pregnancies. Alloimmunization to other red cell antigens remains a small but significant problem in other women. Although great advances have been made in the monitoring of these pregnancies, amniotic fluid analysis remains a mainstay for the third-trimester evaluation of alloimmunized pregnancies. Noninvasive methods such as ultrasound evaluation and the monocyte assays may supplement, but cannot entirely replace, the need for direct assessment. The most striking advancement in the evaluation and treatment of these infants has been the ability to access the fetal circulation directly through intravenous umbilical cord sampling. This method allows for an immediate assessment of fetal anemia as well as a route for direct fetal transfusion. The method has also permitted a more complete assessment of fetal physiology. However, the method may be overutilized at the present time and has some degree of risk to the fetus, even in experienced hands. Additional methods of treatment for the alloimmunized pregnancy include plasma exchange, intravenous immunoglobulin infusion, and promethazine hydrochloride. The popularity of plasma exchange has probably decreased with the advent of more direct fetal sampling and treatment techniques, but it may be useful in the treatment of first-trimester pregnancy losses. Intravenous immunoglobulin and promethazine hydrochloride appear to be promising alternatives that require more investigation. It is apparent that efforts need to be channeled towards prevention of HDN in a health system that is highly aware of increasing costs and the benefits of preventive medicine.
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PMID:Maternal immunity to red cell antigens and fetal transfusion. 152 27

Forty-nine cases of idiopathic and secondary autoimmune hemolytic anaemia (AIHA) were observed. Infections developed in 64% of them before the diagnosis of the idiopathic form had been established. In 83% of the patients warm antibodies were found, and in half these cases complement activation was observed on the blood cells. In 16% of cases autoantibodies to other tissues were present as well. Higher grade of anaemia and more severe course of the disease were observed in secondary AIHA. The survival time of patients with malignant lymphoma and haemolysis developing during the proliferation phase was twice as long as in cases with simultaneously diagnosed lymphoma and haemolysis. Idiopathic AIHA was more frequent chronic and mild. During over 2 years of follow-up haemolysis regressed in 44% of cases (in idiopathic AIHA in 65% and in secondary AIHA in 19%). The followed-up group comprised 36 patients. No immediate favourable effect of splenectomy was noted. In 43% of cases infections were a complication of pharmacological treatment and (or) splenectomy. Alloimmunization against blood cells was noted in 16% of cases.
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PMID:[Long-term observation of patients with autoimmune hemolytic anemia]. 182 69

Alloimmunization following multiple transfusions, a frequently observed complication in sickle cell anemia patients, may make subsequent transfusion attempts extremely hazardous. The case of a young woman with sickle-cell anemia is reported; she was hospitalized for the treatment of extensive invalidating leg ulcers. The transfusion program that was initiated as a prerequisite to skin allografts had to be stopped, due to rapid occurrence of multiple alloimmunization. Broad spectrum alloantibodies precluded further selection of compatible blood units. In an attempt to get round this impossibility, a treatment with Hydroxyurea was initiated in order to boost synthesis of F-hemoglobin. After one year under this treatment, the patient's clinical status has clearly improved without requiring any new blood transfusion.
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PMID:[A sickle cell homozygote with transfusion deadlock. Favorable outcome with hydroxyurea treatment]. 811 73

Alloimmunization to red cell antigens and haemolytic transfusion reactions may occur after red blood cell transfusion. We describe a case of life threatening postransfusion hyperhaemolysis in a beta thalassaemia patient. For many years, transfusion therapy was stopped but the patient developed a profound anaemia which required splenectomy. At that time, the serum contained a red cell alloantibody with anti-KN3 specificity. In vivo red cell survival studies were performed trying to determine the capacity of this antibody to cause red cell destruction. Unfortunately, these studies triggered again an intense haemolytic process explained by the appearance of red cell auto- and alloantibodies. This case underlines a possible link between the development of alloimmunization and the induction of potentially serious autoimmune haemolytic anaemia.
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PMID:[Severe hemolysis related to an association of erythrocyte allo- and autoantibodies in a thalassemia patients]. 898 18

Hemolytic disease of the fetus and newborn (HDFN) is a consequence of maternal alloimmunization against fetal red blood cell antigens. Alloimmunization against D antigen from Rhesus (Rh) blood group system is particularly important because of its strong immunogenicity. During the last few decades, the introduction of RhD prophylaxis by postpartum administration of anti-D immunoglobulin to RhD negative women, now improved with antenatal prophylaxis, has led to a dramatic decrease in perinatal mortality and morbidity from HDFN. However, severe cases have not disappeared, mostly due to prophylaxis failure. In our case, inappropriate prenatal care during the first pregnancy in an RhD negative mother resulted in primary immunization. In the next pregnancy with an RhD positive child, the mother's secondary immune response was extremely strong and led to early development of severe fetal anemia. The fetus survived thanks to the treatment with intrauterine transfusions (IUT), but they caused suppression of erythropoiesis, which lasted for months after birth. The long lasting, late anemia was treated with repeated postnatal red cell transfusions and recombinant human erythropoietin (rHuEPO). Despite the severity of HDFN in our case, the short-term outcome is good. The boy has normal growth until now, but due to the possibility of an adverse long-term neurodevelopmental outcome, this case requires continuous follow up. It also reminds of the fact that RhD alloimmunization remains an actual problem in daily routine. Antenatal prophylaxis is a crucial step in quality care of those who are at a risk of HDFN.
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PMID:[The importance of antenatal immunoprophylaxis for prevention of hemolytic disease of the fetus and newborn]. 2156 74

Anaemia may be multifactorial in origin. We present a woman with autoimmune hepatitis and secondary warm autoimmune haemolytic anaemia and most likely also concomitant anaemia of chronic disease. A relapse of autoimmune haemolysis was successfully treated with steroids and high-dose intravenous immunoglobulin. At the same time, bleeding from angiodysplasia in the coecum was masked by unauthorised perorally administrated iron. No other cause of bleeding was found. During that period, she required extensive blood transfusions, up to several times per month. Surgical or endoscopic treatment of the bleeding angiodysplasia was not possible. Alloimmunisation developed as a complication to the large number of transfusions, despite the use of steroids. Treatment with somatostatin analogue markedly reduced the need of our patient for blood transfusions for a follow-up period of more than one year, and she has not experienced any side effects. We do not know how long the haemostasis achieved will last, however, we believe that this treatment may be an alternative for other patients as bleeding from angiodysplasia is not uncommon and is often difficult to eradicate.
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PMID:[A woman in her 60s with multifactorial anaemia]. 2256 31

The Kell and Kx blood group systems are expressed as covalently linked molecules on red blood cells (RBCs). The Kell blood group system is very polymorphic, with 35 antigens assigned to the system. The expression of Kell glycoprotein on RBCs is not critical to the erythrocyte function. However, the expression of KX is critical to normal morphology, and null mutations are associated with the McLeod neuroacanthocytosis syndrome. The immunogenicity of the K anigen is second only to the D anigen, and alloantibodies to Kell anigens can cause transfusion reactions and hemolytic disease of the fetus and newborn. Kell alloantibodies in pregnancy are known to suppress erythropoiesis, which can result in serious disease despite low amniotic bilirubin levels and low antibody titers. Late-onset anemia with reticulocytopenia is thought to be attributable to the continual suppression of erythropoiesis from residual alloantibody in the infant. Alloimmunization to XK protein is rare, and expressed polymorphisms have not been reported. Together these two blood group systems share an integral relationship in transfusion medicine, neurology, and musculoskeletal biology.
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PMID:Kell and Kx blood group systems. 2630 65

Haemolytic disease of the foetus and newborn (HDFN) is caused by maternal red blood cells (RBC) alloimmunisation resulted from incompatibility of maternal and foetal RBCs. However, only a few HDFN attributed to anti-M were reported, varying from asymptomatic to severe anaemia with hydrops foetalis and even intrauterine death. A case of severe HDFN due to anti-M alloantibody from an alloimmunized grandmultiparous Malay woman with recurrent pregnancy loss is reported here. The newborn was delivered with severe and prolonged anaemia which required frequent RBC transfusions, intensive phototherapy and intravenous immunoglobulin administration. Although anti-M is rarely known to cause severe HDFN, a careful serological work-up and close assessment of foetal well-being is important, similar to the management of RhD HDFN. Alloimmunisation with anti-M type can lead to severe HDFN and even foetal loss.
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PMID:Anti-M induced severe haemolytic disease of foetus and newborn in a Malay woman with recurrent pregnancy loss. 2841 8

The diagnosis and management of fetal anemia has been at the forefront of advances in the fields of fetal physiology, immunology, fetal imaging, and fetal therapy among others. Alloimmunization and parvovirus infection are the leading cause of fetal anemia in the United States. The middle cerebral artery peak systolic velocity (MCA-PSV) diagnoses fetal anemia. Its discovery is considered one of the most important achievements in fetal medicine. Accumulation of experience in recent years as well as refinement of surgical techniques have led to safer invasive procedures. It is expected that long term follow-up of affected pregnancies, continues to reflect all these improvements in care. It is also expected that treatment of other less common causes of fetal anemia becomes more frequently reported and that the management principles of fetal anemia are successfully applied to other fetal pathologies.
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PMID:Fetal anemia. 3031 79

Patients with beta thalassemia major (TM) are transfusion-dependent (TD) since early childhood and for life. Development of alloantibodies and autoantibodies against red blood cell (RBC) antigens is increasingly recognized as a significant transfusion hazard, especially among heavily transfused patients. The aim of this study is to assess RBC alloimmunization and autoimmunization rates in TD TM patients treated in our Comprehensive Center of Adult Thalassemia, Hemoglobinopathies and Rare Anemias. TD TM patients, regularly transfused every 2-3 weeks, were included in the study. Clinical and RBC transfusion records, including RBC antibodies, since diagnosis in early childhood, were retrieved from patients' files and from the blood bank database. Forty TD TM patients, > 18 years of age, were included in the study. Alloimmunization was demonstrated in 17 (42.5%) patients. Thirty-four alloantibodies were detected, with the most frequent being RH related (12 of 34, 35.3%) followed by those of the Kell system (8 of 34, 23.5%). Age at first transfusion was positively related to the probability of developing alloantibodies (p = 0.02). Splenectomy was found to be correlated with developing alloantibodies (p = 0.016). Logistic regression analysis of the lifelong probability of developing alloantibodies on the age at first transfusion and splenectomy demonstrates a strong positive relationship (p = 0.002). A substantially high rate of alloimmunization was found among adult TD TM patients. Early initiation of RBC transfusions, avoidance of splenectomy and extended Rh and K antigen matching, can reduce the incidence of alloimmunization in TD TM patients.
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PMID:Alloimmunization and autoimmunization in adult transfusion-dependent thalassemia patients: a report from a comprehensive center in Israel. 3248 1


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