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Query: UMLS:C0002878 (hemolytic anemia)
 7,530 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hemolysis is a frequent complication of the implant of prosthetic valves, and is conditioned by a variety of factors, most of which related to the type of valve implanted and to the hemodynamic conditions following implantation. If sufficiently severe, it may lead to varying degrees of hemolytic anemia. The following laboratory tests are useful to diagnose and assess the severity of hemolytic anemia: hemoglobin levels; reticulocyte count; the demonstration of schistocytes on a blood smear; serum levels of lactic dehydrogenase, haptoglobin and iron. Treatment of hemolysis includes the supplementation of iron and folate when their deficiency is evident. Transfusions are necessary only in cases of severe anemia refractory to treatment. The use of beta-blockers appears to decrease the severity of hemolysis, likely because of the induction of bradycardia and of their negative inotropic effects. Some cases have been described of erythropoietin treatment for hemolytic anemia in these conditions, with favorable outcome. However erythropoietin use should currently be restricted to patients with severe hemolytic anemia in whom surgical repair or transfusions should be avoided or deferred. The recognition and the estimation of severity of hemolysis after valve implantation are important steps in the patients' follow-up and the premise for a rational treatment.
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PMID:[Hemolysis following valve surgery]. 1466 92

Clinical uses for recombinant human erythropoietin (rHuEPO) therapy continue to expand. Initial use was in anaemia associated with end-stage renal disease, but more recently there have been many reports of the benefits of erythropoietin in other clinical situations such as cancer-related anaemia. Recombinant erythropoietin reduces the need for blood transfusion and hence exposure to donor blood products as well as improving quality of life. We report four patients who were transfusion dependent, none of whom had licensed indications for the use of recombinant erythropoietin. Two patients had microangiopathic haemolytic anaemia secondary to mechanical valve haemolysis and were unsuitable for any further cardiac intervention. One patient had anaemia of chronic disease and anti-Vel red cell antibodies, making compatible blood transfusions difficult to obtain. The fourth patient had primary thrombocythaemia and developed transfusion-dependent anaemia secondary to myelosuppressive agents. All four patients had a relative deficiency in endogenous erythropoietin levels ranging between 7 and 41 IU/l. After commencing recombinant erythropoietin therapy, all had a response in haemoglobin of at least 1 g/dl with an overall improvement in their quality of life. We conclude that rHuEPO is a very convenient and useful form of treatment in transfusion-dependent anaemia and in some cases beyond the licensed indications.
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PMID:Novel uses for recombinant erythropoietin therapy in unlicensed indications. 1504 70

Regulatory interferences at the iron transporter ferroportin 1 (Fpn1) cause transient defects in iron homeostasis and erythropoiesis in polycythaemia (Pcm) mutant mice. The present study identified decreased Fpn1 expression in placental syncytiotrophoblast cells at late gestation as the mechanism of neonatal iron deficiency in Pcm mutants. Tissue specificity of embryonic Fpn1 dysregulation was evident from concomitant decreases in Fpn1 mRNA and protein expression in placenta and liver, as opposed to upregulation of Fpn1 protein despite decreased transcript levels in spleen, implicating post-transcriptional regulation of Fpn1. Dysregulation of Fpn1 and decreased iron levels in Pcm mutant spleens correlated with apoptotic cell death in the stroma, resulting in a semidominant spleen regression. At 7 weeks of age, a transient increase in spleen size in Pcm heterozygotes reflected a transient erythropoietin-mediated polycythemia. Structurally, Pcm mutant spleens displayed a severe defect in red pulp formation, including disruption of the sinusoidal endothelium, as well as discrete defects in white pulp organization during postnatal development. Reduced functional competence of the Pcm mutant spleen was manifested by an impaired response to chemically induced hemolytic anemia. Thus, aberrant Fpn1 regulation and iron homeostasis interferes with development of the spleen stroma during embryogenesis, resulting in a novel defect in spleen architecture postnatally.
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PMID:Dysregulation of ferroportin 1 interferes with spleen organogenesis in polycythaemia mice. 1534 64

RBC transfusions in a patient with a history of autoimmune hemolytic anemia (AIHA) can represent both a laboratory and a clinical challenge. The development of high-titer low-avidity antibodies and antibodies to high-frequency antigens may further impair the ability to identify compatible donor RBCs. Not infrequently, incompatible RBCs must be used and the desire to increase oxygen carrying capacity conflicts with the desire to avoid exacerbating the autoimmune hemolytic process with RBC transfusions. A 66-year-old Caucasian female with coronary artery disease and a history of refractory AIHA had recently developed anemia and required multiple RBC transfusions. The patient had maintained adequate RBC counts with erythropoietin and prednisone therapy for the previous 16 months. With the recent worsening of her hemolytic anemia, she had developed angina that was treated with RBC transfusions in an outpatient setting. However, her angina increased as her RBC counts decreased, leading to hospital admission for further management of her hemolytic anemia and angina. She subsequently required multiple incompatible RBC transfusions despite increased prednisone therapy and did not improve until after coronary artery stent placement and high dose IVIG therapy. This case demonstrates the usefulness of early patient phenotyping in a case of accelerating hemolytic anemia to aid in donor RBC selection, the value of communicating with clinicians and the patient regarding the use of least-incompatible RBCs, and the importance of optimizing the patient's clinical condition to avoid ischemia. In addition, it demonstrates the value of repeated attempts with IVIG treatment despite previous refractoriness to this treatment.
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PMID:Case report: exacerbation of hemolytic anemia requiring multiple incompatible RBC transfusions. 1537 49

Mechanical circulatory assist devices (MCADs) are increasingly utilized independently of cardiac transplantation in the management of heart failure. Though MCAD use incorporates inherent mechanical risks, the inevitable onset of chronic anemia, with its associated morbidity and mortality, is also a significant concern. MCAD support has been correlated with elevated plasma levels of inflammatory cytokines TNF-alpha, IL-1beta, and IL-6, which have separately been found to inhibit erythropoietin (Epo)-induced erythrocyte (RBC) maturation. Previous analysis of hematological parameters for MCAD-supported patients concluded that an amplified inflammatory response impedes RBC proliferation and recovery from hemolytic anemia. Additional analysis may bolster this assertion. Hemoglobin concentration (HC), RBC distribution width (RDW), mean cell volume (MCV), and cardiac index were retrospectively analysed for 78 MCAD-supported patients implanted for greater than 30 days at the University of Arizona Health Sciences Center from 1996 to 2002. Analysis confirms that the HC, a conventional marker for anemia, declines with MCAD placement and remains below the clinically defined, minimum normal value. Inversely, the RDW rises above maximum normal measure, signifying an increased fraction of juvenile RBCs. The MCV remains unchanged and within normal limits, demonstrating adequate substrate for RBC formation. MCAD performance also stabilizes as adequate perfusion returns. These results further support our previously published conclusion that a sufficient response of erythropoiesis occurs in reaction to the onset of anemia by an increased production of immature RBCs. However, the cells never fully mature and join circulation. The patient's inflammatory cytokine response to the implanted device most likely mediates the chronic MCAD-induced anemia by inhibition of Epo effects.
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PMID:Inflammatory cytokine inhibition of erythropoiesis in patients implanted with a mechanical circulatory assist device. 1591 45

Haematological abnormalities are common in systemic lupus erythematosus. Anaemia is found in about 50% of patients, with anaemia of chronic disease being the most common form. Impaired erythropoietin response and presence of antibodies against erythropoietin may contribute to the pathogenesis of this type of anaemia. Patients with autoimmune haemolytic anaemia usually belong to a distinct category, which is associated with anticardiolipin antibodies, thrombosis, thrombocytopenia, and renal disease, often in the context of secondary antiphospholipid syndrome. Autoantibodies, T lymphocytes, and deregulation of the cytokine network can affect bone marrow erythropoiesis, leading to anaemia.
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PMID:Anaemia in systemic lupus erythematosus: from pathophysiology to clinical assessment. 1607 64

Nucleos(t)ide analogues have proven useful in the treatment of viral infections. Ribavirin is a nucleoside, guanosine analogue, whose mechanisms of action include inhibition of inosine monophosphate dehydrogenase (IMPDH), which is the key step in de novo guanine synthesis, a requirement for viral replication. In combination with pegylated interferon alfa, ribavirin is the standard of care for the treatment of chronic hepatitis C today. However, the medication is associated with significant haemolytic anaemia, which may require dose reduction, discontinuation or treatment with recombinant human erythropoietin. Dose reduction also appears to decrease sustained viral clearance rates. Newer IMPDH inhibitors are in various stages of development. Viramidine, a liver-targeting prodrug of ribavirin, has demonstrated significant antiviral activity and erythrocyte-sparing properties. It is currently in Phase 3 trials. Clinical trials of merimepodib, another investigational IMPDH inhibitor, have completed enrolment for a Phase 2b study as a third medication for administration with pegylated interferon plus ribavirin. Although other IMDPH inhibitors also have antiviral activity, these medications appear best suited as immunosuppressive medications at this time.
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PMID:Treating HCV with ribavirin analogues and ribavirin-like molecules. 1629 77

The adverse effects of antiviral drugs are dose dependent and often reversible. The major side effects include influenza-like symptoms, hematologic abnormalities and neuropsychiatric symptoms. The influenza-like syndrome can be prevented by paracetamol taken at the time of the injection. Psychiatric adverse effects range from irritability to a severe depressive syndrome. Antidepressants, such as selective serotonin reuptake inhibitors, may be useful in the management. Adverse hematologic effects can occur very early during treatment. The platelet count often stabilizes rapidly, but neutropenia can deteriorate throughout treatment. In selected patients treatment with hematopoietic growth factor (filgrastim) may be useful. Ribavirin therapy may result in a dose-dependent reversible intravascular hemolytic anemia in 10% of patients. Adjunctive therapy with erythropoietin for ribavirin-induced anemia is currently under evaluation. Interferons and ribavirin are contraindicated in pregnancy. Contraception must be continued for 4 months (women) and 7 months (men) after ribavirin cessation. Lactic acidosis may be a rare complication of combination therapy in patients undergoing therapy for HIV and HCV. Any sign of mitochondrial DNA depletion syndrome calls for blood lactate measurement and, possibly, a modification of antiretroviral treatment. Lamivudine is well tolerated but the emergence of lamivudine-resistant (YMDD) HBV mutants is associated with the loss of clinical response. Adefovir dipivoxil effectively suppresses lamivudine-resistant HBV in chronic hepatitis B.
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PMID:[Treatment of the side effects of antiviral therapy]. 1638 Dec 45

We report a 3-year-old boy with glutathione synthetase deficiency, who in the newborn period developed severe persistent haemolytic anaemia. Treatment with erythropoietin was introduced with good clinical and haematological response.
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PMID:Trial of erythropoietin treatment in a boy with glutathione synthetase deficiency. 1643 14

A 63-year-old male was admitted to our department in 2001 because of autoimmune haemolytic anaemia and splenomegaly. Bone marrow examination revealed an erythroid hyperplasia and a normal chromosome analysis. Splenectomy was performed in 2002, resulting in severe transfusion-dependent anaemia with a need for two blood transfusions each week. Treatment with prednisolone, erythropoietin and danocrine was without effect. A new bone marrow examination then showed myelodysplastic syndrome. Treatment with thalidomide, 50-150 mg/d, and prednisolone, 25 mg/d, resulted in an increase in haemoglobin to 8.9 mmol/l during the following months. The use of immunomodulatory and anti-angiogenetic agents in refractory anaemia needs more study.
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PMID:[Refractory anaemia successfully treated with thalidomide]. 1647 24


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