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American Family Physician Sep 2018Hemolytic anemia is defined by the premature destruction of red blood cells, and can be chronic or life-threatening. It should be part of the differential diagnosis for...
Hemolytic anemia is defined by the premature destruction of red blood cells, and can be chronic or life-threatening. It should be part of the differential diagnosis for any normocytic or macrocytic anemia. Hemolysis may occur intravascularly, extravascularly in the reticuloendothelial system, or both. Mechanisms include poor deformability leading to trapping and phagocytosis, antibody-mediated destruction through phagocytosis or direct complement activation, fragmentation due to microthrombi or direct mechanical trauma, oxidation, or direct cellular destruction. Patients with hemolysis may present with acute anemia, jaundice, hematuria, dyspnea, fatigue, tachycardia, and possibly hypotension. Laboratory test results that confirm hemolysis include reticulocytosis, as well as increased lactate dehydrogenase, increased unconjugated bilirubin, and decreased haptoglobin levels. The direct antiglobulin test further differentiates immune causes from nonimmune causes. A peripheral blood smear should be performed when hemolysis is present to identify abnormal red blood cell morphologies. Hemolytic diseases are classified into hemoglobinopathies, membranopathies, enzymopathies, immune-mediated anemias, and extrinsic nonimmune causes. Extrinsic nonimmune causes include the thrombotic microangiopathies, direct trauma, infections, systemic diseases, and oxidative insults. Medications can cause hemolytic anemia through several mechanisms. A rapid onset of anemia or significant hyperbilirubinemia in the neonatal period should prompt consideration of a hemolytic anemia.
Topics: Anemia, Hemolytic; Blood Cell Count; Diagnosis, Differential; Humans
PubMed: 30215915
DOI: No ID Found -
American Family Physician Jun 2004Hemolysis presents as acute or chronic anemia, reticulocytosis, or jaundice. The diagnosis is established by reticulocytosis, increased unconjugated bilirubin and... (Review)
Review
Hemolysis presents as acute or chronic anemia, reticulocytosis, or jaundice. The diagnosis is established by reticulocytosis, increased unconjugated bilirubin and lactate dehydrogenase, decreased haptoglobin, and peripheral blood smear findings. Premature destruction of erythrocytes occurs intravascularly or extravascularly. The etiologies of hemolysis often are categorized as acquired or hereditary. Common acquired causes of hemolytic anemia are autoimmunity, microangiopathy, and infection. Immune-mediated hemolysis, caused by antierythrocyte antibodies, can be secondary to malignancies, autoimmune disorders, drugs, and transfusion reactions. Microangiopathic hemolytic anemia occurs when the red cell membrane is damaged in circulation, leading to intravascular hemolysis and the appearance of schistocytes. Infectious agents such as malaria and babesiosis invade red blood cells. Disorders of red blood cell enzymes, membranes, and hemoglobin cause hereditary hemolytic anemias. Glucose-6-phosphate dehydrogenase deficiency leads to hemolysis in the presence of oxidative stress. Hereditary spherocytosis is characterized by spherocytes, a family history, and a negative direct antiglobulin test. Sickle cell anemia and thalassemia are hemoglobinopathies characterized by chronic hemolysis.
Topics: Algorithms; Anemia, Hemolytic; Autoimmune Diseases; Communicable Diseases; Hemoglobinopathies; Hemolysis; Humans; Physical Examination
PubMed: 15202694
DOI: No ID Found -
Nutrients Jan 2023Favism is a hemolytic disease due to the ingestion of fava beans in individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency. There is wide inter- and... (Review)
Review
Favism is a hemolytic disease due to the ingestion of fava beans in individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency. There is wide inter- and intra-individual variability in the development of hemolytic crisis, and several factors influence it: quantity, quality, ripeness of fava beans, and age of onset. In this narrative review of case reports and case series, we reported the predisposing factors and clinical features for four different age groups classified as follows: pregnant women and infants (i.e., exclusively breastfed children); children, from weaned to 11 years; preadolescents and adolescents, from 11 to 18 years; and adults (18 years and older). Some symptoms developed only in specific age groups: death in infants; visual impairment in children; systolic murmur in infants, children, and adolescents; and renal failure in adults. In youngest children or pregnant women the severity is the highest. Some other symptoms were present in all: jaundice, increased bilirubin, splenomegaly, hepatomegaly, discolored urine, tachycardia, pallor, abdominal pain, malaise, vomit, nausea, and dizziness. Laboratory findings are characterized by anemia, reticulocytosis, elevated bilirubin level, and sometimes urinary urobilinogen and methemoglobinemia. In most cases the symptomatology is self-limited and does not release sequelae, but hospitalization and transfusion are often required.
Topics: Pregnancy; Child; Infant; Adolescent; Adult; Humans; Female; Favism; Glucosephosphate Dehydrogenase Deficiency; Vicia faba; Hemolysis; Bilirubin
PubMed: 36678214
DOI: 10.3390/nu15020343 -
Disease Markers 2015Several hemolytic markers are available to guide the differential diagnosis and to monitor treatment of hemolytic conditions. They include increased reticulocytes, an... (Review)
Review
Several hemolytic markers are available to guide the differential diagnosis and to monitor treatment of hemolytic conditions. They include increased reticulocytes, an indicator of marrow compensatory response, elevated lactate dehydrogenase, a marker of intravascular hemolysis, reduced haptoglobin, and unconjugated hyperbilirubinemia. The direct antiglobulin test is the cornerstone of autoimmune forms, and blood smear examination is fundamental in the diagnosis of congenital membrane defects and thrombotic microangiopathies. Marked increase of lactate dehydrogenase and hemosiderinuria are typical of intravascular hemolysis, as observed in paroxysmal nocturnal hemoglobinuria, and hyperferritinemia is associated with chronic hemolysis. Prosthetic valve replacement and stenting are also associated with intravascular and chronic hemolysis. Compensatory reticulocytosis may be inadequate/absent in case of marrow involvement, iron/vitamin deficiency, infections, or autoimmune reaction against bone marrow-precursors. Reticulocytopenia occurs in 20-40% of autoimmune hemolytic anemia cases and is a poor prognostic factor. Increased reticulocytes, lactate dehydrogenase, and bilirubin, as well as reduced haptoglobin, are observed in conditions other than hemolysis that may confound the clinical picture. Hemoglobin defines the clinical severity of hemolysis, and thrombocytopenia suggests a possible thrombotic microangiopathy or Evans' syndrome. A comprehensive clinical and laboratory evaluation is advisable for a correct diagnostic and therapeutic workup of the different hemolytic conditions.
Topics: Anemia, Hemolytic, Autoimmune; Biomarkers; Diagnosis, Differential; Erythrocyte Count; Hemolysis; Humans
PubMed: 26819490
DOI: 10.1155/2015/635670 -
The Western Journal of Medicine Mar 1975The significance of erythroblastemia must be considered in the context of the clinical setting in which it is found. Interpretation should take into account the number...
The significance of erythroblastemia must be considered in the context of the clinical setting in which it is found. Interpretation should take into account the number and spectrum of maturity of the nucleated red cells; the presence or absence of reticulocytosis and other red cell abnormalities (for example, poikilocytosis); the presence or absence of anemia; the presence or absence of circulating immature granulocytes or bizarre platelets and the presence or absence of the spleen. Circulating nucleated red cells indicate intravascular hemopoiesis or disruption of marrow structure or the inability of the bone marrow's screen mechanism to prevent their passage into circulation. In the latter situations, it usually indicates an unfavorable prognosis.
Topics: Anemia, Hemolytic; Anemia, Hypochromic; Bone Diseases; Bone Marrow; Bone Marrow Cells; Erythroblasts; Graft Rejection; Heart Failure; Hematologic Diseases; Hematopoietic System; Humans; Infections; Kidney Transplantation; Prognosis; Transplantation, Homologous
PubMed: 1096474
DOI: No ID Found -
Case Reports in Hematology 2013Drug-induced immune hemolytic anemia is a rare condition with an incidence of 1 per million of the population. We report the case of a 36-year-old female who presented...
Drug-induced immune hemolytic anemia is a rare condition with an incidence of 1 per million of the population. We report the case of a 36-year-old female who presented to the emergency department complaining of shortness of breath and dark colored urine. Physical examination was significant for pale mucous membranes. The patient reported using ibuprofen for a few days prior to presentation. Complete blood count performed before starting ibuprofen revealed normal platelets and hemoglobin values. On admission, the patient had evidence of hemolytic anemia with hemoglobin of 4.9 g/dL, hematocrit of 14.2%, lactate dehydrogenase 435 IU/L, and reticulocytosis 23.2%. Further testing ruled out autoimmune disease, lymphoma, and leukemia as etiologies for the patient's new onset hemolytic anemia. Ibuprofen was immediately stopped with a gradual hematologic recovery within 3 days.
PubMed: 23710383
DOI: 10.1155/2013/142865 -
BMC Pediatrics Nov 2022Screening for G6PD deficiency in newborns can help prevent severe hemolysis, hyperbilirubinemia, and bilirubin encephalopathy, as recommended by the World Health...
BACKGROUND
Screening for G6PD deficiency in newborns can help prevent severe hemolysis, hyperbilirubinemia, and bilirubin encephalopathy, as recommended by the World Health Organization (WHO). It has been speculated that the presence of a high number of reticulocytes in newborns interferes with the diagnosis of G6PD deficiency since reticulocytes contain higher amounts of G6PD enzyme than mature erythrocytes. Therefore, the purposes of this study were to assess the effect of reticulocytosis in the determination of blood G6PD activity in Thai newborns by using a novel automated UV-based enzymatic assay and to validate the performance of this assay for the detection of G6PD deficiency in newborn samples.
METHODS
The levels of reticulocytes and G6PD activity were measured in blood samples collected from 1,015 newborns. G6PD mutations were identified using TaqMan SNP genotyping assay, PCR-restriction fragment length polymorphism (PCR-RFLP), and direct sequencing. The correlation between the levels of reticulocytes and G6PD activity was examined. The performance of the automated method was compared with that of the fluorescent spot test (FST) and the standard quantitative assay.
RESULTS
The automated assay detected G6PD deficiency in 6.5% of the total newborn subjects compared to 5.3% and 6.1% by the FST and the standard method, respectively. The minor allele frequencies (MAFs) of G6PD Viangchan, G6PD Mahidol, and G6PD Union were 0.066, 0.005, and 0.005, respectively. The reticulocyte counts in newborns with G6PD deficiency were significantly higher than those in normal male newborns (p < 0.001). Compared with normal newborns after controlling for thalassemias and hemoglobinopathies, G6PD-deficient patients with the G6PD Viangchan mutation exhibited elevated reticulocyte counts (5.82 ± 1.73%, p < 0.001). In a group of G6PD normal newborns, the percentage of reticulocytes was positively correlated with G6PD activity (r = 0.327, p < 0.001). However, there was no correlation between G6PD activity and the levels of reticulocytes in subjects with G6PD deficiency (r = -0.019, p = 0.881). The level of agreement in the detection of G6PD deficiency was 0.999, while the area under the receiver operating characteristic (AUC) curve demonstrated that the automated method had 98.4% sensitivity, 99.5% specificity, 92.4% positive predictive value (PPV), 99.9% negative predictive value (NPV), and 99.4% accuracy.
CONCLUSIONS
We report that reticulocytosis does not have a statistically significant effect on the detection of G6PD deficiency in newborns by both qualitative and quantitative methods.
Topics: Infant, Newborn; Humans; Male; Glucosephosphate Dehydrogenase Deficiency; Reticulocytosis; Cross-Sectional Studies; Phosphates; Glucose
PubMed: 36419023
DOI: 10.1186/s12887-022-03740-1