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Blood Jun 2000The membrane skeleton, a dynamic network of proteins associated with the plasma membrane, determines the shape and mechanical properties of erythrocytes. Deficiencies or...
The membrane skeleton, a dynamic network of proteins associated with the plasma membrane, determines the shape and mechanical properties of erythrocytes. Deficiencies or defects in membrane skeletal proteins are associated with inherited disorders of erythrocyte morphology and function. Adducin is one of the proteins localized at the spectrin-actin junction of the membrane skeleton. In this work we show that deficiency of beta-adducin produces an 80% decrease of alpha-adducin and a fourfold up-regulation of gamma-adducin in erythrocytes. beta-Adducin or any other isoform generated by translation of abnormally spliced messenger RNAs could not be detected by our antibodies either in ghosts or in cytoplasm of -/- erythrocytes. Actin levels were diminished in mutant mice, suggesting alterations in the actin-spectrin junctional complexes due to the absence of adducin. Elliptocytes, ovalocytes, and occasionally spherocytes were found in the blood film of -/- mice. Hematological values showed an increase in reticulocyte counts and mean corpuscular hemoglobin concentration, decreased mean corpuscular volume and hematocrit, and normal erythrocyte counts that, associated to splenomegaly, indicate that the mice suffer from mild anemia with compensated hemolysis. These modifications are due to a loss of membrane surface and dehydration that result in an increase in the osmotic fragility of red blood cells. The marked alteration in osmotic fragility together with the predominant presence of elliptocytes is reminiscent of the human disorder called spherocytic hereditary elliptocytosis. Our results suggest that the amount of adducin remaining in the mutant animals (presumably alphagamma adducin) could be functional and might account for the mild phenotype. (Blood. 2000;95:3978-3985)
Topics: Animals; Calmodulin-Binding Proteins; Chimera; Crosses, Genetic; Cytoskeletal Proteins; Elliptocytosis, Hereditary; Erythrocytes; Female; Hematocrit; Hemoglobins; Heterozygote; Humans; Male; Mice; Mice, Inbred C57BL; Mice, Knockout; Osmotic Fragility; Reticulocyte Count
PubMed: 10845937
DOI: No ID Found -
North American Journal of Medical... Oct 2011Schistocytes are fragmented red blood cells due to the flow of blood through damaged capillaries and indicate endothelial injury. They are typical of microangiopathic...
CONTEXT
Schistocytes are fragmented red blood cells due to the flow of blood through damaged capillaries and indicate endothelial injury. They are typical of microangiopathic hemolytic anemia seen in life threatening conditions like disseminated intravascular coagulation or thrombotic thrombocytopenic purpura/hemolytic uremic syndrome .We report a rare sub-acute presentation of pernicious anemia with hemolysis, thrombocytopenia and numerous schistocytes that was initially diagnosed as a more serious thrombotic thrombocytopenic purpura.
CASE REPORT
A 31-year-old Caucasian woman presented with fatigue and paresthesia of both feet for 1 week. Past medical history included hypertension and gastro-esophageal reflux disease. Examination revealed scleral icterus and pallor. Examination of the abdomen did not show hepatosplenomegaly. Initial laboratory tests showed severe anemia, and low platelets. Indirect bilirubin and serum Lactate De Hydrogenase were elevated. Prothrombin time, partial thromboplastin time, serum fibrinogen, and serum fibrin degradation product levels were normal. Peripheral smear revealed numerous schistocytes, anisocytosis and macro-ovalocytes. Thrombotic thrombocytopenic purpura (TTP) was suspected due to the constellation of sub-acute onset of fatigue and paresthesia along with thrombocytopenia, schistocytes and an elevated LDH. Plasmapheresis was initiated for possible TTP. However, platelet count worsened despite plasmapheresis for 4 days. On re-evaluation, vitamin B(12) was found to be low. Treatment with intra-muscular vitamin B(12) led to symptomatic and hematologic improvement. Pernicious anemia was confirmed by the presence of anti-intrinsic factor antibodies, elevated serum gastrin level and atrophic gastritis.
CONCLUSION
Clinicians must be aware of unusual clinical presentation of vitamin B(12) deficiency with schistocytes as the management is simple and effective.
PubMed: 22363087
DOI: 10.4297/najms.2011.3472 -
Pediatric Nephrology (Berlin, Germany) Dec 2017A previously healthy 15-year-old girl was evaluated following five episodes of reddish urine discoloration after walking for approximately 30 min on a smooth roadway....
A previously healthy 15-year-old girl was evaluated following five episodes of reddish urine discoloration after walking for approximately 30 min on a smooth roadway. In each episode, the discoloration lasted for four to five urinations and followed by normal urine dipstick tests. No other exercise-produced urine discoloration and no other symptoms were reported. Laboratory evaluation during the episodes revealed a reddish urine sample with 3+ hemoglobin/myoglobin and absence of hematuria. Full blood count, serum creatinine, liver function tests, and electrolyte levels were all within normal limits. Myoglobulinuria was excluded, since muscle enzymes were within normal limits. Blood smear analysis showed mild anisopoikilocytosis with stomatocytes and ovalocytes, leading to extended evaluation for erythrocyte disorders. This case is interesting in that the hemoglobinuria occurred after mild walking and was accompanied by erythrocyte morphological changes. This quiz discusses the differential diagnosis of hemoglobinuria with particular reference to the conditions of appearance (after walking) and emphasizes the importance of step-by-step investigations to reach a definitive diagnosis.
Topics: Adolescent; Diagnosis, Differential; Female; Hemoglobins; Hemoglobinuria; Humans; Urine; Walking
PubMed: 28194571
DOI: 10.1007/s00467-017-3596-5 -
International Journal of Hematology Oct 2005The purposes of this study were to evaluate the reliability of the previously described diagnostic criteria for Southeast Asian ovalocytosis (SAO) in adults in the...
The purposes of this study were to evaluate the reliability of the previously described diagnostic criteria for Southeast Asian ovalocytosis (SAO) in adults in the diagnosis of SAO in newborns and to describe the role of SAO in newborn infants presenting with pallor and jaundice. The inclusion criteria in this retrospective descriptive study were that the patient be a newborn with pallor or jaundice and with ovalocytes in the peripheral blood smear (PBS). The exclusion criteria were newborn status with other causes of neonatal hemolysis or anemia. Controls were age-matched newborn infants who did not have SAO or other causes of neonatal anemia or hemolysis. Hematological data were assessed with a hematology analyzer. DNA analysis for SAO band 3 was done by polymerase chain reaction. Among 107 newborn infants with SAO, 30 infants were excluded from the study. The exclusions were premature infants, an infant with congenital syphilis, low-birth-weight infants, infants with ABO blood group incompatibility, infants with 3-thalassemia, infants with hemoglobin E heterozygote or homozygotes, glucose-6-phosphate dehydrogenase-deficient infants, and infants with fetomaternal hemorrhage. The DNA analysis for SAO band 3 was done in 56 newborns, and 54 had positive results for SAO band 3 gene deletion. Approximately one half of the 54 newborn infants with SAO had hyperbilirubinemia, and 3 had severe hyperbilirubinemia. The mean hemoglobin concentration, packed cell volume, and red blood cell (RBC) count in the infants with SAO in the first week of life were significantly lower than those in control infants. The mean absolute number of reticulocytes, mean corpuscular hemoglobin, and red cell volume distribution width in infants with SAO band 3 in the first week of life were significantly higher than those in control infants. The neonatal diagnosis of SAO can be made by examination of RBC morphology in the PBS with the presence of stomatocytes, theta cells, and > or = 25% ovalocytes. SAO plays a role in anemia and hyperbilirubinemia in newborn infants.
Topics: Anemia, Neonatal; Asia, Southeastern; Elliptocytosis, Hereditary; Female; Gene Deletion; Humans; Infant, Newborn; Jaundice, Neonatal; Male; Reticulocyte Count; Retrospective Studies
PubMed: 16207591
DOI: 10.1532/IJH97.A20505 -
The American Journal of Medical... Mar 1976An extensive study was undertaken to compare five methods of blood smear preparation. Three mechanical devices for preparing blood smears were compared to the...
An extensive study was undertaken to compare five methods of blood smear preparation. Three mechanical devices for preparing blood smears were compared to the traditional coverslip and manual wedge methods. A series of five slides was prepared from each sample of blood. One hundred-cell leukocyte differential counts, red cell morphology, and platelet distribution were compared. Differential counts along the axis and margins of each slide failed to reveal any significant distribution difference by method. On the other hand, there were a significantly fewer neutrophils as well as higher lymphocyte counts on the two spinner preparations. Subsequent analysis suggested that spinner preparations create more smudge cells in smears of normal and granulocytic leukemia patients, and significantly fewer smudge cells in smears of lymphocytic leukemia patients. Both spinner methods produced significant increases in erythrocytic target cells, spherocytes, and cells with shifted pallor; and significantly fewer burr cells and ovalocytes, as compared to the reference methods. The mechanical wedge device was most similar to both reference methods. Of the two reference methods, the cumbersome coverslip preparation apparently offers no practical advantage over wedge methods.
Topics: Analysis of Variance; Anemia; Anemia, Sickle Cell; Blood Cell Count; Erythrocytes, Abnormal; Humans; Infections; Leukemia
PubMed: 1258894
DOI: No ID Found -
Journal of the Medical Association of... Mar 1989Ovalocytic red blood cells were significantly increased in the peripheral blood films of patients infected with P. falciparum (mean +/- S.D. = 6.3 +/- 8.4%) and P. vivax...
Ovalocytic red blood cells were significantly increased in the peripheral blood films of patients infected with P. falciparum (mean +/- S.D. = 6.3 +/- 8.4%) and P. vivax (8.3 +/- 14.0%), whereas, there was only 0.6 +/- 0.4 per cent ovalocytes in normal individuals. Per cent parasitemia of ovalocytes counted at least 100 ovalocytes of the blood films of malaria infected individuals was significantly reduced when compared with that of discocytes which revealed at least 100 discocytes in the same blood smears. Relative increase in ovalocyte and their low parasitemia might be the response of malaria infected individuals to cope with malaria multiplication in their circulation.
Topics: Animals; Erythrocyte Count; Erythrocytes; Humans; Malaria; Plasmodium falciparum; Plasmodium vivax; Retrospective Studies
PubMed: 2661709
DOI: No ID Found -
Veterinary Clinical Pathology 1994Medical records from 39 cats with hepatic disease, examined at the Veterinary Medical Teaching Hospital, University of Florida, between 1987 and 1992 were...
Medical records from 39 cats with hepatic disease, examined at the Veterinary Medical Teaching Hospital, University of Florida, between 1987 and 1992 were retrospectively evaluated for alterations in red blood cell (RBC) morphology. Diagnoses included: hepatic lipidosis, neoplasia, cholangiohepatitis, hepatitis/hepatopathy, systemic histoplasmosis, and portocaval shunt. A total of 56 laboratory data sets were studied which included complete blood counts and serum chemistry results. Stained blood smears were evaluated from 51 of the data sets. Twenty-two cats (56%) were determined to have poikilocytosis on the basis of blood smear evaluation. Eleven (28%) cats had moderate to marked poikilocytosis (2+ to 4+). Acanthocytes accounted for 62.6 -/+ 22.1% of morphologically abnormal RBC and were observed in blood smears from 100% of cats with poikilocytosis. Elliptocytes (ovalocytes) comprised 19.5 -/+ 15.8% of poikilocytes and were found in smears from 82% of cats with poikilocytosis. Keratocytes (7.0 -/+ 6.8%), schistocytes (3.6 -/+ 4.4%), and blister cells (2.6 -/+ 6.4%) were present in lower numbers and in fewer cats. Serum total cholesterol values were significantly greater (p < 0.05) in cats with moderate to marked alterations in RBC morphology. Cats with hepatic lipidosis were significantly (p < 0.04) more likely to have poikilocytosis than cats with other types of hepatic disease.
PubMed: 12666032
DOI: 10.1111/j.1939-165x.1994.tb01007.x -
Annals of Clinical and Laboratory... 1988Blood is drawn into capillary tubes containing saponin and the tubes placed into the reagent packs. Hemoglobin is denatured by mixing the hemosylate with a reagent...
Blood is drawn into capillary tubes containing saponin and the tubes placed into the reagent packs. Hemoglobin is denatured by mixing the hemosylate with a reagent containing lithium hydroxide and a non-ionic detergent. The absorbance is measured bichromatically at wavelengths of 577 and 633 nm. The calibration curve is stable and can be stored for at least 30 days. There are no interferences from fetal hemoglobin, glycosylated hemoglobin (20 percent), hemoglobin S, samples with hematocrits up to 0.55, paraproteins, and lipemia. Specimens with rouleau formation, nucleated and fragmented red blood cells, target cells, ovalocytes, teardrop cells, spherocytes, leukocyte counts of 29 X 10(9) per L and reticulocyte counts of 0.32; Howell-Jolly bodies did not interfere with the assay. The within run and between run precision gave average coefficient or variations of 2.3 and 1.9 percent, respectively. Comparison of the hemoglobin results obtained in 149 samples with the Vision (y) and Coulter Counter System (x) gave r = 0.987, Y = 1.01X - 1.89 g per L.
Topics: Alkalies; Calibration; Hemin; Hemoglobinometry; Hemoglobins; Humans; Hydrogen-Ion Concentration
PubMed: 3382158
DOI: No ID Found -
British Journal of Haematology Jun 1998Southeast-Asian ovalocytosis (SAO) was diagnosed in children from Madang, Papua New Guinea, by detection of the SAO band 3 gene variant using the polymerase chain...
Southeast-Asian ovalocytosis (SAO) was diagnosed in children from Madang, Papua New Guinea, by detection of the SAO band 3 gene variant using the polymerase chain reaction. SAO band 3 was present in 16/241 (6.6%) children living in the community and 32/389 (8.2%) children with acute Plasmodium falciparum malaria (P=0.42). SAO band 3 was detected in 8.2% (23/281) of alpha+-thalassaemia homozygotes, 9.4% (20/214) of heterozygotes and 2.4% (2/85) of children with a normal alpha-globin genotype (P=0.12). The most consistent feature of SAO band 3 on microscopy of thin blood films was red cells with two or more linear or irregularly-shaped pale regions. In children living in the community, these were present in 15 with SAO band 3 (sensitivity 93.8%) and only two normals (specificity 99.1%). The presence of > or = 20% ovalocytosis was a poorer indicator of SAO band 3 (sensitivity 68.8% and specificity 100%). Haematological data were similar in SAO band 3 and normal children. However, in children with acute malaria, haemoglobin levels and red cell counts were significantly lower in SAO band 3 than normal children. The degree of ovalocytosis was lower in children with SAO band 3 during acute malaria, suggesting that a selective loss of ovalocytes may contribute to malaria anaemia in Southeast-Asian ovalocytosis.
Topics: Anemia; Anion Exchange Protein 1, Erythrocyte; Case-Control Studies; Child; Child, Preschool; Elliptocytosis, Hereditary; Erythrocytes, Abnormal; Humans; Malaria, Falciparum; Papua New Guinea; Parasitemia; Prospective Studies; alpha-Thalassemia
PubMed: 9633878
DOI: 10.1046/j.1365-2141.1998.00742.x