We present two cases of HS treated by subtotal splenectomy, alongside the advantages and disadvantages of this therapy. [ncbi.nlm.nih.gov]

A structural abnormality of spectrin is present in which RBCs fragment when heated to only 45°C. Normal RBCs fragment at 49°C. [med-ed.virginia.edu]

PPLP2-positive vesicular structures are present in the cytoplasm of trophozoites (TZ) and schizonts (SZ). [phenoplasm.org]

Hereditary ElliptocytosisClinical presentation depends upon degreeof membrane defect.Asymptomatic diagnosed on blood film.Chronic compensated hemolytic state. 21. [slideshare.net]

Harvard Medical School, Boston, Massachusetts, USA Published online: January 2006 DOI: 10.1038/npg.els.0006094 Full Article on Wiley Online Library Abstract Erythrocyte membrane disorders, such as hereditary spherocytosis and elliptocytosis, usually present [els.net]

• Anemia

  Anemia, Hemolytic, Congenital/blood* Anemia, Hemolytic, Congenital/complications Anemia, Hemolytic, Congenital/surgery Elliptocytosis, Hereditary/blood* Elliptocytosis, Hereditary/complications Elliptocytosis, Hereditary/surgery Erythrocyte Membrane/ [ncbi.nlm.nih.gov]

  anemia associated with ulcerative colitis Hemolytic anemia, hereditary Hemolytic anemia, lymphoproliferative disease Hemolytic anemia, with ulcerative colitis Hereditary hemolytic anemia Clinical Information A congenital hemolytic anemia caused by defects [icd10data.com]

  Hemoglobinopathies-defective globin synthesis Thalassemias - decreased or absent chain production Membrane structural defects enzyme defects in rbc metabolism Acquired hemolytic anemias Paroxysmal nocturnal hemoglobinuria Destruction anemias caused by [quizlet.com]

  Also recall that iron deficiency anemia causes microcytosis, and thus iron deficiency anemia causes hypochromic, microcytic anemia. [theartofmed.wordpress.com]

• Inflammation

  Influence of particle surface area on inflammation and clearance. Inhal Toxicol 2000; 12: 1113-1126. Abstract Views: 116 PDF Views: 0 [i-scholar.in]

  Some cases of chronic inflammation Polychromasia Polychromasia is a medical condition in which there is an abnormally high amount of immature red blood cells being released into the bloodstream. [theartofmed.wordpress.com]

• Swelling

  PBS hereditary spherocytosis spherocytes stomatocytes dimorphic smear -polychromaphillic cells and spherocytes Lab test: osmotic fragility test PLACE RBC IN INDIVIDUAL TUBES each tube has a lower concentration of NaCl RBC on hypotonic solutions will swell [quizlet.com]

  Osmosis leads to the red blood cell having a constant tendency to swell and burst. This tendency is countered by manipulating the flow of sodium and potassium ions. [theartofmed.wordpress.com]

• Fatigue

  Some patients remain asymptomatic until adulthood, others may present moderate anemia (8-10g/dl) pallor, jaundice, fatigue, splenomegaly, gallstones. Sometimes, the only sign of HS in childhood is the presence of splenomegaly. [ncbi.nlm.nih.gov]

  Beyond the neonatal period, jaundice is intermittent and is associated with fatigue, cold exposure, emotional distress, or pregnancy. [oncohemakey.com]

• Family History of Anemia

  Hereditary SpherocytosisDiagnosis– Must be distinguished primarily from thespherocytic hemolytic anemias associated withRBC antibodies.– If present, family history of anemia and/orsplenectomy.– Immune spherocytosis  positive directCoombs test. 15. [slideshare.net]

• Ulcer

  […] disease Hemolytic anemia, with ulcerative colitis Hereditary hemolytic anemia Clinical Information A congenital hemolytic anemia caused by defects of the erythrocyte membrane, enzyme deficiencies, or hemoglobinopathies. [icd10data.com]

  Three of the affected family members had recurrent leg ulcers, and two had undergone cholecystectomy for recurrent gallstones. Osmotic fragility was determined in all the family members studied. [nejm.org]

• Coombs Negative Hemolytic Anemia

  Deficiency of protein 4.2 in erythrocytes from a patient with a Coombs negative hemolytic anemia: evidence for a role of protein 4.2 in stabilizing ankyrin on the membrane. J Clin Invest 1988 ; 81: 893 –901. 13. [nejm.org]

Treatment of hereditary spherocytosis with splenectomy is curative in most patients. Growing recognition of the long-term risks of splenectomy has led to re-evaluation of the role of splenectomy. [ncbi.nlm.nih.gov]

[…] mild anemia if anemia at all compensated by increased erythropoieisis as in spherocytosis young cells and reticulocytes are normal in shape ....elliptocytes formed during circulation sometimes more severe -poikilocytosis Osmotic fragility test- normal treatment [quizlet.com]

Hereditary SpherocytosisTreatment– Acute, severe hemolytic crises requirestransfusion. 18. Hereditary ElliptocytosisHeterogeneous disorders in elliptocytic redcells. [slideshare.net]

We have investigated the fatty acid composition of red blood cells and the effect of treatment with n-3 fatty acids on blood parameters. [londonmet.ac.uk]

While it seems likely that some of these play an important role in sickle cell disease (SCD) pathophysiology, their etiology remains obscure. This application is divided into three interrelated projects. [grantome.com]

A contribution to etiology, genetics, and diagnosis. Bosman, G.J., Bartholomeus, I.G., De Grip, W.J., Horstink, M.W. Brain Res. [wikigenes.org]

In our study, only 1 out of 11 (9%) had ademonstrable deficiency of spectrin but 4 out of 11 (36%) had ankyrin deficiency, which appeared to be one of the two most important etiological factors with band 3. [annsaudimed.net]

Thirty percent to 50% of adult HS patients report a history of neonatal jaundice and the diagnosis of HS is prominent in etiologic studies of severe neonatal hyperbilirubinemia. 70, 71 The magnitude of hyperbilirubinemia may be severe, requiring phototherapy [oncohemakey.com]

Relevant External Links for EPB41L1 Genetic Association Database (GAD) EPB41L1 Human Genome Epidemiology (HuGE) Navigator EPB41L1 Atlas of Genetics and Cytogenetics in Oncology and Haematology: EPB41L1 No data available for Genatlas for EPB41L1 Gene Excess [genecards.org]

Daak AA, Elsamani E, Ali EH, Mohamed FA, Abdel-Rahman ME, Elderdery AY, Talbot O, Kraft P, Ghebremeskel K, Elbashir MI, Fawzi W (2016) Sickle cell disease in western Sudan: genetic epidemiology and predictors of knowledge attitude and practices. [londonmet.ac.uk]

While it seems likely that some of these play an important role in sickle cell disease (SCD) pathophysiology, their etiology remains obscure. This application is divided into three interrelated projects. [grantome.com]

RBC deformability can be significantly altered by various pathophysiological conditions, and the alterations in RBC deformability in turn influence pathophysiology, since RBC deformability is an important determinant of blood viscosity and thus blood [intechopen.com]

Erythrocytes, Abnormal with chemical compounds BACKGROUND: Solute(s) retained during uraemia cause increased exposure of aminophospholipid phosphatidylserine (PS) on the outer surface of erythrocyte membranes, and this phenomenon may be involved in the pathophysiology [wikigenes.org]

Pathophysiology of hereditary spherocytosis (HS). The primary defect in HS is a deficiency of membrane surface area, leading to the formation of spherocytes. [oncohemakey.com]

Peters LL, Shivdasani RA, Liu SC, et al. (1996) Anion exchanger 1 (band 3) is required to prevent erythrocyte membrane surface loss but not to form the membrane skeleton. Cell 86: 917–927. [els.net]

Study of a kindred with seven deaths in infancy: Value of riboflavin therapy in preventing this syndrome. Harpey, J.P., Charpentier, C., Goodman, S.I., Darbois, Y., Lefèbvre, G., Sebbah, J. J. [wikigenes.org]

Daak A, Ghebremeskel K, Clough P, Elbashir M, Attalla B, Hassan Z and Crawford MA (2010) The effect of omega 3 fatty acids on prevention of vaso-occlusive crisis in homozygous sickle cell disease. 9th International Meeting for the Study of Fatty acids [londonmet.ac.uk]