scholarly journals ALTERATIONS IN OSMOTIC AND MECHANICAL FRAGILITY RELATED TO IN VIVO ERYTHROCYTE AGING AND SPLENIC SEQUESTRATION IN HEREDITARY SPHEROCYTOSIS*†

1960 ◽  
Vol 39 (1) ◽  
pp. 89-101 ◽  
Author(s):  
Robert C. Griggs ◽  
Russell Weisman ◽  
John W. Harris
Keyword(s):  
Hereditary Spherocytosis ◽  
Splenic Sequestration ◽  
Erythrocyte Aging

scholarly journals Recurrent acute splenic sequestration crisis due to interacting genetic defects: hemoglobin SC disease and hereditary spherocytosis

Blood ◽  
1990 ◽  
Vol 75 (1) ◽  
pp. 266-270 ◽  
Author(s):  
TE Warkentin ◽  
RD Barr ◽  
MA Ali ◽  
N Mohandas
Keyword(s):  
Hereditary Spherocytosis ◽  
Genetic Defects ◽  
Mechanical Instability ◽  
Splenic Sequestration ◽  
Alpha Thalassemia ◽  
Unusual Occurrence ◽  
Distinguishing Features ◽  
Hemoglobin Sc Disease ◽  
Reduced Surface ◽  
In Cells

Abstract A 14-year-old boy with hemoglobin SC disease and alpha-thalassemia-2 experienced five episodes of acute splenic sequestration crisis (ASSC), while two of his siblings with identical globin genotypes (SC and - alpha/alpha alpha) had no such experience. To determine if an additional red blood cell (RBC) defect was responsible for the unusual occurrence of frequent ASSCs, we performed detailed rheologic characterization and membrane protein analysis on RBCs from the proband and other members of his family. Reduced surface area, increased mechanical instability, and decreased spectrin content of the membrane, distinguishing features of RBCs in hereditary spherocytosis, were observed in cells from the proband and his mother, but not in cells from other family members. These findings are consistent with the dominant inheritance of spherocytosis by the proband. We suggest that the combined effects of SC disease and spherocytosis in the proband resulted in decreased RBC deformability and led to increased splenic trapping, intrasplenic sickling, and consequently, recurrent sequestration crisis. Marked clinical and hematologic improvement occurred from splenectomy. Thus, inheritance of interacting genetic defects, sickling hemoglobinopathy, and hereditary spherocytosis appear to be responsible for the unusual clinical manifestation of recurrent ASSC in this patient.

Splenic sequestration associated with sickle cell trait and hereditary spherocytosis

1992 ◽  
Vol 40 (2) ◽  
pp. 110-116 ◽  
Author(s):  
Yih-Ming Yang ◽  
Cynthia Donnell ◽  
Walter Wilborn ◽  
Steven R. Goodman ◽  
Bea Files ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Hereditary Spherocytosis ◽  
Sickle Cell Trait ◽  
Splenic Sequestration

scholarly journals Leukocyte Transfusions: Function of Transfused Granulocytes from Donors with Chronic Myelocytic Leukemia

Blood ◽  
1970 ◽  
Vol 36 (4) ◽  
pp. 432-442 ◽  
Author(s):  
HARMON J. EYRE ◽  
IRA M. GOLDSTEIN ◽  
SEYMOUR PERRY ◽  
ROBERT G. GRAW
Keyword(s):  
Severe Neutropenia ◽  
Chronic Myelocytic Leukemia ◽  
Phagocytic Capacity ◽  
Splenic Sequestration ◽  
Myelocytic Leukemia ◽  
The Poor ◽  
Transfusion Reactions

Abstract The in vivo function of chronic myelocytic leukemia (CML) leukocytes transfused into infected patients with severe neutropenia was evaluated and the results in four representative patients are reported. The intravascular survival, extravascular migration, and phagocytic capacity of these cells appeared normal in two patients without preformed leukoagglutinins. In two other patients who had only small transient increments in circulating granulocytes and severe transfusion reactions, preformed leukoagglutinins were found. The poor granulocyte recoveries in these patients with antibody could probably be explained by splenic sequestration of the transfused cells. These studies provide evidence supporting the use of CML leukocyte transfusions in patients without preformed leukocyte antibodies.

Comparative proteomics of erythrocyte aging in vivo and in vitro

2010 ◽  
Vol 73 (3) ◽  
pp. 396-402 ◽  
Author(s):  
G.J.C.G.M. Bosman ◽  
E. Lasonder ◽  
Y.A.M. Groenen-Döpp ◽  
F.L.A. Willekens ◽  
J.M. Werre ◽  
...  
Keyword(s):  
Comparative Proteomics ◽  
Erythrocyte Aging

scholarly journals Mechanics of diseased red blood cells in human spleen and consequences for hereditary blood disorders

2018 ◽  
Vol 115 (38) ◽  
pp. 9574-9579 ◽  
Author(s):  
He Li ◽  
Lu Lu ◽  
Xuejin Li ◽  
Pierre A. Buffet ◽  
Ming Dao ◽  
...  
Keyword(s):  
Hereditary Spherocytosis ◽  
Severe Disease ◽  
Blood Disorders ◽  
Pathogenic Role ◽  
Human Spleen ◽  
Two Component ◽  
Hereditary Blood Disorders ◽  
Unique Ability

In red blood cell (RBC) diseases, the spleen contributes to anemia by clearing the damaged RBCs, but its unique ability to mechanically challenge RBCs also poses the risk of inducing other pathogenic effects. We have analyzed RBCs in hereditary spherocytosis (HS) and hereditary elliptocytosis (HE), two typical examples of blood disorders that result in membrane protein defects in RBCs. We use a two-component protein-scale RBC model to simulate the traversal of the interendothelial slit (IES) in the human spleen, a stringent biomechanical challenge on healthy and diseased RBCs that cannot be directly observed in vivo. In HS, our results confirm that the RBC loses surface due to weakened cohesion between the lipid bilayer and the cytoskeleton and reveal that surface loss may result from vesiculation of the RBC as it crosses IES. In HE, traversing IES induces sustained elongation of the RBC with impaired elasticity and fragmentation in severe disease. Our simulations thus suggest that in inherited RBC disorders, the spleen not only filters out pathological RBCs but also directly contributes to RBC alterations. These results provide a mechanistic rationale for different clinical outcomes documented following splenectomy in HS patients with spectrin-deficient and ankyrin-deficient RBCs and offer insights into the pathogenic role of human spleen in RBC diseases.

scholarly journals Red Cell Filtration and the Pathogenesis of Certain Hemolytic Anemias

Blood ◽  
1961 ◽  
Vol 18 (2) ◽  
pp. 133-148 ◽  
Author(s):  
JAMES H. JANDL ◽  
RICHARD L. SIMMONS ◽  
WILLIAM B. CASTLE
Keyword(s):  
Perfusion Pressure ◽  
Hereditary Spherocytosis ◽  
Red Cells ◽  
Red Cell ◽  
Low Oxygen ◽  
Millipore Filter ◽  
Minor Population ◽  
Membrane Type ◽  
Pass Through

Abstract Studies were made of the filterability of normal and abnormal red cells through an inert membrane-type (Millipore) filter having uniform non-branching capillary pores 5 µ in diameter. With this filter it was possible to remove selectively certain kinds of abnormal red cells from mixed suspensions of normal and abnormal red cells. The red cells of patients with sickle cell anemia were selectively trapped by the filter under low oxygen tensions. Such sickled red cells were unable to pass through the filter even under a pressure differential across the filter (perfusion pressure) of over 120 mm. Hg. Red cells agglutinated by anti-A were completely retained by the filter at perfusing pressures of 4 mm. Hg., but were partially disagglutinated and forced through the filter when the pressure was increased. Red cells coated with an incomplete (Rh) agglutinin and red cells from a patient with acquired hemolytic anemia were retained by the filter only in the presence of rouleaux-producing agents such as P.V.P. or fibrinogen and only at low perfusion pressures. A small fraction of the red cells of patients with hereditary spherocytosis were trapped by a single passage through the filter; the trapped cells were largely derived from the minor population of hyperspheroidal cells. The relevance of these findings to the mechanisms of red cell sequestration in vivo is discussed.

scholarly journals Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin/ankyrin- and band 3–deficient hereditary spherocytosis

Blood ◽  
2002 ◽  
Vol 100 (6) ◽  
pp. 2208-2215 ◽  
Author(s):  
Ramune Reliene ◽  
Mariagabriella Mariani ◽  
Alberto Zanella ◽  
Walter H. Reinhart ◽  
M. Leticia Ribeiro ◽  
...  
Keyword(s):  
Hereditary Spherocytosis ◽  
Band 3 ◽  
Vesicle Budding ◽  
Naturally Occurring ◽  
Cell Age ◽  
Before And After ◽  
Membrane Bound ◽  
Naturally Occurring Antibodies ◽  
Rbc Deformability

Abstract Red cell (RBC) deformability and membrane-bound immunoglobulin G (IgG) were studied to better understand premature clearance of erythrocytes in hereditary spherocytosis. Averaged deformability profiles from cells having comparable cell age revealed that splenectomy was more beneficial for spectrin/ankyrin-deficient than for band 3–deficient RBCs. Splenectomy prevented an early loss of young cells in both types of deficiencies. It had an additional beneficial effect on spectrin/ankyrin-deficient but not band 3–deficient RBCs. It prolonged the survival of mature spectrin/ankyrin-deficient RBCs such that they lost their deformability more slowly than RBCs from patients who had not undergone splenectomy. Band 3–deficient RBCs lost their deformability at the same rate before and after splenectomy. In HS patients with band 3 deficiency who underwent splenectomy, RBC deformability inversely correlated with the number of RBC-bound IgG (up to 140 molecules per cell). In spectrin/ankyrin deficiency, RBC-bound IgG remained at control levels (60 IgG or less per cell). It appears that spectrin/ankyrin-deficient RBCs escaped opsonization by releasing band 3–containing vesicles because their band 3 content and deformability dropped in parallel with increasing cell age. Band 3–deficient RBCs did not lose band 3 with increasing cell age. Hence, it is possible that band 3 clusters required for bivalent binding of low-affinity–IgG, naturally occurring antibodies were retained in band 3–deficient RBCs with a relative excess of skeletal proteins but were released from spectrin/ankyrin-deficient RBCs, in which vesicle budding was facilitated by an impaired skeleton.

scholarly journals HEREDITARY NONSPHEROCYTIC HEMOLYTIC ANEMIA

Blood ◽  
1950 ◽  
Vol 5 (3) ◽  
pp. 233-253 ◽  
Author(s):  
WILLIAM H. CROSBY
Keyword(s):  
Hemolytic Anemia ◽  
Hereditary Spherocytosis ◽  
Red Cells ◽  
Cellular Survival ◽  
The Family ◽  
Group A ◽  
Relationship Of ◽  
Hemoglobin Metabolism

Abstract 1. An hereditary hemolytic anemia is described which is normocytic and normochromic, transmitted as a mendelian dominant and not cured by splenectomy. In the family studied, the anemia was associated but not genetically linked with brachyphalangia. Acute idiopathic porphyria may also have been associated. All anemic members were of blood group A. 2. Neither the degree of anemia nor the rate of hemolysis was favorably influenced by splenectomy. 3. Various studies of erythrocyte fragility and hemoglobin metabolism are presented. 4. Although the red cells in this anemia were more resistant to fragility tests in vitro than the red cells of hereditary spherocytosis, they were more rapidly destroyed in vivo. 5. The disease is differentiated from other hereditary and hemolytic anemias. Of the hereditary anemias, this disease seems most closely to resemble hereditary spherocytosis. Yet the differences of cellular survival in vivo and in vitro and the failure of splenectomy in hereditary nonspherocytic hemolytic anemia suggest a difference in the hemolytic mechanism. 6. The demonstration of porphobilinogen in this patient suggests a possible relationship of this hereditary hemolytic anemia to hereditary porphyria.

scholarly journals Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin/ankyrin- and band 3–deficient hereditary spherocytosis

Blood ◽  
2002 ◽  
Vol 100 (6) ◽  
pp. 2208-2215 ◽  
Author(s):  
Ramune Reliene ◽  
Mariagabriella Mariani ◽  
Alberto Zanella ◽  
Walter H. Reinhart ◽  
M. Leticia Ribeiro ◽  
...  
Keyword(s):  
Hereditary Spherocytosis ◽  
Band 3 ◽  
Vesicle Budding ◽  
Naturally Occurring ◽  
Cell Age ◽  
Before And After ◽  
Membrane Bound ◽  
Naturally Occurring Antibodies ◽  
Rbc Deformability

Red cell (RBC) deformability and membrane-bound immunoglobulin G (IgG) were studied to better understand premature clearance of erythrocytes in hereditary spherocytosis. Averaged deformability profiles from cells having comparable cell age revealed that splenectomy was more beneficial for spectrin/ankyrin-deficient than for band 3–deficient RBCs. Splenectomy prevented an early loss of young cells in both types of deficiencies. It had an additional beneficial effect on spectrin/ankyrin-deficient but not band 3–deficient RBCs. It prolonged the survival of mature spectrin/ankyrin-deficient RBCs such that they lost their deformability more slowly than RBCs from patients who had not undergone splenectomy. Band 3–deficient RBCs lost their deformability at the same rate before and after splenectomy. In HS patients with band 3 deficiency who underwent splenectomy, RBC deformability inversely correlated with the number of RBC-bound IgG (up to 140 molecules per cell). In spectrin/ankyrin deficiency, RBC-bound IgG remained at control levels (60 IgG or less per cell). It appears that spectrin/ankyrin-deficient RBCs escaped opsonization by releasing band 3–containing vesicles because their band 3 content and deformability dropped in parallel with increasing cell age. Band 3–deficient RBCs did not lose band 3 with increasing cell age. Hence, it is possible that band 3 clusters required for bivalent binding of low-affinity–IgG, naturally occurring antibodies were retained in band 3–deficient RBCs with a relative excess of skeletal proteins but were released from spectrin/ankyrin-deficient RBCs, in which vesicle budding was facilitated by an impaired skeleton.

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