HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

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

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.
AuthorsRamune Reliene, Mariagabriella Mariani, Alberto Zanella, Walter H Reinhart, M Leticia Ribeiro, Emanuele Miraglia del Giudice, Silverio Perrotta, Achille Iolascon, Stefan Eber, Hans U Lutz
JournalBlood (Blood) Vol. 100 Issue 6 Pg. 2208-15 (Sep 15 2002) ISSN: 0006-4971 [Print] United States
PMID12200387 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Anion Exchange Protein 1, Erythrocyte
  • Ankyrins
  • Immunoglobulin G
  • Membrane Proteins
  • Spectrin
Topics
  • Anion Exchange Protein 1, Erythrocyte (deficiency, genetics, metabolism)
  • Ankyrins (deficiency)
  • Case-Control Studies
  • Cell Survival
  • Erythrocyte Aging (physiology)
  • Erythrocyte Deformability
  • Erythrocytes (chemistry, cytology)
  • Family Health
  • Female
  • Humans
  • Immunoglobulin G (metabolism)
  • Male
  • Membrane Proteins (deficiency, genetics, metabolism)
  • Protein Binding
  • Spectrin (deficiency)
  • Spherocytosis, Hereditary (blood, etiology, surgery)
  • Splenectomy
  • Treatment Outcome

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: