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

Ramune Reliene, Mariagabriella Mariani, Alberto Zanella, Walter H. Reinhart, M. Leticia Ribeiro, Emanuele Miraglia Del Giudice, Silverio Perrotta, Achille Iolascon, Stefan Eber, Hans U. Lutz

Research output: Contribution to journalArticle

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 deform-ability 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.

Original languageEnglish
Pages (from-to)2208-2215
Number of pages8
JournalBlood
Volume100
Issue number6
Publication statusPublished - Sep 15 2002

ASJC Scopus subject areas

  • Hematology

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    Reliene, R., Mariani, M., Zanella, A., Reinhart, W. H., Leticia Ribeiro, M., Del Giudice, E. M., Perrotta, S., Iolascon, A., Eber, S., & Lutz, H. U. (2002). Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin/ankyrin- and band 3-deficient hereditary spherocytosis. Blood, 100(6), 2208-2215.