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Blood, 15 September 2002, Vol. 100, No. 6, pp. 2208-2215
RED CELLS
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, and
Hans U. Lutz
From the Institute of Biochemistry, Swiss Federal Institute of
Technology, Zurich; Department of Internal Medicine, Kantonsspital
Chur, and Children's Hospital, University of Zurich, Switzerland;
Division of Hematology, IRCCS Ospedale Maggiore, Milan; Pediatric
Clinic, Department of Pediatrics, Second University of Naples;
Institute for Pediatrics, University of Foggia and CEINGE-Advanced
Biotechnology Naples, Italy; and Department of Hematology, Centro
Hospitalar de Coimbra, Coimbra, Portugal.
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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