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Clinical impacts of DNA-based typing and provision of antigen-matched red blood cell units for chronically transfused patients with thalassemia

P. Watanaboonyongcharoen
P. Watanaboonyongcharoen
, 
S. Onspun
S. Onspun
 and 
P. Rojnuckarin
P. Rojnuckarin
   | Jan 01, 2020
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Article Category: article
Published Online: Jan 01, 2020
Page range: 137 - 145
DOI: https://doi.org/10.21307/immunohematology-2020-053
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© 2020 P. Watanaboonyongcharoen et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Fig. 1

Mean pre-transfusion hemoglobin (Hb) levels before and after antigen-matched transfusion. Hb levels in g/dL for 22 regularly transfused thalassemia patients are shown. The mean pre-transfusion Hb level before enrollment versus the level after 6 months of antigen-matched transfusions from enrollment was 7.2 ± 1.8 versus 7.4 ± 1.6 g/dL. Seventeen patients with no improvement in mean pre-transfusion Hb are represented by dashed lines. The five patients who showed significant improvement in the mean pretransfusion Hb levels (³1.0 g/dL) are represented by solid lines.
Mean pre-transfusion hemoglobin (Hb) levels before and after antigen-matched transfusion. Hb levels in g/dL for 22 regularly transfused thalassemia patients are shown. The mean pre-transfusion Hb level before enrollment versus the level after 6 months of antigen-matched transfusions from enrollment was 7.2 ± 1.8 versus 7.4 ± 1.6 g/dL. Seventeen patients with no improvement in mean pre-transfusion Hb are represented by dashed lines. The five patients who showed significant improvement in the mean pretransfusion Hb levels (³1.0 g/dL) are represented by solid lines.

Fig. 2

Mean timing between transfusions before and after antigen-matched transfusions. The timing between transfusions in 22 regularly transfused patients with thalassemia is shown in days. Seventeen patients with no improvement in mean pre-transfusion hemoglobin (Hb) are represented by dashed lines. However, one of these patients showed markedly longer intervals between transfusions after antigen matching (arrow). Five patients who showed significant improvement in the mean pre-transfusion Hb levels (³1.0 g/dL) are represented by solid lines.
Mean timing between transfusions before and after antigen-matched transfusions. The timing between transfusions in 22 regularly transfused patients with thalassemia is shown in days. Seventeen patients with no improvement in mean pre-transfusion hemoglobin (Hb) are represented by dashed lines. However, one of these patients showed markedly longer intervals between transfusions after antigen matching (arrow). Five patients who showed significant improvement in the mean pre-transfusion Hb levels (³1.0 g/dL) are represented by solid lines.

Clinical characteristics of 24 patients with thalassemia

Patient number Age, years Gender Type of thalassemia Splenectomy Complications/comorbidities Antibody specifcity Predicted phenotype* Mean pre-transfusion Hb levels before antigen-matched transfusion (g/dL) Mean pre-transfusion Hb levels after antigen-matched transfusion (g/dL)
1 29 F Beta-thalassemia Hb E No Iron overload Lea, Leb, E, Mia, Fyb, Dia E–, K–, Fy(b–), S–, Mi(a–), Di (a–) 4.5 5.5
2 12 M Homozygous beta-thalassemia Yes (3 years before the study) Iron overload, chronic portal, and splenic vein Mia, Leb, Jkb, unidentifed E–, K–, Fy(b–), Jk(b–), Mi(a–), Di (a–) 7.2 9.1
3 3 F Homozygous beta-thalassemia No Iron overload E, c, Mia, S E–, c–, K–, Fy(b–), N–, S–, Mi(a–), Di (a–) 8.9 8.6
4 36 F Beta-thalassemia Hb E No Iron overload E, c, Mia, S, Jkb E–, c–, K–, Jk(b–), Fy(b–), N–, S–, Mi(a–), Di (a–) 6.8 7.2
5 40 F Homozygous beta-thalassemia No Iron overload M, c, K, Jkb, Mia E–, c–, K–, Jk(b–), M–, S–, Mi(a–), Di (a–) 7.3 6.9
6 39 F Beta-thalassemia Hb E Yes (23 years before the study) Iron overload Lea, Leb, S, unidentifed K–, Fy(b–), N–, S–, Di (a–) 6.9 7.4
7 14 M Beta-thalassemia Hb E No Iron overload E, c, Mia, Dia E–, c–, K–, Fy(b–), N–, S–, Mi(a–), Di (a–) 7.9 7.7
8 20 F Homozygous beta-thalassemia Yes (16 years before the study) Iron overload, transaminitis E, c, S, Chido E–, c–, K–, N–, S–, Di (a–) 8.7 7.4
9 27 M Homozygous beta-thalassemia Yes (23 years before the study) Iron overload E, c, Lea, Fyb E-, c-, K-, Fy(b-), S-, Mi(a-), Di(a-) 4.7 3.6
10 42 F Beta-thalassemia Hb E No Iron overload Jka, Mia, unidentifed C–, K–, Fy(b–), Jk(a–), M–, S–, Mi(a–), Di (a–) 5.8 6.1
11 53 F Hb H Constant Spring Yes (20 years before the study) Iron overload, pulmonary hypertension, chronic deep vein thrombosis, and pulmonary embolism E, c, Mia E–, c–, K–, S–, Mi(a–), Di (a–) 9.8 9.7
12 44 M Homozygous beta-thalassemia Yes (during the study) Iron overload, extramedullary hematopoiesis at spine causing neurodefciency E, c, Mia E–, c–, K–, Fy(b–), Mi(a–), Di (a–) 6.3 7.3
13 58 F Beta-thalassemia Hb E Yes (13 years before the study) Iron overload E, auto-C E–, c–, K–, Jk(b–), Fy(b–), S–, Mi(a–), Di (a–) 8.1 7.6
14 20 F Homozygous beta-thalassemia Yes (6 years before the study) Iron overload, pulmonary hypertension Mia, S E-, c-, K-, Jk(b-), S-, Mi(a-) 6.5 7.3
15 20 F Beta-thalassemia Hb E No Iron overload, epilepsy E, c E–, c–, K–, S–, Mi(a–), Di (a–) 3.7 5.1
16 2 F Homozygous beta-thalassemia No None Jka, unidentifed K–, Jk(a–), N–, S–, Mi(a–), Di (a–) 9.0 9.2
17 19 F Beta-thalassemia Hb E No Iron overload E, unidentifed E–, c–, K–, Jk(b–), Fy(b–), M–, S–, Mi(a–), Di (a–) 7.9 8.3
18 7 F Beta-thalassemia Hb E No Iron overload Chido E–, c–, K–, Jk(b–), Fy(b–), S–, Mi(a–), Di (a–) 8.7 9.1
19 11 F Beta-thalassemia Hb E No Iron overload, ventricular septal defect/atrial septal defect Mia E–, K–, N–, S–, Mi(a–), Di (a–) 7.6 7.3
20 35 M Beta-thalassemia Hb E Yes (22 years before the study) Iron overload E E-, c-, K-, Fy(b-), S-, Mi(a-), Di(a-) 3.7 4.9
21 28 F Beta-thalassemia Hb E Yes (23 years before the study) Iron overload Nonspecifc cold E-, K-, Fy(b-), N-, S-, Mi(a-), Di(a-) 8.5 8.9
22 6 F Homozygous beta-thalassemia No Iron overload No alloantibody E–, c–, K–, Jk(a–), N–, S–, Mi(a–), Di (a–) Patient did not receive regular transfusion before enrollment; excluded from study.
23 17 F Beta-thalassemia Hb E No Iron overload No alloantibody E–, c–, K–, Fy(b–), N–, S–, Mi(a–), Di (a–) 9.2 7.6
24 27 F Beta-thalassemia Hb E No Iron overload No alloantibody E–, c–, K–, Jk(b–), N–, S–, Mi(a–), Di (a–) Patient did not receive regular transfusion before enrollment; excluded from study.
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