Table 1.
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. | |
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.
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.