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

Watanaboonyongcharoen, P.; Onspun, S.; Rojnuckarin, P.

doi:10.21307/immunohematology-2020-053

Figures & Tables

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 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	Le^a, Le^b, E, Mi^a, Fy^b, Di^a	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	Mi^a, Le^b, Jk^b, 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, Mi^a, 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, Mi^a, S, Jk^b	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, Jk^b, Mi^a	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	Le^a, Le^b, 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, Mi^a, Di^a	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, Le^a, Fy^b	E-, c-, K-, Fy(b-), S-, Mi(a-), Di(a-)	4.7	3.6
10	42	F	Beta-thalassemia Hb E	No	Iron overload	Jk^a, Mi^a, 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, Mi^a	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, Mi^a	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	Mi^a, 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	Jk^a, 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	Mi^a	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.

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

Figures & Tables

Fig. 1

Fig. 2

Clinical characteristics of 24 patients with thalassemia

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