Li BH, Hu SY. Child with Wiskott–Aldrich syndrome underwent atypical immune reconstruction after umbilical cord blood transplantation: A case report. World J Clin Cases 2019; 7(21): 3622-3631 [PMID: 31750346 DOI: 10.12998/wjcc.v7.i21.3622]
Corresponding Author of This Article
Shao-Yan Hu, PhD, Chief Doctor, Department of Hematology and Oncology, Children’s Hospital of Soochow University, 92 Zhongnan Street, Suzhou 215000, Jiangsu Province, China. hsy139@126.com
Research Domain of This Article
Medicine, Research & Experimental
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Clin Cases. Nov 6, 2019; 7(21): 3622-3631 Published online Nov 6, 2019. doi: 10.12998/wjcc.v7.i21.3622
Table 1 Details of human leukocyte antigen matching
Patient 0
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
HLA-A(patient/donor)
02:07 11:02
02:01 33:03
02:07 30:01
24:20 30:01
32:01 11:01
03:01 01:01
02:07 24:02
02:06 33:03
02:07 30:01
24:20 31:01
32:01 02:06
03:01 32:01
HLA-B(patient/donor)
54:01 40:02
40:06 58:01
13:02 46:01
13:02 40:01
44:03 48:01
27:05 57:01
54:01 56:01
40:06 58:01
13:02 46:01
13:02 40:01
44:03 48:01
27:05 57:01
HLA-C(patient/donor)
01:02 07:02
03:02 08:01
01:02 06:02
06:02 07:02
04:01 08:01
02:02 06:02
01:02 07:02
03:02 08:01
01:02 06:02
06:02 07:02
04:01 08:01
02:02 06:02
HLA-DRB1(patient/donor)
14:54 12:02
03:01 09:01
07:01 09:01
07:01 08:03
07:01 15:01
15:01 11:04
14:55 12:02
03:01 09:01
07:01 09:01
07:01 08:03
07:01 15:01
15:01 10:01
HLA-DQB1(patient/donor)
05:03 03:02
02:01 03:03
02:02 03:03
02:02 06:01
02:02 06:02
03:01 03:01
05:03 03:01
02:01 03:03
02:02 03:03
02:02 06:01
02:02 05:03
05:01 06:02
Matches
6/10
9/10
10/10
9/10
8/10
6/10
Table 2 Details of drug use
The conditioning regimen
Bu + FLu + CTX + ATG
BU 1.0 mg/kg, day-9 to day-6; FLu, 40 mg/m2, day-5 to day-2; CTX, 60 mg/kg, day-5 to day-4; ATG 2.5 mg/kg, qd, day-8 to day-5
Prophylaxis for GVHD
CSA + MMF
CSA (6-8 mg/kg/d) and MMF (20 mg/kg/d)
Preventing hepatic VOD
Heparin and alprostadil
Preventing infection
Ganciclovir, acyclovir, micafungin and meropenem
Table 3 Basic clinical parameters of five other umbilical cord blood transplantation patients
Patient 0 (case)
Patient 1
Patient 2
Patient 3
Patient 4
Patient 5
Age in yr
1
4
< 1 (8 m)
1
5
2
Sex
Male
Male
Male
Male
Male
Male
Time for UCBT
2017/10/15
2017/6/3
2018/6/8
2018/3/24
2017/7/7
2017/12/15
CD34 dose
4.7 × 105/kg
2 × 105/kg
4.92 × 105/kg
2.4 × 1055/kg
4.55 × 105/kg
3.0 × 105/kg
TNC
9.17 × 107/kg
6.73 × 107/kg
15.3 × 107/kg
11.1 × 107/kg
6.26 × 107/kg
9.23 × 107/kg
Conditional regimen
Bu + Flu + CTX + ATG
BU + Flu + CTX + ATG
BU + Flu + CTX + ATG
BU + Flu + CTX + ATG
BU + Flu + CTX + ATG
BU + Flu + CTX + ATG
HLA (/6)
4/6
6/6
6/6
5/6
5/6
4/6
HLA (/10)
6/10
9/10
10/10
9/10
8/10
6/10
Blood type (patient/donor)
B/B
A/O
B/B
O/O
O/O
A/AB
Neutrophil grafted time
D34
D16
D13
D15
D17
D14
Lymphocytes>0.5 × 109/L
D23
D28
D42
D17
D30
D65
PLT grafted time
D66
D28
D29
D30
D33
D27
Main complication
No
Hemorrhagic cystitis
Infection by Pneumocystis carinii
No
Intestinal infection
Leak syndrome, hemorrhagic cystitis
Table 4 Details of short tandem repeats
Day
PB or BM
STR
14
PB
76.80%
24
PB
98.60%
31
PB
99.10%
38
PB
98.80%
45
PB
99.30%
52
PB
98.80%
60
PB
99.30%
66
PB
98.60%
75
PB
98.70%
102
PB
98.70%
17
BM
62.30%
26
BM
98.10%
43
BM
98.70%
Citation: Li BH, Hu SY. Child with Wiskott–Aldrich syndrome underwent atypical immune reconstruction after umbilical cord blood transplantation: A case report. World J Clin Cases 2019; 7(21): 3622-3631