Cerebral Blood Flow and Marrow Diffusion Alterations in Children with Sickle Cell Anemia after Bone Marrow Transplantation and Transfusion

M.T. Whitehead; A. Smitthimedhin; J. Webb; E.S. Mahdi; Z.P. Khademian; J.L. Carpenter; A. Abraham

doi:10.3174/ajnr.A5830

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Fig 1.
A 13-year-old girl with sickle cell disease. Pre-bone marrow transplantation axial DWI (A) (TR/TE = 10,000/83 ms, slice thickness = 5 mm) through the greater sphenoid wing (thin arrow) and occipital bone (thick arrow) and corresponding quantitative ADC map with ROIs (B) placed demonstrating reduced diffusion of the bone marrow. Follow-up MR imaging at 14 years of age from the same patient post-bone marrow transplantation with axial DWI (C) (TR/TE = 10,000/79.8 ms, slice thickness = 5 mm) at the same level as A and corresponding quantitative ADC map (D) demonstrating facilitated diffusion with respect to the pretransplantation scan.
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Fig 2.
An 11-year-old boy with sickle cell disease. Pre-bone marrow transplantation, axial pseudocolor ASL (A) depicts manual ROIs drawn to interrogate CBF from the bilateral ACA, MCA, and PCA territories. Diffusely increased signal is present throughout the cerebrum, consistent with elevated cerebral blood flow. Follow-up MR imaging at 12 years of age from the same patient, post-bone marrow transplantation, with axial pseudocolor ASL (B) depicting persistent-but-improved/decreased hyperperfusion.
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Fig 3.
Comparison of arterial territory–specific quantitative mean CBF in patients with SCA before BMT (left) and at the first scan after BMT (right). R indicates right; L, left.
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Fig 4.
A 31-month-old girl with sickle cell disease. Pretransfusion axial pseudocolor ASL (A) depicts manual ROIs drawn to interrogate CBF from the bilateral ACA, MCA, and PCA territories. Diffusely increased signal is present throughout the cerebrum, consistent with elevated cerebral blood flow. Follow-up MR imaging at 36 months of age from the same patient at the same level posttransfusion with axial pseudocolor ASL (B) depicting persistent hyperperfusion without significant change.
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Fig 5.
Comparison of arterial territory–specific quantitative mean CBF in patients with SCA before initial transfusion (left) and at the first scan after transfusion initiation (right). R indicates right; L, left.

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Table 1:

Transplant group—hemoglobinopathy subtype, transplant data, and MR imaging data analyzed^{^a}

Pt	Diagnosis	Source	Donor	HLA Match	Scans (No.)	Age (yr)	DWI (No.)	ASL (Right Cerebrum) (No.)	ASL (Left Cerebrum) (No.)
1	HbSS	BM	R	8/8	2	18,19	2	2	2
2	HbSS	BM	R	8/8	4	15,16,16,18	4	4	4
3	HbS-B0Th	PBSC	R, H	4/8	2	12,13	2	2	2
4	HbSC	BM	R	8/8	5	13,14,14,16,17	5	5	5
5	HbSS	BM	R	8/8	4	7,10,11	3	3	3
6	HbSS	BM	R	8/8	2	7,9	2	2	2
7	HbS-B0Th	BM	R	8/8	2	10,12	2	2	2
8	HbSS	BM	R	8/8	3	11,11,12	3	2	2
9	HbSS	BM	R	8/8	4	5,7,8,9	4	4	4
10	HbSS	UCB	R	5/6	4	8,8,9,10	4	0	4
11	HbSS	BM	R	8/8	3	8,9,10	3	3	3
12	HbSS	PBSC	R, H	3/6	3	4,5,5	3	0	3
13	HbSS	BM	U	8/8	5	18,18,18,19,20	5	3	3
14	HbSS	BM	R	8/8	2	1,2	2	2	2
15	HbSS	BM	R	10/10	3	3,3,3	2	3	3
16	HbSS	BM	R	8/8	2	15,16	2	2	2
17	HbSS	UCB	U	6/6	3	3,3,4	3	3	3

Note:—Pt indicates patient; BM, bone marrow; PBSC, peripheral blood stem cell; UCB, umbilical cord blood; R, related; H, haploidentical; U, unrelated; HbSS, homozygous hemoglobin S; HbSC, sickle cell hemoglobin C; HbS-B0Th, hemoglobin S-β-thalassemia disease; HLA, human leukocyte antigen.
↵a SCA subtype, transplant information, total MRI scans, number of DWIs, and number of ASL sequences analyzed (right and left cerebral hemispheres) from 17 patients with transplants.

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Table 2:

Chronic transfusion group—hemoglobinopathy subtype and MR imaging data analyzed^{^a}

Pt	Diagnosis	Scans (No.)	Age (yr)	DWI (No.)	ASL (Right Cerebrum) (No.)	ASL (Left Cerebrum) (No.)
1	HbSS	3	7,9,11	0	3	0
2	HbSS	6	0,1,2,5,6	3	1	5
3	HbSS	2	7,8	0	2	2
4	HbSS	2	8,11	2	0	2
5	HbSS	6	2,3,4,5,6,7	0	6	6
6	HbSS	2	4,6	1	2	2
7	HbSS	2	6,8	1	2	2
8	HbSS	3	3,4,5	1	3	3
9	HbSS	3	9,10,11	0	3	3

Note:—Pt indicates patient; HbSS, homozygous hemoglobin S.
↵a SCA subtype, total MRI scans, number of DWIs, and number of ASL sequences analyzed (right and left cerebral hemispheres) from 9 patients with transfusion.

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Table 3:

Pre- and posttransplantation ASL perfusion changes^{^a}

Pt	Pretransplantation							Posttransplantation							P Value
Pt	MCA (R)	MCA (L)	ACA (R)	ACA (L)	PCA (R)	PCA (L)	Avg	MCA (R)	MCA (L)	ACA (R)	ACA (L)	PCA (R)	PCA (L)	Avg	P Value
1	71	70	68	73	55	60	66	78	79	69	75	58	68	71	.02^{^b}
2	92	82	88	85	96	89	89	74	78	70	73	61	65	70	.008^{^c}
3	82	80	79	83	92	90	84	60	63	62	64	64	64	63	<.001^{^c}
4	66	63	64	60	50	51	59	34	35	37	38	28	30	34	<.001^{^c}
5	100	80	93	85	97	83	90	62	61	62	61	63	54	61	<.001^{^c}
6	90	89	96	93	87	91	91	81	77	74	70	71	73	74	<.001^{^c}
7	68	65	68	63	63	59	64	69	62	37	59	51	57	56	.14
8	76	72	73	67	73	73	72	59	54	56	52	48	46	53	<.001^{^c}
9	84	65	77	64	86	77	76	97	87	86	84	79	71	84	.16
10	NA	57	NA	56	NA	55	56	NA	45	NA	48	NA	40	44	.03^{^c}
11	73	81	76	77	72	74	76	78	73	75	74	71	74	74	.47
12	89	NA	77	NA	100	NA	95	82	NA	72	NA	87	NA	80	.07
13	63	66	59	68	66	37	60	61	60	67	55	58	67	61	.82
14	85	81	79	76	77	74	79	77	79	65	70	62	63	69	.006^{^c}
15	68	71	67	72	63	64	68	64	65	63	62	53	50	60	.004^{^c}
16	76	73	76	71	75	71	74	66	66	57	58	45	47	57	.005^{^c}
17	100	99	101	94	91	94	97	40	40	37	37	35	37	38	<.001^{^c}
T	81	76	79	75	78	72	77	66	63	60	60	57	55	60	<.001^{^c}

Note:—Pt indicates patient; R, right; L, left; Avg, average/mean whole-brain CBF; NA, not applicable/excluded; T, mean of each arterial territory.
↵a Arterial territory–specific and mean whole-brain CBF values (in milliliters/100 g of tissue/min) from 17 patients with BMT prior to (left columns) and at the first scan after (right columns) transplantation. In the interest of space and simplicity, CBF values were rounded to the nearest whole number for this display.
↵b Significant increase in mean whole-brain CBF.
↵c Significant.

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Table 4:

Pre- and postinitiation of chronic transfusion therapy ASL perfusion changes^{^a}

Pt	Pretransfusion							Posttransfusion							P Value
Pt	MCA (R)	MCA (L)	ACA (R)	ACA (L)	PCA (R)	PCA (L)	Avg	MCA (R)	MCA (L)	ACA (R)	ACA (L)	PCA (R)	PCA (L)	Avg	P Value
1	96	NA	78	NA	93	NA	89	95	NA	94	NA	87	NA	92	.69
2	79	71	64	65	62	73	69	NA	87	NA	84	NA	80	84	.06
3	103	91	102	98	111	107	102	75	78	76	76	73	75	76	<.001^{^b}
4	NA	89	NA	80	NA	80	83	NA	75	NA	82	NA	76	78	.37
5	90	96	81	93	82	88	88	93	96	95	101	91	93	95	.02^{^c}
6	84	102	88	100	78	83	90	79	96	81	86	78	76	83	.02^{^b}
7	98	93	88	90	92	88	92	81	84	74	85	74	83	81	.005^{^b}
8	70	68	70	65	65	64	67	74	79	64	71	60	60	68	.74
9	70	70	64	66	63	71	67	69	74	77	85	73	80	87	.02^{^c}
T	86	78	79	82	81	82	81	81	84	80	80	77	78	80	.47

Note:—Pt indicates patient; R, right; L, left; Avg, average/mean whole-brain CBF; NA, not applicable/excluded; T, mean of each arterial territory.
↵a Arterial territory–specific and mean whole-brain CBF values (in milliliters/100 g of tissue/min) from 9 patients with transfusion prior to (left columns) and at the first scan after (right columns) transfusion initiation. In the interest in space and simplicity, CBF values were rounded to the nearest whole number for this display.
↵b Significant.
↵c Significant increase in mean whole-brain CBF.