Clinical course, laboratory parameters and outcome of TTP pediatric patients presenting with posterior reversible encephalopathy syndrome

Figures & data

Table 1. Clinical and laboratory data of our study.

Patient data	1	2	3	4	5	6	7
Sex/age (years)	Male/5	Male/8	Female/14	Male/12	Female/18	Female/16	Female/9
Presentation (duration)	Headache, fever, seizures, bloody diarrhea (10 d) and hematuria	Vomiting, altered sen- sorium, oliguria and abdominal pain (10 d)	Easy fatigability, encephalopathy, headache and hematochezia (4 d)	Altered sensorium, oliguria, headache and vomiting (12 d)	Rash, fever, pain abdomen and diarrhea (5 d)	Decreased urine output, breathlessness, seizures and anasarca (5 d)	Progressive swelling of body, oliguria, seizures and rash (21 d)
B.P (mmHg)	180/100	186/120	170/110	200/110	210/120	180/100	140/100
Urine output (mL/day)	200	300	1100	1500	400	Urine output (mL/d)	200
Creatinine (mg/dL)/RAS	1.9/F	2.1/F	2.2/F	4.0/F	3.0/F	3.5//F	1.8/F
White blood cells (×10^9/L)	9.4	10.31	22.8	9.8	14.6	10.2	8.0
Platelet count/mm3	80,000	110,000	58,000	185,000	121,000	195,000	100,000
S. Bilirubin levels (mg/dL)	1.5	1.3	2.0	1.0	1.9	.80	2.3
Fibrinogen levels (mg/dL)	250	400	350	390	430	400	270
LDH levels (IU/L)	400	650	320	850	760	300	640
Proteinuria	+++	+	+++	Nil	Nil	++	Nil
Hematuria/HPF	20–26	8–10	28–30	Nil	Nil	Nil	Nil
Outcome	Died	Improved	Improved	Died	Died	Died	Improved
Follow-up MRI	Not applicable	Normal	Normal	Not applicable	Not applicable	Normal	Normal

Note: BP: Blood pressure; RAS: RIFLE AKI Stage; HPF: high power field; MRI: magnetic resonance imaging.

Figure 1. MRI at presentation of PRES. T2-weighted image (A1) and FLAIR image (B1) show high signal abnormalities bilaterally in the parietooccipital regions, in both subcortical white matter and cortical gray matter. MRI after 2 weeks of follow-up shows complete disappearance of all high signal abnormalities in both T2-weighted image (A2) and FLAIR image (B2).

Figure 2. Glomerulus from a H & E stained section, showing significant loss of mesangial matrix, with a fluffy appearing remaining mesangial area and retraction of capillary tufts (mensagiolysis). The glomerular capillaries are congested and dilated (H&E × 250).

Figure 3. (A) Non-contrast CT head at presentation showing diffuse white matter and gray matter edema. (B) MRI brain of the same patient showing hyperintense lesions in parietooccipital area and cerebellar regions predominantly on the left side on T2W images.

Figure 4. Photomicrographs showing ischemic shrinkage of capillary tufts with dilated and congested capillary lumina, loss of mesangial anchorage and presence of few fibrin thrombi in glomerular capillaries. (Masson's Trichrome × 250).

Table 2. Comparison between present study and two other pediatric studies.

Parameters	de Laat et al.	Horton et al.	Present study
No. of patients	7	7	7
Age range (years)	2–17	7–15	8–18
Primary diagnosis	ALL-4; BL-1; NHL-1; ES- 1	TTP	TTP
Presentation
Hypertension	100%	Nil	100%
Bleeding	Nil	42.8% (GI, UT)	28.5%
Neurological presentation	100%	71.4%	100%
Renal failure	N	28.5%	100%
Other lab abnormalities	NS	Bilirubin; platelet count	Leukocytosis; platelet count;  proteinuria
TTP	_	+	-
PRES	+	_	-
Both	_	_	+
Outcome
(i) Improved	100%	100%	42.8%
(ii) Death	Nil	Nil	57.1%
(iii) Sequela	Nil	Nil	Nil

Note: ALL: Acute lymphoblastic leukemia; BL: Burkitts lymphoma; NHL: non-Hodgkin’s lymphoma; ES: Ewing’s sarcoma; GIT: gastrointestinal tract; UT: urinary tract; N: Normal; NS: non specific.

Burrus TM, Mandrekar J, Wijdicks EF, Rabinstein AA. Renal failure and posterior reversible encephalopathy syndrome in patients with thrombotic thrombocytopenic purpura. Arch Neurol. 2010;67:831–834

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