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Comparison of laboratory findings in hereditary TTP versus ABO incompatibility in neonates

Comparison of laboratory findings in hereditary TTP versus ABO incompatibility in neonates
Characteristic hTTP
(n = 4)
ABO incompatibility
(n = 20)
Platelet count minimum 17,000±12,000/microL 291,000±76,000/microL
Anemia (percentage of patients) 100% 25%
Minimum hemoglobin (g/dL) 10.6±2.4 16.3±2.3
Maximum total bilirubin (mg/dL) 24±6 16±3
Time to jaundice onset (hours) 10 (5 to 13) 69 (18 to 82)
Time to maximum bilirubin (hours) 39±16 74±27
Bilirubin response to phototherapy and IVIG (change, in mg/dL) 3±4 –4±3
BUN (mmol/L) 8 (5 to 42) 41 (28 to 75)
Creatinine (micromol/L) 93 (58 to 149) 41 (28 to 75)
Positive direct Coombs test, percentage 0% 65%
All patients with hTTP had ADAMTS13 activity <5% without an inhibitor and biallelic pathogenic variants in the ADAMTS13 gene. A major difference between groups was severe thrombocytopenia in the TTP group and positive direct Coombs test in the ABO incompatibility group. Other differences included earlier and more severe jaundice, with greater likelihood of anemia, in neonates with hTTP. Most of the above differences were statistically significant with p values <0.001 (exceptions: time to maximum bilirubin, p = 0.02; presence of anemia, p = 0.01; creatinine, p = 0.02; positive direct Coombs test, p = 0.07). Parameters that were not statistically different between groups included WBC count, C-reactive protein, albumin, and hepatic alanine aminotransferase. Refer to the original paper for details and to UpToDate for additional discussions of hTTP.
hTTP: hereditary thrombotic thrombocytopenic purpura; BUN: Blood urea nitrogen; WBC: white blood cell.
Adapted with permission from: Liu J, Zhang Y, Li X, et al. Early indicators of neonatal-onset hereditary thrombotic thrombocytopenia purpura. Res Pract Thromb Haemost 2022; 6:e12820. https://onlinelibrary.wiley.com/doi/full/10.1002/rth2.12820. Copyright © 2022 The Authors. Reproduced with permission of John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, shared or emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission' link accompanying this article on Wiley Online Library (https://onlinelibrary.wiley.com/).
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