Journal article
Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia
Blood Research, Vol.53(2)
2018
Abstract
Background
Thrombotic microangiopathy (TMA) with non-deficient ADAMTS-13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif 13) outcome is unknown hence the survival analysis correlating with ADAMTS-13 activity is conducted in Malaysia.
Methods
This was a retrospective epidemiological study involving all cases of TMA from 2012–2016.
Results
We evaluated 243 patients with a median age of 34.2 years; 57.6% were female. Majority of the patients were Malay (62.5%), followed by Chinese (23.5%) and Indian (8.6%). The proportion of patients with thrombotic thrombocytopenic purpura (TTP) was 20.9%, 72.2% of which were acquired while 27.8% were congenital. Patients with ADAMTS-13 activity ≥5% had a four-fold higher odds of mortality compared to those with ADAMTS-13 activity <5% (odds ratio: 4.133, P=0.0425). The mortality rate was 22.6% (N=55). Most cases had secondary etiologies (42.5%), followed by acquired TTP (16.6%), atypical hemolytic uremic syndrome (HUS) or HUS (12.8%) and congenital TTP (6.4%). Patients with secondary TMA had inferior overall survival (P=0.0387). The secondary causes comprised systemic lupus erythematosus (30%), infection (29%), pregnancy (10%), transplant (8%), malignancy (6%), and drugs (3%). Transplant-associated TMA had the worst OS (P=0.0016) among the secondary causes. Plasma exchange, methylprednisolone and intravenous immunoglobulin were recorded as first-line treatments in 162 patients, while rituximab, bortezomib, vincristine, azathioprine, cyclophosphamide, cyclosporine, and tacrolimus were described in 78 patients as second-line treatment.
Conclusion
This study showed that TMA without ADAMTS-13 deficiency yielded inferior outcomes compared to TMA with severeADAMTS-13 deficiency, although this difference was not statistically significant.
Details
- Title
- Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia
- Authors/Creators
- Y.Y. Yap (Author/Creator) - Murdoch UniversityJ. Sathar (Author/Creator) - KPJ Ampang Puteri Specialist HospitalK.B. Law (Author/Creator) - Ministry of HealthP.A.B. Zulkurnain (Author/Creator) - KPJ Ampang Puteri Specialist HospitalS.C. Edmund (Author/Creator) - KPJ Ampang Puteri Specialist HospitalK.M. Chang (Author/Creator) - KPJ Ampang Puteri Specialist HospitalR. Baker (Author/Creator) - Murdoch University
- Publication Details
- Blood Research, Vol.53(2)
- Publisher
- Kamje Press
- Identifiers
- 991005543518007891
- Copyright
- © 2018 Korean Society of Hematology
- Murdoch Affiliation
- Murdoch University
- Language
- English
- Resource Type
- Journal article
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