Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia.
- Author:
Yee Yee YAP
1
;
Jameela SATHAR
;
Kian Boon LAW
;
Putri Astina Binti ZULKURNAIN
;
Syed Carlo EDMUND
;
Kian Meng CHANG
;
Ross BAKER
Author Information
- Publication Type:Original Article
- Keywords: Thrombotic microangiopathy; TTP; ADAMTS13 activity; Overall survival; Secondary TMA
- MeSH: Asian Continental Ancestry Group; Atypical Hemolytic Uremic Syndrome; Azathioprine; Bortezomib; Cyclophosphamide; Cyclosporine; Epidemiologic Studies; Female; Humans; Immunoglobulins; Lupus Erythematosus, Systemic; Malaysia*; Methylprednisolone; Mortality; Plasma Exchange; Pregnancy; Purpura, Thrombotic Thrombocytopenic; Retrospective Studies; Rituximab; Tacrolimus; Thrombospondins; Thrombotic Microangiopathies*; Vincristine
- From:Blood Research 2018;53(2):130-137
- CountryRepublic of Korea
- Language:English
- 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.
