Blood Res.  2018 Jun;53(2):130-137. 10.5045/br.2018.53.2.130.

Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia

Affiliations
  • 1Department of Hematology, Hospital Ampang, Ampang, Malaysia. mandyyapyy@yahoo.com
  • 2Clinical Trial Unit, Clinical Research Centre, Ministry of Health, Putrajaya, Malaysia.
  • 3Perth Blood Institute, Murdoch University, Perth, Australia.
  • 4Western Australian Centre for Thrombosis and Hemostasis, Murdoch University, Perth, Australia.

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.

Keyword

Thrombotic microangiopathy; TTP; ADAMTS13 activity; Overall survival; Secondary TMA

MeSH Terms

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
Azathioprine
Bortezomib
Cyclophosphamide
Cyclosporine
Immunoglobulins
Methylprednisolone
Rituximab
Tacrolimus
Thrombospondins
Vincristine
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