Clinical characteristics and outcomes of thrombotic microangiopathy in Malaysia
Blood Research
;
: 130-137, 2018.
Artigo
em Inglês
| WPRIM
| ID: wpr-714931
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.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Troca Plasmática
/
Púrpura Trombocitopênica Trombótica
/
Azatioprina
/
Vincristina
/
Imunoglobulinas
/
Metilprednisolona
/
Estudos Epidemiológicos
/
Estudos Retrospectivos
/
Mortalidade
/
Tacrolimo
Tipo de estudo:
Estudo observacional
/
Estudo prognóstico
Limite:
Feminino
/
Humanos
/
Gravidez
País/Região como assunto:
Ásia
Idioma:
Inglês
Revista:
Blood Research
Ano de publicação:
2018
Tipo de documento:
Artigo
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