Blood Res 2018; 53(2):
Published online June 25, 2018
https://doi.org/10.5045/br.2018.53.2.130
© The Korean Society of Hematology
1Department of Hematology, Hospital Ampang, Ampang, Malaysia.
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.
Correspondence to : Yee Yee Yap, M.D. Department of Haematology, Hospital Ampang, Jalan Mewah Utara, Pandan Mewah, 68000 Ampang, Selangor, Malaysia. mandyyapyy@yahoo.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
This was a retrospective epidemiological study involving all cases of TMA from 2012–2016.
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,
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.
Keywords Thrombotic microangiopathy, TTP, ADAMTS13 activity, Overall survival, Secondary TMA
Blood Res 2018; 53(2): 130-137
Published online June 25, 2018 https://doi.org/10.5045/br.2018.53.2.130
Copyright © The Korean Society of Hematology.
Yee Yee Yap1,3,4*, Jameela Sathar1, Kian Boon Law2, Putri Astina Binti Zulkurnain1, Syed Carlo Edmund1, Kian Meng Chang1, and Ross Baker3,4
1Department of Hematology, Hospital Ampang, Ampang, Malaysia.
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.
Correspondence to:Yee Yee Yap, M.D. Department of Haematology, Hospital Ampang, Jalan Mewah Utara, Pandan Mewah, 68000 Ampang, Selangor, Malaysia. mandyyapyy@yahoo.com
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
This was a retrospective epidemiological study involving all cases of TMA from 2012–2016.
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,
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.
Keywords: Thrombotic microangiopathy, TTP, ADAMTS13 activity, Overall survival, Secondary TMA
Table 1 .
Abbreviation: SLE, Systemic lupus erythematosus..
Table 3 .
a)TTP includes primary acquired TTP and congenital TTP. b)TMA includes TMA and TTP with secondary causes and unknown causes. c)Cyclopsorine and tacrolimus were only used in TA-TMA..
Abbreviation: IVIG, Intravenous immunoglobulin..
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