1.Persistent thrombocytopenia following dengue fever: What should we do?
Yang Liang Boo ; Suat Yee Lim ; Hon Shen P&rsquo ; ng ; Christopher Chin Keong Liam ; Nai Chien Huan
Malaysian Family Physician 2019;14(3):71-73
Thrombocytopenia is a common laboratory finding in dengue infection. However, it usually
resolves as the patient recovers from the infection. Persistent thrombocytopenia following dengue
infection requires further investigation. Here, we present a case of immune thrombocytopenic
purpura (ITP) following dengue infection complicated by intracranial bleeding.
4.Validating lactate dehydrogenase (LDH) as a component of the PLASMIC predictive tool (PLASMIC-LDH)
Christopher Chin KEONG LIAM ; Jim Yu-Hsiang TIAO ; Yee Yee YAP ; Yi Lin LEE ; Jameela SATHAR ; Simon MCRAE ; Amanda DAVIS ; Jennifer CURNOW ; Robert BIRD ; Philip CHOI ; Pantep ANGCHAISUKSIRI ; Sim Leng TIEN ; Joyce Ching MEI LAM ; Doyeun OH ; Jin Seok KIM ; Sung-Soo YOON ; Raymond Siu-Ming WONG ; Carolyn LAUREN ; Eileen Grace MERRIMAN ; Anoop ENJETI ; Mark SMITH ; Ross Ian BAKER
Blood Research 2023;58(1):36-41
Background:
The PLASMIC score is a convenient tool for predicting ADAMTS13 activity of <10%.Lactate dehydrogenase (LDH) is widely used as a marker of haemolysis in thrombotic thrombocytopenic purpura (TTP) monitoring, and could be used as a replacement marker for lysis. We aimed to validate the PLASMIC score in a multi-centre Asia Pacific region, and to explore whether LDH could be used as a replacement marker for lysis.
Methods:
Records of patients with thrombotic microangiopathy (TMA) were reviewed. Patients’ ADAMTS13 activity levels were obtained, along with clinical/laboratory findings relevant to the PLASMIC score. Both PLASMIC scores and PLASMIC-LDH scores, in which LDH replaced traditional lysis markers, were calculated. We generated a receiver operator characteristics (ROC) curve and compared the area under the curve values (AUC) to determine the predictive ability of each score.
Results:
46 patients fulfilled the inclusion criteria, of which 34 had ADAMTS13 activity levels of <10%. When the patients were divided into intermediate-to-high risk (scores 5‒7) and low risk (scores 0‒4), the PLASMIC score showed a sensitivity of 97.1% and specificity of 58.3%, with a positive predictive value (PPV) of 86.8% and negative predictive value (NPV) of 87.5%. The PLASMIC-LDH score had a sensitivity of 97.1% and specificity of 33.3%, with a PPV of 80.5% and NPV of 80.0%.
Conclusion
Our study validated the utility of the PLASMIC score, and demonstrated PLASMIC-LDH as a reasonable alternative in the absence of traditional lysis markers, to help identify high-risk patients for treatment via plasma exchange.
5.Haemophilia care and outcome in a major haemophilia treatment centre in Malaysia
Yang Liang Boo ; Christopher Chin Keong Liam ; Kar Ying Yong ; Rui Jeat Fann ; Grace Wan Chieng Lee ; Gilbert Wilfred ; Jameela Sathar
The Medical Journal of Malaysia 2021;76(1):46-50
Introduction/Objective: The management of potential
treatment-related complications and bleeding events in
haemophilia is challenging in developing countries.
Providing optimal care among these patients improve their
quality of life (QOL) and life expectancy. This study explores
the demographic characteristics and treatment outcome in a
major haemophilia treatment centre in Malaysia.
Materials and Methods: A total of 260 patients were recruited
in this retrospective cross-sectional analysis. Clinical data,
including treatment regimens and outcome, were collected
and analysed.
Results: A total of 211 patients were diagnosed with
haemophilia A (HA) (severe disease, 72.5%) and 49 patients
had haemophilia B (HB) (severe disease, 65.3%). The median
age was 31 (IQR;2-84) years. Majority of the patients had at
least one episode of musculoskeletal bleeding since
diagnosis. The mean annual bleeding event (ABE) was 4.91
(SD±6.07) in 2018. Target joints were identified in 80.4% of
the patients. Chronic arthropathy and synovitis collectively
accounted for more than half of the musculoskeletal
complications. 30.1% of the patients had contracted
hepatitis C with less than half received treatment. Thirty-one
patients (16.8%) with severe haemophilia developed
inhibitor and 12 patients successfully underwent immune
tolerance induction. More than three-quarters of the severe
haemophilia patients were treated with factor concentrate
prophylaxis. The mean prophylaxis dose for HA and HB were
41.3 (SD±19.1) and 48.6 (SD±21.5) IU/kg/week, respectively.
In patients with severe disease, prophylaxis significantly
reduced the ABE (5.45,9.03;p=0.005).
Conclusion: The importance of utilising a low to moderate
dose regimen as prophylaxis in haemophilic patients is
highlighted in our study. Future studies should include QOL
assessment will further improve the management in
haemophilia.