Acute Myeloid Leukemia (AML) is a rare condition presenting in pregnancy and still poses a challenge in its management which involves maintaining a balance between maternal and fetal well-being. This study aims to create a local registry for hematological malignancy in pregnancy and to review outcomes of both maternal and fetal outcomes. Twelve pregnancies were identified from 11 patients. 3 pregnancies were terminated upon diagnosis and 1 miscarried. One maternal death prior to decision. Out of the 6 pregnancies continued, 5 received chemotherapy during pregnancy with all successfully achieving complete remission and were delivered at 33-37 weeks gestation. All babies delivered with good Apgar score with 1 reported neonatal death. One fetal anomaly reported in a patient exposed to Decitabine in early pregnancy. Chemotherapy can be safely administered during second or third trimester with delivery planned as close to term as possible. Decitabine should be avoided during pregnancy due to its potential teratogenicity.

Thalassaemia is one of the most common autosomal recessive blood disorders in the world and its carrier status is prevalent in nearly 15% of Malaysian population. The global and economic burden for lifelong care of those affected increases every year. Currently, there is no policy on thalassaemia-carrier screening for couples prior to marriage besides HIV/AIDS screening in Malaysia. Other countries such as Iran, Iraq, Turkey, Bahrain and Saudi Arabia have established a policy for thalassaemia prevention by conducting premarital thalassaemia screening. Zero thalassaemia cases in new-born child in Cyprus have proven that thalassaemia can be prevented. This study aimed to investigate the willingness of premarital Malays on premarital thalassaemia screening. A set of questionnaire was distributed to 57 persons at premarital course sites and wedding fairs and expositions held in Kuala Lumpur and Selangor. Components in the questionnaire included: 1) demographic 2) knowledge about thalassaemia, signs and screening method 3) attitudes towards thalassaemia premarital screening 4) practices of premarital thalassaemia screening. Analysis for the questionnaire was performed using IBM SPSS Statistics 23.0. A majority of the participants (75.44%) have never heard of thalassaemia as a medical condition. However, at least half of the participants (56.14%) agreed that premarital screening is needed to know their status of thalassaemia. In terms of facilities provided, widely covered campaigns about facilities of the screening test is also one of the factors that could encourage people to do premarital thalassaemia screening (35.09%). Even though premarital thalassaemia screening is not an obligation, 50.88% of participants agreed that it is important. The main reason people refused to do premarital thalassaemia screening was because they were afraid to know the test result and how it may affect their lives. Although preliminary, we have demonstrated that the level of knowledge concerning thalassemia among Malay Malaysians is still low. This may contribute to the low awareness about the effects of the disease and the importance of premarital thalassemia screening. However, most participants agreed that it is beneficial to perform the premarital thalassemia screening to identify individuals at risk of this disease. Nevertheless, many factors need to be taken into consideration if premarital thalassaemia screening is to be implemented in Malaysia as a national policy.

Introduction: Thrombotic thrombocytopenic purpura (TTP) is characterized by severe thrombocytopenia and microangiopathic hemolytic anemia. Although some patients also have a combination of fever and neurologic and/or renal manifestations, these are not essentially required for diagnosis. Ultra-large von Willebrand factor (vWf) multimers found in the patient's plasma are the basis for the platelet thrombi. Recent evidence has linked the abnormal fragments of vWf with deficiency of a plasma enzyme named vWf-cleaving protease or ADAMTS-13. Our aim is to raise awareness that clinical suspicion is paramount in clinching the diagnosis and highlight the importance of early detection and prompt management to improve patient's chance of survival. Case Report: We present a case of a 41-year-old Malay lady initially diagnosed with Severe Dengue, who subsequently demonstrated clinical and laboratory findings of TTP. When it was thought that she was recovering from the Dengue infection, she developed marked direct hyperbilirubinemia, which was quickly complicated by encephalopathy. Further laboratory investigations strongly pointed towards ongoing haemolysis with evidence of microangiopathic haemolytic anaemia. Despite, a within-range ADAMTS-13 level, three cycles of plasma exchange with fresh frozen plasma were promptly constituted to the patient of which she made a remarkable recovery. Discussion and Conclusion: The absence of severe ADAMTS-13 deficiency, as measured by current static assays, should not be used to argue against the use of plasma exchange, which has been highly effective even several years before the discovery of ADAMTS-13. Thus the determination of ADAMTS-13 activity has only a limited role in the diagnosis of TTP.