Chronic myeloid leukaemia (CML) is a myeloproliferative neoplasm arising from a pluripotent haematopoietic stem cell. It is associated with an oncogenic fusion gene BCR-ABL, encoding a protein with tyrosine kinase activity. 1 The emergence of tyrosine kinase inhibitors (TKIs) such as imatinib, nilotinib, dasatinib, and ponatinib has revolutionised the treatment and improved overall survival of patients with CML. Pericardial and pleural effusion, pulmonary oedema, left ventricular failure, arrhythmia, and coronary heart disease have rarely been reported in clinical trials with nilotinib. 2 We report a case of acute coronary syndrome in a young woman treated with nilotinib. A 33-year-old woman presented to Hospital Sultanah Nora Ismail, Johor, Malaysia in February 2017 with first-episode sudden-onset, left-sided chest pain that lasted more than 30 minutes and was associated with nausea, vomiting, palpitations, and profuse sweating. She had been diagnosed with CML 2 years previously and had initially received imatinib with low Sokal score. Her BCR-ABL1 fusion transcript was more than 0.1% after 1 year of treatment, and thus, her treatment was changed to nilotinib 400 mg twice daily, as a second-line TKI. Tyrosine kinase domain mutation analysis was not performed due to lack of resources. She was compliant with medication and showed a good response with her BCR-ABL1 transcript dropping to less than 0.1% after 3 months of therapy. She had no other significant medical or family history. On arrival, she was haemodynamically stable and physical examination was normal. Her initial blood investigations showed normal haemoglobin level (13.6 g/dL), white cell count (8.3 × 10 9 /L), platelet count (240 × 10 9 /L), kidney, and liver function. Her initial electrocardiogram showed T wave inversion over V2 to V6 with a Q wave in lead III. Subsequent electrocardiograms showed evolving ischaemic changes with ST depression over V2 to V6. Her
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.
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