CASE REPORT: We described a patient with ET whose disease evolved into MDS with fibrosis and complex karyotype after 15 years of stable disease. She was asymptomatic and was on hydroxyurea (HU) treatment until recently when she presented with worsening anaemia. Physical examination showed mild splenomegaly. Full blood picture showed leukoerythroblastic picture with presence of 3% circulating blasts and background of dysplastic features such as hypogranular cytoplasm and nuclear hyposegmentation of neutrophils. The bone marrow aspiration was haemodiluted but revealed presence of 6% blast cells, trilineage dysplasia and predominant erythroid precursors (60%). Trephine biopsy showed no excess of blast cells and normal quantity of erythroid precursors, but there was increased in fibrosis (WHO grade 2) and presence of dysmegakaryopoeisis such as nuclear hypolobation, multinucleation and micromegakaryocytes. Cytogenetic study showed complex karyotype; monosomy of chromosome 2, chromosome 5, chromosome 18 and presence of a marker chromosome (42~44, XX,-2,-5,-18,+mar). Fluorescence in situ hybridisation (FISH) showed 5q deletion (CSF1R and EGR1).
CONCLUSION: The findings were consistent with transformation of ET to MDS with fibrosis and complex karyotype. ET progression to MDS is considered rare. The presence of complex karyotype and fibrosis in MDS are associated with unfavourable outcome.
METHODS: A deterministic, prevalence-based model was used to project the annual health burden and direct medical costs associated with recurrent ASCVD events over a 10-year horizon. The target population included adults (≥30 years) with established ASCVD and uncontrolled LDL-C levels (>1.8 mmol/L). The model comprised 3 modules: population size projection, recurrent ASCVD risk calculation (by means of the Secondary Manifestations of ARTerial disease [SMART] risk model), and direct medical and productivity cost estimation. The current status quo and a scenario with a 50% improvement in mean LDL-C were compared.
RESULTS: We projected over 800 000 adults with established ASCVD in 2023, increasing to approximately 1.4 million by 2032. Under the status quo, about 55 000 recurrent ASCVD events were expected within 10 years, with significant direct medical costs and productivity losses. Improved LDL-C control could potentially reduce recurrent events by 7000 cases (13% reduction), prevent 2100 premature deaths, and save approximately 32 400 years of life. Economically, this could lead to a reduction of approximately 72 million MYR in direct medical costs and a gain of approximately 132.4 million MYR in productivity over a decade.
CONCLUSIONS: Optimizing LDL-C control in high-risk patients with ASCVD presents a critical opportunity to reduce health and economic burdens in Malaysia.