Genomic investigation of inherited thrombotic microangiopathy-aHUS and TTP.
Anoop K EnjetiTheo de MalmancheKent ChapmanAndrew ZiolkowskiPublished in: International journal of laboratory hematology (2021)
Thrombotic microangiopathies (TMA) are a heterogeneous group of red cell fragmentation syndromes characterized by a tendency for thrombosis and pathognomonic red cell fragments in peripheral blood, which results in thrombosis in the microvasculature due to endothelial damage. Genomic investigations into inherited TMAs are of diagnostic, prognostic and therapeutic value. Here, we present two cases that capture the importance of performing genomic testing in rare disorders. Treatment options for these conditions, such as plasma exchange and monoclonal antibodies against complement factors, are intensive and expensive health care interventions. The results of genomic investigation into rare TMAs can better inform the clinicians and their patients of prognosis and suitable personalized treatment options.
Keyphrases
- copy number
- peripheral blood
- healthcare
- end stage renal disease
- single cell
- pulmonary embolism
- cell therapy
- newly diagnosed
- ejection fraction
- chronic kidney disease
- physical activity
- peritoneal dialysis
- palliative care
- stem cells
- endothelial cells
- patient reported outcomes
- gene expression
- mesenchymal stem cells
- dna methylation
- genome wide