B TALBOT1,2, J OTHMAN2, RCF CHAN2, M KRISHNASWAMY2, K ARCHER2, V CHEN2,3, S SEN2
1The George Institute, Sydney, NSW; 2Concord Repatriation General Hospital, Sydney, NSW; 3The University of Sydney, Sydney, NSW.
Background:
Thrombotic microangiopathy (TMA) describes a pathological process of microvascular thrombosis, consumptive thrombocytopenia and microangiopathic haemolytic anaemia leading to end-organ ischaemia and infarction. Patients may present with acute renal failure and/or cerebral dysfunction. TMA is a feature of several clinical disorders and rarely can be associated with malignancy.
Case Report: A 71 year old gentleman presented with flank pain, fevers and deteriorating renal function. He was known to have unilateral hydronephrosis and a poorly defined hilar soft tissue density in the left kidney.
On arrival he was septic and haemodynamically unstable. Investigations revealed microcytic anaemia, acute kidney injury and raised inflammatory markers. Significant fragmentation was seen on the blood film, reflected in the low mean corpuscular volume (MCV). Iron deficiency was excluded. Repeat imaging did not explain the renal deterioration. Antibiotics were given and haemodialysis initiated due to anuria, fluid overload and hyperkalaemia.
Biopsy of the unobstructed kidney revealed florid TMA without involvement of non-glomerular vessels. Serological markers did not reveal an underlying cause.
Ongoing sepsis required intensive care support and left nephrostomy insertion. Spontaneous bleeding from his left renal artery required angiographic embolisation and subsequent open nephrectomy. Histology showed high grade invasive papillary urothelial carcinoma with evidence of infarction following embolisation. Disseminated disease was later confirmed.
An increase in MCV and haemoglobin paralleled the reduction in fragmentation seen following embolisation and nephrectomy suggesting direct effect of these treatments on the TMA.
Despite these interventions he deteriorated and died with palliative care support.
Conclusion: We present a case of acute kidney injury secondary to TMA associated with urothelial cancer, which is the first described and showed haematological improvement with treatment of the malignancy.