Macroscopic Haematuria

Macroscopic (visible) haematuria is urine discoloured red, pink, brown or tea coloured by blood, appearing when more than about 1 mL of blood is present in 1 L of urine. It must be confirmed by dipstick and microscopy, as red urine may be pigment: myoglobin, haemoglobin, porphyrin, beetroot, rifampicin, phenazopyridine or senna.

It is a symptom of significant pathology until proven otherwise. Painless visible haematuria carries a malignancy risk of 10% to 20%, mainly urothelial carcinoma of the bladder, upper tract urothelial carcinoma and renal cell carcinoma.

Other causes include calculi, infection (including haemorrhagic cystitis from adenovirus, BK virus, cyclophosphamide or ifosfamide), prostatic bleeding, trauma, glomerular disease (IgA nephropathy, post-infectious, Alport syndrome), polycystic cyst rupture, papillary necrosis, renal infarction, sickle cell disease, schistosomiasis and coagulopathy or anticoagulants.

Timing in the stream localises the source:

• Initial haematuria: urethra
• Terminal haematuria: bladder neck or prostate
• Total haematuria: bladder or upper tract
• Clots: usually bladder or lower tract (worm like clots suggest an upper tract source)

• Painless: bladder or renal tumour, polycystic kidney disease, glomerular disease
• Painful with colic: calculus or clot passage
• With dysuria and frequency: cystitis
• With sore throat 24 to 72 hours earlier (synpharyngitic): IgA nephropathy; 1 to 3 weeks after throat infection: post-infectious glomerulonephritis
• Weight loss, smoking, flank mass: malignancy
• Associated oedema, hypertension and cola colour: glomerular disease
• Recent instrumentation, radiotherapy, cyclophosphamide, trauma

Red flags: clot retention (suprapubic pain, inability to void), haemodynamic instability, falling haemoglobin, anticoagulation, anaemia, smoking, age over 45, loin mass, hydronephrosis.

• Haemodynamic assessment, haemoglobin, coagulation screen, group and save, creatinine and urea
• Urinalysis and microscopy (dysmorphic cells and casts), culture, urine cytology where indicated
• Ultrasound with bladder scan for residual volume and hydronephrosis
• CT urogram (non-contrast phase for stones, then contrast phases) for all adults aged 45 or over, smokers and those with unexplained haematuria; MR urography or ultrasound with retrograde studies if contrast is contraindicated
• Flexible cystoscopy for all adults with visible haematuria without a clear benign cause
• Albumin:creatinine ratio, complement, ASO titre, and biopsy only when glomerular features
• Haemoglobin electrophoresis if sickling is suspected

Differentials: pseudohaematuria (pigment), vaginal or perianal bleeding contamination, myoglobinuria (rhabdomyolysis; dipstick positive, no red cells), haemoglobinuria (haemolysis).

Emergency

• Assess airway, breathing and circulation; two large bore cannulae, crossmatch.
• Resuscitation with balanced crystalloid 500 mL boluses; transfuse red cells for haemoglobin below 70 g/L (below 80 g/L with cardiovascular disease) or ongoing instability.
• Reverse anticoagulation where life threatening: vitamin K 5 to 10 mg intravenously and prothrombin complex concentrate 25 to 50 units/kg for warfarin; idarucizumab 5 g for dabigatran; andexanet alfa or prothrombin complex concentrate 50 units/kg for factor Xa inhibitors.
• Clot retention: insert a 22 to 24 Fr three way catheter, perform manual bladder washout with 50 mL aliquots of 0.9% sodium chloride until the clots are cleared, then continuous bladder irrigation at a rate that keeps effluent pink clear.
• Tranexamic acid 500 mg to 1 g orally two to three times daily may be used for refractory lower tract bleeding; avoid in upper tract bleeding because clots can obstruct the ureter.

Specialist

• Cystoscopic clot evacuation and diathermy, transurethral resection of bladder tumour, ureteroscopy for stones, angioembolisation of renal bleeding, nephrectomy for uncontrolled bleeding.
• Haemorrhagic cystitis from cyclophosphamide or ifosfamide: hyperhydration (at least 3 L daily), mesna at 60% to 100% of the cyclophosphamide dose given in divided doses; refractory cases need intravesical alum or hyperbaric oxygen.

Urgent referral

• Urgent urology assessment for any adult 45 or over with unexplained visible haematuria, within 2 weeks.
• Nephrology for glomerular causes.

Special groups

• Children: glomerular causes predominate; check blood pressure, oedema, complement and recent infection. Malignancy is rare.
• Anticoagulated patients: haematuria needs full evaluation; do not attribute it to the drug.
• Sickle cell disease: hydration, alkalinisation, avoid antifibrinolytic agents in upper tract bleeding.