most cases of hematuria can be diagnosed by urinalysis , urine culture , urinary cytology , computed tomography , and rigid and flexible cysto - ureteroscopy .
we present a rare cause of hematuria that was challenging both to diagnose and to treat .
a 50-year - old woman presented with a 1-month history of painless gross hematuria without clots .
she was a known diabetic , hypertensive and ischemic heart disease patient and on regular treatment but not on any anticoagulants . on examination , she was hemodynamically stable .
urinalysis revealed significant hematuria . bleeding time , clotting time , liver function tests , urine culture and urine cytology were unremarkable .
cystoscopy with retrograde pyelogram revealed efflux of blood from the right ureteric orifice and a filling defect in the renal pelvis [ figure 1 ] .
biopsy of the floating brownish lesion with attachment to the renal pelvis using a semi - rigid ureteroscope was reported as fibrocollagenous material with no evidence of malignancy . however , the semi - rigid ureterorenoscope was unable to definitively rule out malignancy and flexible ureteroscope was planned .
the patient continued to have hematuria and received eight units of packed red blood cells and eight units of fresh frozen plasma .
however , she developed swelling and ecchymosis at the right thigh ( puncture site ) , which was confirmed to be a pseudoaneurysm in the duplex scan .
during anamnesis , it was revealed that the patient had bruises 2 weeks ago following insulin injection . filling defect rgp retrograde pyelogram at this juncture ,
her activated partial thromboplastin time ( aptt ) was raised but d dimer , fibrinogen and fibrin degradation product were normal .
the bethesda test confirmed acquired factor viii inhibitor syndrome with a value of 1.6 bethesda unit .
flexible ureteroscopy showed hyperemic patches and brownish floating material in the right renal pelvis that was completely removed using a dormia basket .
the patient was subsequently managed jointly with a hematologist and treated with prednisolone and factor viii inhibitor bypass activity ( feiba ) , and hematuria settled .
isolated presentation of hematuria is even rarer as these bleeding disorders are accompanied by bleeding at other sites .
acquired factor viii inhibitor syndrome is one such condition with an incidence of about one case per million per year . in this condition , autoantibodies are formed against factor viii .
acquired hemophilia is different from the congenital type as it has no genetic inheritance pattern and hemarthroses are seldom present .
it could cause significant morbidity with bleeding tendencies , and the mortality rate is 8 - 22% .
acquired hemophilia is associated with autoimmune disorders , malignancy ( solid , lymphoproliferative ) , skin diseases ( pemphigus , epidermolysisbullosa ) , infections , drugs and post - partum state , but these are mostly idiopathic .
the diagnosis is based on isolated prolongation of activated partial thromboplastin time not corrected by ptt correction study and confirmation by nijmegen modification of the bethesda assay showing reduced factor viii levels with evidence of factor viii inhibitor activity .
treatment is aimed at ( 1 ) controlling bleeding and its complications and ( 2 ) eradication of the inhibitor . fresh frozen plasma and cryoprecipitate will not control bleeding as they do not contain sufficient factor viii to overcome the inhibitor .
if the plasma levels of factor viii are raised to 30 - 50% in an acquired hemophilic patient , hemostasis could be generally achieved if the inhibitor assay is less than 5 bu ( bethesda unit ) .
1-deamino-8-d - arginine vasopressin ( ddavp ) or infusion of factor viii ( either human or porcine ) is used to achieve the higher levels . however , if high - titer antibodies are present ( more than 5 bethesda unit ) to obtain hemostasis , bypassing agents like either activated prothrombin complex concentrate ( apcc ) ( feiba ) or recombinant activated factor 7(rfvii ) ( novaseven ) is needed .
corticosteroids , cytotoxic drugs such as cyclophosamide , azathioprine , vincristine , cyclosporine and rituximab , and high - dose intravenous immunoglobulins are used alone or in combination to eradicate the autoantibodies .
the possibility of acquired hemophilia should be considered if elderly individuals present with severe hematuria , isolated aptt elevation , and when all other urological investigations were not contributory .
this case is presented in view of the rarity of acquired factor viii inhibitor syndrome , with only hematuria as the main symptom mimicking urological malignancy .
 a 50-year - old woman presented with gross hematuria for 1 month . 
 clinical examinations , laboratory investigations , ultrasound and contrast computed tomography were normal , except anemia . 
 cystoscopy revealed bloody efflux from the right side . 
 retrograde pyelogram showed filling defect in the renal pelvis and biopsy was inconclusive . 
 renal angiogram was normal . 
 she developed ecchymosis on the right thigh and arm with elevated activated partial thromboplastin time . 
 the partial thromboplastin time correction study and bethesda study confirmed the presence of acquired factor viii inhibitor ( acquired hemophilia ) . with flexible ureterorenoscopy 
 , the mass in the renal pelvis was removed and its histopathology revealed clotted blood . 
 the patient was subsequently managed with steroids and factor eight inhibitor bypass activity . 
INTRODUCTION
CASE REPORT
DISCUSSION
CONCLUSION