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Acquired factor VIII inhibitor syndrome: A rare cause of hematuria.

Kannan MS, Raj Kumar TR, Subramanian S - Indian J Urol (2015 Jan-Mar)

Bottom Line: Retrograde pyelogram showed filling defect in the renal pelvis and biopsy was inconclusive.The partial thromboplastin time correction study and Bethesda study confirmed the presence of acquired factor VIII inhibitor (acquired hemophilia).The patient was subsequently managed with steroids and Factor eight inhibitor bypass activity.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, SRM Medical College, Kattankulathur, Tamil Nadu, India.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus

Filling defect RGP retrograde pyelogram
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Figure 1: Filling defect RGP retrograde pyelogram

Mentions: A 50-year-old woman presented with a 1-month history of painless gross hematuria without clots. She had no history of loss of weight or appetite. 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. Abdominal examination was unremarkable. Full blood count showed hemoglobin to be 10.6 g/dL. Urinalysis revealed significant hematuria. Bleeding time, clotting time, liver function tests, urine culture and urine cytology were unremarkable. Ultrasound and contrast CT KUB showed no calculus or mass lesions. 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. She underwent super selective renal angiogram that was normal. However, she developed swelling and ecchymosis at the right thigh (puncture site), which was confirmed to be a pseudoaneurysm in the duplex scan. She also started developing ecchymotic patches around the intravenous puncture sites. During anamnesis, it was revealed that the patient had bruises 2 weeks ago following insulin injection.


Acquired factor VIII inhibitor syndrome: A rare cause of hematuria.

Kannan MS, Raj Kumar TR, Subramanian S - Indian J Urol (2015 Jan-Mar)

Filling defect RGP retrograde pyelogram
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4300578&req=5

Figure 1: Filling defect RGP retrograde pyelogram
Mentions: A 50-year-old woman presented with a 1-month history of painless gross hematuria without clots. She had no history of loss of weight or appetite. 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. Abdominal examination was unremarkable. Full blood count showed hemoglobin to be 10.6 g/dL. Urinalysis revealed significant hematuria. Bleeding time, clotting time, liver function tests, urine culture and urine cytology were unremarkable. Ultrasound and contrast CT KUB showed no calculus or mass lesions. 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. She underwent super selective renal angiogram that was normal. However, she developed swelling and ecchymosis at the right thigh (puncture site), which was confirmed to be a pseudoaneurysm in the duplex scan. She also started developing ecchymotic patches around the intravenous puncture sites. During anamnesis, it was revealed that the patient had bruises 2 weeks ago following insulin injection.

Bottom Line: Retrograde pyelogram showed filling defect in the renal pelvis and biopsy was inconclusive.The partial thromboplastin time correction study and Bethesda study confirmed the presence of acquired factor VIII inhibitor (acquired hemophilia).The patient was subsequently managed with steroids and Factor eight inhibitor bypass activity.

View Article: PubMed Central - PubMed

Affiliation: Department of Urology, SRM Medical College, Kattankulathur, Tamil Nadu, India.

ABSTRACT
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.

No MeSH data available.


Related in: MedlinePlus