Intractable hematuria secondary to systemic amyloidosis with bladder involvement.

OBJECTIVE: To present the therapeutic management of intractable hematuria secondary to systemic amyloidosis with bladder involvement. METHODS: We describe the clinical case, the medical management, the endo-urological technique used, and the results supported by relevant published literature. RESULTS: A 50-year-old woman with a 20-year history of rheumatoid arthritis in chronic treatment with corticosteroids and non-steroidal anti-inflammatory drugs in addition to chronic renal insufficiency not requiring hemodialysis. Twenty-four hours after resection of a hepatic hydatid cyst she presented intractable hematuria. The ultrasound and CT scan showed the formation of a large blood clot in the bladder not affecting the upper urinary tract. An intra-operative cystoscopy revealed a distended bladder showing signs of inflammation with diffuse, widespread bleeding. Hemostasis was achieved and a biopsy of the mucosa was taken, associated to bladder irrigation with potassium alum as a hemostatic. Given the persistence of the hematuria, further revision in the operating room as well as blood transfusion were carried out and, due to the hemodynamic instability that could not be controlled, finally selective embolization was performed. Intravesical instillation of dimethyl sulphoxide every 72 hours was used to control any remaining hematuria. The biopsy showed bladder amyloidosis. The addition of intravenous steroids and orally administered colchicine successfully controlled the patient's clinical status. CONCLUSIONS: Secondary amyloidosis of the bladder is a condition associated with hematuria that is difficult to manage. Hematuria control is often difficult, requiring aggressive treatment in addition to more conservative approaches.

Hematuria incoercible generada en la amiloidosis sistémica con afectación vesical / Intractable hematuria secondary to systemic amyloidosis with bladder involvement

OBJETIVO: Presentar el manejo terapéutico de la hematuria incoercible generada en la amiloidosis sistémica con afectación vesical. MÉTODO: Descripción del caso clínico, el manejo médico, la técnica endourológica utilizada y de los resultados con apoyo de la literatura publicada al respecto. RESULTADOS: Mujer de 50 años con antecedentes personales de artritis reumatoide (AR) de 20 años de evolución en tratamiento crónico con corticoides y AINES así como insuficiencia renal crónica sin necesidad de hemodiálisis, que tras 24 horas de la resección de un quiste hidatídico hepático presenta hematuria incoercible. Ecografía y TAC revelan gran coágulo vesical organizado sin repercusión de la vía urinaria. La cistoscopia intraoperatoria muestra una vejiga distendida de aspecto inflamatorio con sangrado difuso generalizado. Se realiza hemostasia y toma de biopsias de la mucosa asociando al lavado vesical alumbre potásico como hemostático. Dada la persistencia de la hematuria se procede a nueva revisión en quirófano más transfusión de hemoderivados en vista de la inestabilidad hemodinámica sin lograr control de la misma por lo que se realiza embolización selectiva. Se asocia instilación vesical con DMSO cada 72 hrs. para el control de la hematuria remanente. La biopsia revela el hallazgo de amiloidosis vesical agregándose al tratamiento corticoide intravenoso y colchicina oral controlando satisfactoriamente la clínica de la paciente. CONCLUSIONES: La amiloidosis vesical secundaria es una entidad que cursa con hematuria de difícil manejo. El control de la hematuria suele ser difícil por lo que además de los tratamientos conservadores a veces requiere de tratamientos más agresivos

OBJECTIVE: To present the therapeutic management of intractable hematuria secondary to systemic amyloidosis with bladder involvement. METHODS: We describe the clinical case, the medical management, the endo-urological technique used, and the results supported by relevant published literature. RESULTS: A 50-year-old woman with a 20-year history of rheumatoid arthritis in chronic treatment with corticosteroids and non-steroidal anti-inflammatory drugs in addition to chronic renal insufficiency not requiring hemodialysis. Twenty-four hours after resection of a hepatic hydatid cyst she presented intractable hematuria. The ultrasound and CT scan showed the formation of a large blood clot in the bladder not affecting the upper urinary tract. An intra-operative cystoscopy revealed a distended bladder showing signs of inflammation with diffuse, widespread bleeding. Hemostasis was achieved and a biopsy of the mucosa was taken, associated to bladder irrigation with potassium alum as a hemostatic. Given the persistence of the hematuria, further revision in the operating room as well as blood transfusion were carried out and, due to the hemodynamic instability that could not be controlled, finally selective embolization was performed. Intravesical instillation of dimethyl sulphoxide every 72 hours was used to control any remaining hematuria. The biopsy showed bladder amyloidosis. The addition of intravenous steroids and orally administered colchicine successfully controlled the patient’s clinical status. CONCLUSIONS: Secondary amyloidosis of the bladder is a condition associated with hematuria that is difficult to manage. Hematuria control is often difficult, requiring aggressive treatment in addition to more conservative approaches