P122 Ph-negative chronic myeloproliferative disorder (idiopathic myelofibrosis) as one of the reasons of the Budd-Chiari syndrome. Clinical case of a young patient
✍ Scribed by M.A. Sokolova; N.D. Khoroshko; N.V. Tsvetaeva; M.D. Sanatko
- Publisher
- Elsevier Science
- Year
- 2007
- Tongue
- English
- Weight
- 59 KB
- Volume
- 21
- Category
- Article
- ISSN
- 0268-960X
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✦ Synopsis
Introduction: Uric acid stones comprise less than 5% of all urinary calculi. Most patients with uric acid calculi do not have hyperuricemia. Elevated uric acid levels may occur due to dehydration and excessive purine intake. A consistently low urinary pH is a major risk factor. Patients with gout or myeloproliferative diseases and those treated for malignant conditions with cytotoxic drugs have higher incidence of uric acid lithiasis. In patients osteomyelofibrosis (AMM Agnogenic myeloid metaplasia) hyperuricemia due to increased extramedularry hematopoiesis is absolute indication for immediate chemotherapeutic regiment. But, obstructive bilateral ureteral cvalculosis with acute post-renal insufficiency is extremly rare, but may be life-threatening first sign of AMM. Case Report: A 47 years old male patient was admitted to the Department of urology with symptoms od fatigue and anuria. Laboratory findings showed leuko-and erythrocytosis with teadrop poikilocytosis, increased levels of urea-BUN (32 mmol/l) and creatinine (766 mmol/l) as well as uric acid (820 umol/l) and very low cholesterol (2.5 mmol/l). Clinical examination revealed signs of plethora and enlarged spleen. Immediate abdomunal ultrasoud was perforemed and confirmed extreme splenomegaly (max diameter 26 cm) but also showed bilateral hydronephrosis of grade II III with two stones in proximal part of right ureter and one in proximal part of left ureter as well empty bladder. Stnose were not seen on plain film. As patient was in complete anuria, with further rapid elevation of BUN and creatinine levels, bilateral ureteral stent was applicated together with extensive hydration, urine alkalinization and administration of allopurinol. Bone marrow biopsy was performed and histopathological diagnosis was: Hypercellulary phase of AMM. Patient was trasfered to the Departemnt of hematology for further treatment. Four days later, values of BUN and cr3eatinine turned to normal values togenther with normal diuresis.
Conclusion:
In this paper we presented a very rare case of bilateral ureteral uric acid calculosis and consecutive acute post-renal insufficiency caused by hyperuricemia in previously undiagnosed patient wit AMM.