Subcapsular liver hematoma as a complication of an atypical hemolytic uremic syndrome.

نویسندگان

  • Emanuel Ferreira
  • Nuno Oliveira
  • Maria Marques
  • Helena Pinto
  • Ana Santos
  • Armando Carreira
  • Mário Campos
چکیده

Atypical hemolytic uremic syndrome (aHUS) is a rare, lifethreatening systemic inflammatory disease that presents classically with microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury.1 Extra renal manifestations are observed in 20% of patients.2 A 42-year-old woman with unremarkable past medical history presented in our hospital reporting a 6-day history of headache, nausea and vomiting. Physical examination showed hypertension (220/120mmHg), cutaneous pallor and moderate lower limbs edema. Laboratory results revealed anemia (hemoglobin 8.4 g/dl), thrombocytopenia (76,000/ l), severe azotemia (urea 16.9mmol/l, creatinine 448.8 mol/l), schistocytosis, a negative Coombs test, low blood haptoglobin (<0.07 g/L) and high lactate dehydrogenase levels (1234U/l). Renal ultrasonography was normal. Blood pressure was hardly controlledwith oralmedication. A diagnosis of acute thrombotic microangiopathy (ATM) was made and daily plasma exchange (PEX) was started. Investigations for secondary causes of ATM (pregnancy, auto-immune disease, malignancy, drug-induced), infectioninducedHUS and thrombotic thrombocytopenic purpurawere normal. A presumptive diagnosis of aHUS was made and the administrative process of Eculizumab acquisition was initiated. On the 15th day of admission (D15), hemodialysis was started due to progressive renal failure. All attempts to stop PEX resulted in increased hemolytic activity, forcing to maintain 3 sessions a week. On the D72, after performing 44 PEX sessions, we were still waiting for Eculizumab acquisition. Attending to clinical and analytical stability (Fig. 1), the patient was discharged home to continue hemodialysis and PEX three times a week as an outpatient. Four days after discharge, she was admitted in the emergency room with a 12-hour history of severe right upper quadrant pain and vomiting without history of trauma. Laboratory results revealed stabilized hemoglobin (11.1 g/dl) and both normal platelet count (157,000/mm3) and coagulation tests. Abdominal ultrasonography and Computed Tomography scan showed a large subcapsular liver hematoma (SLH) (Fig. 2). She was transferred to the Intensive Care Unit (ICU). Attending to hemodynamic stability, a conservative approach was attempted. PEX was suspended to prevent increased

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عنوان ژورنال:
  • Nefrologia : publicacion oficial de la Sociedad Espanola Nefrologia

دوره 35 3  شماره 

صفحات  -

تاریخ انتشار 2015