Thrombotic microangiopathic hemolytic anemia in a patient with SLE: diagnostic difficulties.

BACKGROUND: A 19-year-old woman with newly diagnosed systemic lupus erythematosus (SLE) presented with hemolytic anemia, thrombocytopenia, hypertension, tonic-clonic seizures, blurry vision, nephrotic syndrome and renal insufficiency. INVESTIGATIONS: At a general hospital, the investigations included brain MRI, echocardiography, laboratory tests including measurement of the amount of protein excreted daily, platelet count, levels of lactate dehydrogenase, creatinine and anticardiolipin antibodies, direct Coombs' test, peripheral blood smear, and measurement of blood pressure. At a tertiary institution the investigations included physical examination, electroencephalography, brain MRI, magnetic resonance angiography, repetition of laboratory tests plus measurement of von Willebrand factor-cleaving protease activity, measurement of levels of antibodies to double-stranded DNA and platelets, and renal biopsy. DIAGNOSIS: Thrombotic microangiopathic hemolytic anemia with a possible underlying diagnosis of malignant hypertension, antiphospholipid antibody syndrome, catastrophic antiphospholipid antibody syndrome, thrombotic thrombocytopenic purpura, or active SLE. MANAGEMENT: At the general hospital, therapy included a single dose of intravenous cyclophosphamide 500 mg, eight daily plasma exchange treatments, three daily infusions of methylprednisolone 1 g followed by methylprednisolone 60 mg every 8 h, an infusion of rituximab 657 mg and ultrafiltration via hemodialysis. At the tertiary institution, therapy included an infusion of cyclophosphamide 650 mg, aspirin 81 mg daily, prednisone 40 mg daily, mycophenolate mofetil 750 mg twice daily, and aggressive management of hypertension.