Acquired hemophilia complicated by cardiorenal syndrome type 3
A 65-year-old female with history of healed traumatic amputation of left 5th toe 2 months back, transferred to our hospital on mechanical ventilator and dopamine infusion with the complaints of hematuria for 1.5 months, subcutaneous hemorrhage for 15 days, oliguria for 3 days, and respiratory failure for 1 day. At presentation, she was alert, afebrile, and tachycardic, with high oxygen requirement. On examination, she was volume overloaded with multiple ecchymotic patches over limbs [], back, and buttocks.\nUltrasonography of abdomen showed hepatomegaly, right pleural effusion, bilateral hydronephrosis, and hydroureter with no calculus or focal mass lesion and normal blood flow bilaterally in the renal arteries and veins. ECG showed tall T-waves and arterial blood gas analysis (ABG) indicated metabolic acidosis with a base deficit of 6.8. Other investigations were hemoglobin 8.7 gm/dL, total leukocyte count 12,100/μL, platelet 297,000/μL, international normalized ratio 1.1, activated partial thromboplastin time (aPTT) 55.4 s (control 27 s), urea 117 mg/dL, creatinine 7.1 mg/dL, sodium 120 mEq/L, potassium 5.6 mEq/L. Chest radiograph showed bilateral pleural effusions with basal atelectasis []. Her liver function tests were normal. Echocardiography showed global hypokinesia with dilated cardiac chambers and left ventricular ejection fraction (LVEF) 25-30% []. Patient was immediately administered heparin-free hemodialysis with low sodium dialysate and a target ultrafiltrate of 3 L. Packed red blood cells (RBCs) and fresh frozen plasma were transfused during dialysis. All serological tests were normal.\nOver the next 3 days, her general condition improved with correction of dyselectrolytemia, discontinuation of vasopressors, and weaning from the ventilator; but she continued to have hematuria with a raised aPTT. Blood, urine, and tracheal secretions cultures were negative. Blood FVIII level was less than 1% and FVIII inhibitor was detected in serum Recombinant activated factor VII (rFVIIa) was given in a dose of 90 μg/kg as intravenous bolus and repeated after 4 h. Hematuria was relieved followed by normalization of creatinine. She was started on tablet metoprolol 25 mg/day and tablet prednisolone 50 mg/day. Patient was shifted out of ICU on 5th day of admission.\nDrug treatment for cardiac failure was optimized. Repeat echocardiography on the 10th day showed LVEF of approximately 35%. Dobutamine stress echocardiography was negative for reversible ischemia with significant improvement in LVEF. The patient was discharged on the 12th day of hospitalisation.