Thrombotic thrombocytopenic purpura in the first trimester of pregnancy
A 21-year-old primigravida presented at 10 weeks of gestation with pain in abdomen, hematuria, oliguria, and altered sensorium with episodes of generalized tonic clonic seizures. On general examination her blood pressure records were normal. She had pallor, tachycardia and facial puffiness. Laboratory investigations showed anemia with hemoglobin of 4.7 gm% and thrombocytopenia with a platelet count of 29,000/cumm. On peripheral blood film there was anisopoikilocytosis with abundant schistocytes. Renal function tests were deranged with blood urea of 131mg/dl, serum creatinine of 6.2 mg/dl and spot urine albumin was 2+. Total serum bilirubin was 2.8 mg/dl, indirect bilirubin was 2 mg/dl. Levels of plasma lactate dehydrogenase were very high the value being 1062 IU/L (normal range 70-150 IU/L). Ultrasound of abdomen showed increased echogenicity of the pelvicalyceal system of both kidneys. There was no evidence of renal vein thrombosis on renal Doppler. Her thrombophilia screen was negative. This screen included a normal lupus anticoagulant factor, antinuclear antibody and anticardiolipin antibodies. Her coagulation studies were also normal. Unfortunately the measurement of ADAMTS-13 levels was not carried out due to nonavailability of this investigation in our laboratory.\nIn view of a deteriorating neurological condition, a lumbar puncture was done and the cerebrospinal fluid analysis was normal. Hemodialysis was started in view of oliguria and azotemia after nephrology consultation. The next day she needed mechanical ventilation in view of poor respiratory efforts. Platelet count further fell down to 12,000/cumm. On the basis of symptoms and laboratory tests, a clinical diagnosis of TTP was made. Treatment with daily TPE which comprised replacement of 30ml/ kg plasma in combination with infusion of fresh frozen plasma (FFP 15ml/kg) was started using Cobe Spectra TPE machine. After four cycles of TPE, her platelet count rose to 1.5 lakh/cumm. The platelet count was subsequently monitored initially weekly and then biweekly till term and the values remained normal. Hemoglobin increased to 7.9 gm% and there was decrease in number of schistocytes on peripheral blood film. Plasma lactate dehydrogenase levels decreased to 180 IU/L. Urine output improved. She was extubated within next 48 hrs. Her condition was monitored till day 20 of treatment and there was progressive improvement in clinical and laboratory parameters. She thus did not receive any further plasma exchange. Tablet folic acid 5 mg/ day, supplemental iron and calcium were continued. Corticosteroids were not used. Patient was discharged on day 24 of admission in a satisfactory condition. Here pregnancy was monitored regularly in the medicosurgical unit at our institution. At 37 weeks of gestation, her blood pressure was found to be high in the range of 130/90 – 140/100 for which her pregnancy was terminated. She had intrapartum fetal distress for which an emergency Cesarean section was done and she delivered a healthy 2.4-kg baby. She received postpartum prophylaxis for deep vein thrombosis (heparin 5000 IU subcutaneously twice a day). Both the mother and baby were discharged 1 week later in a satisfactory condition.