CASE REPORT
A 40-year-old African female was referred to the Emergency on account of stroke confirmed by a CT scan. She presented with slurred speech, facial asymmetry and left upper and lower limb weakness of a day’s duration. The patient had been diagnosed of hypertension 10 years ago but has been non-compliant with her medication 2 weeks prior to her presentation. Her medications included amlodipine and lisinopril. She admitted to headaches, dizziness, palpitations, and easy fatigue and reported menorrhagia for the past 10 years. Past medical history included two stroke events 5 years ago and encephalitis at age 8 months. There was a family history of hypertension and diabetes but no history of bleeding disorder.
On physical examination, she was neither in respiratory distress nor jaundiced, however was severely pale. She had purpuric patch on the flexural areas of both elbows and knees and posterior aspect of the lower legs. The patient was afebrile with a temperature of 36.8 °C; pulse rate of 81 beats per minute; respiratory rate of 20 cycles per minute; and blood pressure 103/67 mmHg at presentation. Power grading in her left upper and lower limbs were 1/5. All other systems were unremarkable.
The complete blood count revealed a low haemoglobin level of 4.3 g/dl (11.5 – 14.5), platelet count of 18 x 109/L (150 – 450 x 109/L), white cell count of 10.47 x 109/L and a normal renal function. Samples were taken for blood film comment, blood film for malaria parasite, LDH, uric acid, clotting profile, HIV, ANA., Hepatitis B and C. Additionally, electrocardiogram, echocardiogram, abdominopelvic ultrasound, and a chest x-ray were requested. She was transfused with 1 unit of whole blood and neurological physiotherapy started. On days 2 and 3 of admission, her power improved to 4/5.
On day 4, the patient developed tonic-clonic seizures and she gradually developed jaundice the same day. Repeat complete blood count showed Hb of 6.2g/dl, WBC of 13.5 x 109/L, Platelet count of 22 x 109/L. Clotting profile results showed INR of 1.16, PT – 9.8s, APTT – 33.9s, PTT – 0.92s. LDH was 2,6111 u/L (135 – 214), Uric acid was 0.4 mmol/l (0.13 – 0.39), both direct(19.86 umol/l) and indirect (34.7 umol/l) bilirubin were high with low globulin levels (25.7). HIV, Hepatitis B and C, ECG, chest x-ray, blood film for malaria parasite, AST, ALT, AST, ANA were all normal. A Thyroid function test was normal. The result of the blood film comment showed normocytic normochromic RBCs, schistocytes ++ with occasional normoblast, few polychromatic cells, increased WBC count mostly neutrophils with a left shift and reduced platelets with no clumps seen suggestive of microangiopathic hemolytic anaemia. ADAMTS 13 assay could not be conducted as requested due to inadequate funds. Alternatively, the TTP PLASMIC score was used for confirmation which yielded a total score of 7 (high risk) and a 96.2% risk of severe ADAMTS13 deficiency (≤10%). A repeat of renal function test yielded urea of 31.7 mmol/l (2.1 – 7.1) and creatinine of 492µmol/l (62 – 106). TTP was diagnosed, and the patient was transfused with 3 units of whole blood, 4 units of fresh frozen plasma and had 3 days of pulsed methylprednisolone. Subsequently, she had a total of 7 sessions of plasmapheresis and 3 sessions of hemodialysis with concomitant oral prednisolone 80mg and 1g of 10% calcium gluconate prior to plasmapheresis. Her GCS at the end of the sessions rose to 15/15. The patient was not pregnant at any time during her illness (Table 1and Table 2).