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).