Case 1
A 38-year-old Tunisian woman with no medical history presenting with
dizziness and ecchymosis in her upper limbs was referred to the
hematology department. The patient reported that she had received a
first dose of an inactivated virus COVID-19 vaccine Sinopharm
(BBIBP-CorV) twenty days before symptom onset. Laboratory findings
revealed hemoglobin 6 g/dl, platelet count 6×109/L,
lactate dehydrogenase (LDH) 1074 UI/L, haptoglobin 0.54 g/l, creatinine
66 µmol/L, urea 20.7 mg/dl, total bilirubin 3.75 mg/dl and indirect
bilirubin 2.88 mg/dl. Peripheral blood smear showed schistocytes (1 to
2%). During her hospital stay, the patient presented left hemi-body
heaviness and dysarthria. A brain MRI revealed an ischemic stroke in the
territory of the infero-posterior cerebellar artery. A curative
anticoagulation was started. A few hours after ICU admission, the
patient presented a sudden generalized tonico-clonic seizure with status
epilepticus requiring her intubation. Glycemia and electrolytes were
within the normal ranges. The patient was promptly given clonazepam and
intravenous sodium valproate. Analgo-sedation was prolonged with
remifentanyl and midazolam to achieve a Richmond Agitation Sedation
Scale (RASS)17 at -5 to control the status epilepticus
and obtain patient-ventilator synchronization. The presence of
thrombocytopenia, hemolytic anemia and neurologic symptoms were
indicative of a presumptive diagnosis of TTP. The PLASMIC score, used to
identify patients with ADAMTS-13 deficiency in suspected TTP patients,
was at 6 (range, 0-7) indicating a high risk of severe ADAMTS-13
deficiency ˂10%. The patient was promptly treated by methylprednisolone
1000 mg daily for three consecutive days, then 1 mg/kg/day in
combination with daily plasma exchange therapy (PEX).
Infectious screening tests (e.g., Human immunodeficiency virus (HIV),
hepatitis, SARS-CoV-2, Epstein-Barr Virus, and Cytomegalovirus) were
negative. Autoimmunity investigations revealed severe
ADAMTS-13 deficiency (6%) with
positive anti ADAMTS-13
autoantibodies more than 15 U/ml (normal<12 U/ml) confirming
the diagnosis of an acquired TTP.
The patient remained seizure free and was extubated on day 5 of ICU
stay. She had a fully recovery after a 17-day course of glucocorticoids,
12 sessions of PEX and Rituximab. Laboratory parameters improvement
trends (platelet and hemoglobin level) are displayed in figure
1 . The patient was discharged with hemoglobin at 10 g/dl and platelet
count at 370 ×109/L with a follow up at the hematology
department. Prednisone was tapered off over 5 weeks. The patient made a
complete recovery and is currently living a normal life. The latest
ADAMTS-13 activity at the 6-month follow up visit showed 94%.