Case Presentation
A 47 years-old patient, presented to our department of dermatology with
chronic leg ulcer complicated by dermohypodermitis (figure1). He was
treated with Intravenous amoxicillin-clavulanic acid and prophylaxis
doses of sub cutaneous enoxaparin 40 mg per Day. A venous Doppler
ultrasound was performed to explore the persistence of his leg edema,
which did not show any signs of thrombophlebitis, and ciprofloxacin 800
mg IV per day was added. Two days later, a cutaneous examination was
remarkable for mild petechial lesions in his lower limbs. He complained
of dyspnea, sweating and tachycardia. We realized a pulmonary
scintigraphy which revealed a distal pulmonary embolism. His laboratory
data showed a microcytic anemia (hemoglobin level of 8.6 g/dL),
thrombocytopenia (platelet count of 12000/mm3), hepatic cytolysis and
renal failure. A peripheral-blood smear showed schistocytes. Coomb’s
test, and the rest of the immunological workup were negative. All of
these symptoms led us to raise the diagnosis of TTP. The diagnosis was
confirmed with ADAMTS13 activity under 5% and an elevated rate
Anti-ADAMTS13 autoantibody. Three days later the patient presented with
a generalized seizure with post-critical deficit state. The patient was
transferred to the medical intensive care unit. Five sessions of
plasmapheresis with steroids (80mg/day) were performed with a transient
improvement. The patient received four doses of weekly rituximab at a
dose of 375 mg/m2 per dose. The evolution was marked by a therapeutic
escape and the appearance of thrombosis of multiple vessels. The Patient
died of multiple organ failure, five days later.