We report the case of a 23-year-old woman, with no medical or surgical history, single, nulliparous, without ongoing drug treatment or hormonal contraception.
The year 2002 was hospitalized for the first time due to the appearance in a few days of nausea, petechiae and dyspnea in the extremities.
The diagnosis of TTP was formulated and indicated treatment with fresh frozen plasma infusions (FFP), followed by plasma exchange sessions (PE), with clinical and biological regression of TTP.
Seven months later, a similar condition led to a new hospitalization.
As during the first episode, neurological examination and renal function were normal.
Leukocytopenia Neutropenia Leukopenia Neutropenia Leukocytosis Thrombocytaemia Neutropenia Leukocytosis Thrombocytaemia Neutropenia Thrombocytaemia
Direct Coombs test was negative.
Blood smear showed the presence of schizocytes, initially >200/00.
There were no clinical or biological signs of infection.
The initial treatment consisted of the administration of platelet transfusions and erythrocyte concentrates, associated with PFC infusions.
Due to its ineffectiveness, PE sessions were initiated with variable PFC restitution volumes between 2,200 and 3,600 ml per session.
In total, 14 sessions were held between the beginning and the end of May, resulting equally ineffective, so a second-line treatment was initiated.
Vindesine (Vds) (Eldisine®, Laboratorio Lilly, France) 2 mg/m2/week for 5 weeks, associated with rituximab (R) (Mabther Basilea®, Roche Laboratories, Switzerland)
The last infusion of R was performed in early July.
Biological parameters progressively improved reaching normal values in August 2003.
1.
We retrospectively evaluated the plasma activity of ADAMTS13 and the presence or absence of inhibitors by previously described techniques performed in a reference laboratory11.
In relation to the inhibitors, their presence was considered simply as positive (P) or negative (N) depending on the titer observed.
Measurements were taken at the time of relapse, during the therapeutic phase with PE, after treatment with rituximab-vindesine (R-Vds) and later controls.
Figure 2 shows that ADAMTS13 activity was undetectable from the start, despite PE.
Its increase was observed only after administration of R-Vds treatment, but without reaching normal values even 16 months after clinical remission.
At the same time, at the time of relapse and even before R-Vds treatment, the presence of inhibitors was detected.
These disappeared after the end of treatment and were not detectable in the last available controls before the preparation of this report.
After 21 months from the end of treatment, the patient is well, asymptomatic, maintaining regular biological controls.
