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Acquired Thrombotic Thrombocytopenic Purpura: Key Findings of Patients from Southern Italy

1Fondazione IRCCS Casa Sollievo della Sofferenza, Thrombosis and Haemostasis, San Giovanni Rotondo, Italy, 2Azienda Ospedaliera Universitaria, Policlinico di Bari, Transfusion Medicine, Bari, Italy, 3Ospedale di Riferimento Regionale “Antonio Cardarelli”, Campobasso, Italy, 4Ospedale Mons. 'Dimiccoli', Ematologia, Barletta, Italy, 5University of Catania, Haematology, Catania, Italy, 6Fondazione IRCCS Casa Sollievo della Sofferenza, Haematology, San Giovanni Rotondo, Italy, 7Fondazione IRCCS Casa Sollievo della Sofferenza, Clinical Laboratory & Transfusion Medicine, San Giovanni Rotondo, Italy

Abstract Number: PB1882

Meeting: ISTH 2020 Congress

Theme: Thrombotic Microangiopathies » ADAMTS13 and TTP

Background: Clinical features of patients with acquired thrombotic thrombocytopenic purpura
(aTTP) are heterogeneous. Identifying those patients who are at risk for recurrence is difficult. Here, we describe clinical history, symptoms, laboratory and treatment data of patients with aTTP referred to 5 Italian Centres.

Aims: To add knowledge on natural history of patients with aTTP.

Methods: From January 2012 to September 2019, 70 patients were investigated for clinical and
laboratory signs of Thrombotic Microangiopathy (TMA). Sixteen patients were excluded from the present study because of a diagnosis other than aTTP. Thus, 54
patients with a confirmed diagnosis of aTTP disease are described. We report clinical manifestations, potential triggers, and co-morbidities. Demographic and clinical information were collected.
ADAMTS13 activity was measured by means of a chromogenic assay
(Technoclone, Austria) and the presence of inhibitor against ADAMTS13 using a classical
Bethesda method. Patients were followed for at least 6 months.

Results: Demographic, laboratory data and clinical information are reported in table 1. In 43
patients (78%), aTTP started with neurologic symptoms, in 18 (32.7%) skin manifestations, 11
(20%) kidney impairment [(GFR median=37 ml/min (range 35-74)]. Three patients (5.5%)
showed abdominal symptoms, 3 (5.5%) cardiovascular manifestations (1 myocardial infarction
and 2 hearth failure). Co-morbidities were: malignancy (7.4%), autoimmune diseases (3.7%),
psychiatric disease (11%), pregnancy (1.8%), infections (5.5%). Potential triggers were
oral contraceptives ( n=2, 3.7%) , antidepressants (n=6 ,11.0%), antiinflammatory
drugs (n=5 (9%), anti-epileptics (n=1 1.8%) and clopidogrel ( n= 1, 1.8%) .
All acute events were treated with plasma-exchange [(median number=13, (range 4-43)] and steroids.
Rituximab was also used in 16 (29.5%). Four patients (7.4%) died during acute event.
We calculated a recurrence rate of 3 per year (observation period 40 months).

Conclusions: Our data add knowledge to natural history of aTTP and confirm a relatively high mortality and recurrence.

Variables Data
Median age (years), (range) 50 (21-69)
Female, n (%) 38 (70.3)
ADAMTS13 activity (U/dL), mean±SD 1.7±0.9
ADAMTS13 activity (U/dL) during follow-up, mean±SD 69±12
ADAMTS13 inhibitor (Bethesda Unit/mL), median (range) 3 (0.6-28)
Platelet count at first acute event, (109/L), median (range) 13 (6-29)
Relapse, n (%) 10 (18.5%)
Death, n (%) 4 (7.4%)

[Table 1 Demographic, laboratory data and clinical information]

To cite this abstract in AMA style:

Tiscia GL, Cappucci F, Fischetti L, Battista C, Ostuni A, Giordano G, Tarantini G, Santeramo TM, Giuffrida G, Nicolosi D, Scalzulli PR, Cascavilla N, di Mauro L, Grandone E. Acquired Thrombotic Thrombocytopenic Purpura: Key Findings of Patients from Southern Italy [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/acquired-thrombotic-thrombocytopenic-purpura-key-findings-of-patients-from-southern-italy/. Accessed January 27, 2022.

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