Screening of TMA Patients für ADAMTS13 Activity (Adamscreen)
Screening of TMA Patients für ADAMTS13 Activity (Adamscreen)
Screening of TMA patients for ADAMTS13 activity and the description of systemic organ damage
 and/or organ failure in different entities of thrombotic microangiopathies (TMA)
This is a prospective diagnostic analysis enrolling 100 patients with clinically suspected
 thrombotic microangiopathy (TMA) on the basis of laboratory findings such as
 thrombocytopenia, Coombs negative haemolytic anemia with increased LDH and increased
 schistozytes. As a first step of differential diagnosis, patients are classified by
 determining ADAMTS13 activity and antigen concentration and also analyzing for Shigatoxin and
 Shigatoxin producing bacteria respectively. Primary objective is to determine the relative
 incidences of the three major entities i.e. aHUS, STEC-HUS and TTP considering distribution
 of age and gender as well. Mean value of ADAMTS13 activity and antigen measured at date of
 first diagnosis is considered to line out a threshold value for ADAMTS13 activity pronouncing
 clinical apparent TMA. Furthermore the present study intends to characterize clinical
 presentation, clinical course and outcome of different TMA forms particularly with regard to
 treatment procedures. Monitoring of how ADAMTS13 activity and antigen changes in the course
 of the disease and in response to treatment is of particular concern for this study. Family
 history and special clinical conditions should be noted identifying potential genetic
 predisposition and definite clinical triggers leading to disease manifestation.
Inclusion Criteria:

 -Patients with first history of TMA as patients with recurrent TMA or TMA in complete or
 partial remission are eligible for analysis

 Exclusion Criteria:

 -Patients who received plasma intervention more than 3 weeks prior to screening must be
 excluded from the observational study