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

All patients with a constellation of thrombocytopenia, Coombs negative hemolytic anaemia and clinical signs of ischaemic end organ damage or abdominal symptoms.