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Endothelialized Microfluidics for Studying Microvascular Interactions in Hematologic Diseases
Published on: June 22, 2012
A multicenter experience of thrombotic microangiopathies in Turkey: The Turkish Hematology Research and Education
Emre Tekgündüz1, Mehmet Yılmaz2, Mehmet Ali Erkurt3
1Ankara Oncology Hospital, Hematology and BMT Clinic, Ankara, Turkey.
Abstract:
Thrombotic microangiopathies (TMAs) are rare, but life-threatening disorders characterized by microangiopathic hemolytic anemia and thrombocytopenia (MAHAT) associated with multiorgan dysfunction as a result of microvascular thrombosis and tissue ischemia. The differentiation of the etiology is of utmost importance as the pathophysiological basis will dictate the choice of appropriate treatment. We retrospectively evaluated 154 (99 females and 55 males) patients who received therapeutic plasma exchange (TPE) due to a presumptive diagnosis of TMA, who had serum ADAMTS13 activity/anti-ADAMTS13 antibody analysis at the time of hospital admission. The median age of the study cohort was 36 (14-84). 67 (43.5%), 32 (20.8%), 27 (17.5%) and 28 (18.2%) patients were diagnosed as thrombotic thrombocytopenic purpura (TTP), infection/complement-associated hemolytic uremic syndrome (IA/CA-HUS), secondary TMA and TMA-not otherwise specified (TMA-NOS), respectively. Patients received a median of 18 (1-75) plasma volume exchanges for 14 (153) days. 81 (52.6%) patients received concomitant steroid therapy with TPE. Treatment responses could be evaluated in 137 patients. 90 patients (65.7%) achieved clinical remission following TPE, while 47 (34.3%) patients had non-responsive disease. 25 (18.2%) non-responsive patients died during follow-up. Our study present real-life data on the distribution and follow-up of patients with TMAs who were referred to therapeutic apheresis centers for the application of TPE.
Insights
Thrombotic microangiopathies (TMAs) are rare but serious blood disorders. This study shows therapeutic plasma exchange (TPE) achieved remission in 65.7% of TMA patients, highlighting its importance in treatment.
Area of Science:
- Hematology
- Internal Medicine
- Rare Diseases
Background:
- Thrombotic microangiopathies (TMAs) are life-threatening conditions.
- Accurate diagnosis is crucial for effective treatment of TMAs.
Purpose of the Study:
- To evaluate the distribution and outcomes of patients with suspected TMAs treated with therapeutic plasma exchange (TPE).
- To analyze treatment response rates and patient follow-up in a real-world setting.
Main Methods:
- Retrospective analysis of 154 patients diagnosed with TMA and treated with TPE.
- Assessment of serum ADAMTS13 activity and anti-ADAMTS13 antibody levels.
- Evaluation of treatment response, including clinical remission and mortality.
Main Results:
- The most common diagnoses were thrombotic thrombocytopenic purpura (TTP) and infection/complement-associated hemolytic uremic syndrome (IA/CA-HUS).
- Therapeutic plasma exchange (TPE) led to clinical remission in 65.7% of patients.
- Non-responsive disease was associated with a 18.2% mortality rate.
Conclusions:
- Therapeutic plasma exchange (TPE) is an effective treatment for achieving remission in thrombotic microangiopathies (TMAs).
- Early diagnosis and appropriate management are critical for improving outcomes in TMA patients.
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