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.

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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