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Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
Célia Dos Santos1, Juvenal Paiva2, María Lucila Romero2
1Laboratory of Haemostasis and Thrombosis CONICET National Academy of Medicine Institute of Experimental Medicine Buenos Aires Argentina.
Introduction:
Introduction: Thrombotic microangiopathies (TMAs) are rare disorders associated with fatal outcomes if left uncared for. However, healthcare problems in developing countries tend to limit medical assistance to patients.
Methods:
Methods: We prospectively studied an Argentine cohort of 294 consecutive patients from 2013 to 2016. Patients' subcategory classification relied on clinical symptoms and presence or absence of trigger events associated with TMA.
Results:
Main suspected disorders were the primary TMAs known as thrombotic thrombocytopenic purpura (TTP) (n = 72/294, 24%) and atypical haemolytic uraemic syndrome (aHUS) (n = 94/294, 32%). In acute phase, demographic parameters for acquired TTP (aTTP) (n = 28) and aHUS (n = 47) showed that both groups were characterised by a young median age (37 and 25 years, respectively) and female predominance (60% and 86%). Median of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 activity was significantly lower in aTTP than in aHUS group (1.4% vs 83%) and was associated with a more severe thrombocytopenia (15 × 109 vs 53 × 109/L). Creatinine (Cr) and urea (Ur) were significantly increased in aHUS compared to aTTP subjects (Cr: 3.7 vs 0.7 mg/dL, Ur: 118 vs 33 mg/dL). Gastrointestinal and neurological symptoms were more frequent in aHUS and aTTP, respectively.
Conclusion:
The first description of a TMA cohort in Argentina revealed similar clinical presentations to those of other countries.
Insights
Thrombotic microangiopathies (TMAs) are rare, life-threatening disorders. This Argentine study of 294 patients found TMAs like TTP and aHUS present similarly to global cohorts, highlighting the need for accessible healthcare.
Area of Science:
- Hematology
- Rare Diseases
- Nephrology
Background:
- Thrombotic microangiopathies (TMAs) are rare, severe conditions with potentially fatal outcomes.
- Limited healthcare access in developing countries poses challenges for TMA patient management.
Purpose of the Study:
- To describe the clinical characteristics of a prospective Argentine cohort of TMA patients.
- To compare the presentations of thrombotic thrombocytopenic purpura (TTP) and atypical hemolytic uremic syndrome (aHUS) within this cohort.
Main Methods:
- Prospective study of 294 consecutive TMA patients in Argentina (2013-2016).
- Classification based on clinical symptoms and trigger events.
- Analysis of demographic data, ADAMTS13 activity, and renal function markers (creatinine, urea).
Main Results:
- The cohort included 72 patients with TTP (24%) and 94 with aHUS (32%).
- Acquired TTP (aTTP) and aHUS patients were young, with female predominance.
- Lower ADAMTS13 activity and more severe thrombocytopenia were observed in aTTP.
- Higher creatinine and urea levels were noted in aHUS compared to aTTP.
Conclusions:
- This is the first TMA cohort description from Argentina.
- Clinical presentations of TMAs in Argentina are comparable to those reported internationally.
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