Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina
Abstract 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 coh...
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| Format: | Article |
| Language: | English |
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Wiley
2021-05-01
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| Series: | eJHaem |
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| Online Access: | https://doi.org/10.1002/jha2.154 |
| _version_ | 1797740054529966080 |
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| author | Célia Dos Santos Juvenal Paiva María Lucila Romero Mara Agazzoni Ana Catalina Kempfer Sabrina Rotondo María Marta Casinelli María Fabiana Alberto Analía Sánchez‐Luceros |
| author_facet | Célia Dos Santos Juvenal Paiva María Lucila Romero Mara Agazzoni Ana Catalina Kempfer Sabrina Rotondo María Marta Casinelli María Fabiana Alberto Analía Sánchez‐Luceros |
| author_sort | Célia Dos Santos |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-03-12T14:06:37Z |
| format | Article |
| id | doaj.art-5c89136facd644a0964cd9b1a60d206b |
| institution | Directory Open Access Journal |
| issn | 2688-6146 |
| language | English |
| last_indexed | 2024-03-12T14:06:37Z |
| publishDate | 2021-05-01 |
| publisher | Wiley |
| record_format | Article |
| series | eJHaem |
| spelling | doaj.art-5c89136facd644a0964cd9b1a60d206b2023-08-21T14:06:07ZengWileyeJHaem2688-61462021-05-012214915610.1002/jha2.154Thrombotic microangiopathies: First report of 294 cases from a single institution experience in ArgentinaCélia Dos Santos0Juvenal Paiva1María Lucila Romero2Mara Agazzoni3Ana Catalina Kempfer4Sabrina Rotondo5María Marta Casinelli6María Fabiana Alberto7Analía Sánchez‐Luceros8Laboratory of Haemostasis and Thrombosis CONICET National Academy of Medicine Institute of Experimental Medicine Buenos Aires ArgentinaDepartment of Haemostasis and Thrombosis National Academy of Medicine Haematologic Research Institute “Mariano R. Castex” Buenos Aires ArgentinaDepartment of Haemostasis and Thrombosis National Academy of Medicine Haematologic Research Institute “Mariano R. Castex” Buenos Aires ArgentinaDepartment of Haemostasis and Thrombosis National Academy of Medicine Haematologic Research Institute “Mariano R. Castex” Buenos Aires ArgentinaLaboratory of Haemostasis and Thrombosis CONICET National Academy of Medicine Institute of Experimental Medicine Buenos Aires ArgentinaDepartment of Haemostasis and Thrombosis National Academy of Medicine Haematologic Research Institute “Mariano R. Castex” Buenos Aires ArgentinaDepartment of Haemostasis and Thrombosis National Academy of Medicine Haematologic Research Institute “Mariano R. Castex” Buenos Aires ArgentinaDepartment of Haemostasis and Thrombosis National Academy of Medicine Haematologic Research Institute “Mariano R. Castex” Buenos Aires ArgentinaLaboratory of Haemostasis and Thrombosis CONICET National Academy of Medicine Institute of Experimental Medicine Buenos Aires ArgentinaAbstract 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.https://doi.org/10.1002/jha2.154ADAMTS13atypical haemolytic uraemic syndromethrombotic microangiopathiesthrombotic thrombocytopenic purpura |
| spellingShingle | Célia Dos Santos Juvenal Paiva María Lucila Romero Mara Agazzoni Ana Catalina Kempfer Sabrina Rotondo María Marta Casinelli María Fabiana Alberto Analía Sánchez‐Luceros Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina eJHaem ADAMTS13 atypical haemolytic uraemic syndrome thrombotic microangiopathies thrombotic thrombocytopenic purpura |
| title | Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina |
| title_full | Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina |
| title_fullStr | Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina |
| title_full_unstemmed | Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina |
| title_short | Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina |
| title_sort | thrombotic microangiopathies first report of 294 cases from a single institution experience in argentina |
| topic | ADAMTS13 atypical haemolytic uraemic syndrome thrombotic microangiopathies thrombotic thrombocytopenic purpura |
| url | https://doi.org/10.1002/jha2.154 |
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