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. 2021 Jan 19;2(2):149-156.
doi: 10.1002/jha2.154. eCollection 2021 May.

Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina

Célia Dos Santos  1 , Juvenal Paiva  2 , María Lucila Romero  2 , Mara Agazzoni  2 , Ana Catalina Kempfer  1 , Sabrina Rotondo  2 , María Marta Casinelli  2 , María Fabiana Alberto  2 , Analía Sánchez-Luceros  1   2
Affiliations

Thrombotic microangiopathies: First report of 294 cases from a single institution experience in Argentina

Célia Dos Santos et al. EJHaem. .

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.

Keywords: ADAMTS13; atypical haemolytic uraemic syndrome; thrombotic microangiopathies; thrombotic thrombocytopenic purpura.

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Conflict of interest statement

Célia Dos Santos designed the study, collected and analysed data and wrote the manuscript. Célia Dos Santos, Juvenal Paiva, Ana Catalina Kempfer, Sabrina Rotondo, María Marta Casinelli and María Fabiana Alberto performed the laboratory studies. Mara Agazzoni collected data and provided clinical support. María Lucila Romero and Analía Sánchez‐Luceros designed the study, contributed to patient recruitment and provided clinical support. María Lucila Romero, María Fabiana Alberto and Analía Sánchez‐Luceros revised the manuscript. All authors approved the final version for journal submission.

Figures

FIGURE 1
FIGURE 1
Flow chart of laboratory procedure for patients with clinical suspicion of TMA Abbreviations: ADAMTS13, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13; aHUS, atypical haemolytic uraemic syndrome; aTTP, acquired thrombotic thrombocytopenic purpura; cTTP, congenital thrombotic thrombocytopenic purpura; HELLP, haemolysis, elevated liver enzymes, low platelet count; HUS, haemolytic uraemic syndrome; Ig, immunoglobulins; MAHA, microangiopathic haemolytic anemia; STEC‐HUS, Shiga toxin‐producing Escherichia coli HUS; TMA, thrombotic microangiopathy.
FIGURE 2
FIGURE 2
Sex and age distribution of 294 consecutive patients in an Argentine cohort with clinical suspicion of TMA
FIGURE 3
FIGURE 3
Distribution of the Argentine patient cohort (n = 294) in distinct confirmed or suspected diagnostic categories established through clinical manifestations and determination of ADAMTS13 parameters
FIGURE 4
FIGURE 4
Flow chart of TTP and aHUS patients selected for data analysis Abbreviations: AD13, ADAMTS13; aHUS, atypical haemolytic uraemic syndrome; aTTP, acquired thrombotic thrombocytopenic purpura; cTTP, congenital thrombotic thrombocytopenic purpura; Hb, hemoglobin; Ig, immunoglobulins; LDH, lactate dehydrogenase; MAHA, microangiopathic haemolytic anemia; PLT, platelets; TTP, thrombotic thrombocytopenic purpura.
FIGURE 5
FIGURE 5
Laboratory parameters analysis in aTTP versus aHUS patients during acute phase ***P < .05, Mann‐Whitney U test. Abbreviations: aHUS, atypical haemolytic uraemic syndrome; aTTP, acquired thrombotic thrombocytopenic purpura; LDH, lactate dehydrogenase.
See this image and copyright information in PMC

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