The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening syndrome characterized by microangiopathic anemia, thrombocytopenia, diffuse microvascular thrombosis, and ischemia. It is associated with very low levels of ADAMTS-13. Measurement of ADAMTS-13 levels is used for dia...
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doaj-58da787861f24ea3bb61e7386cf680222020-11-25T03:47:56ZengPAGEPress PublicationsItalian Journal of Medicine1877-93441877-93522012-01-015426927310.4081/itjm.2011.26988The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine departmentFilippo PieralliAntonio ManciniAlberto CamaitiGiancarlo BerniCarlo NozzoliIntroduction: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening syndrome characterized by microangiopathic anemia, thrombocytopenia, diffuse microvascular thrombosis, and ischemia. It is associated with very low levels of ADAMTS-13. Measurement of ADAMTS-13 levels is used for diagnostic and prognostic purposes, but in every-day clinical practice, this type of analysis is not always readily available. In this retrospective study, we evaluated prognostic value of clinical and laboratory findings in patients with TTP. <br />Materials and methods: We retrospectively investigated patients with clinically diagnosed TTP treated in a unit of Internal and Emergency Medicine (1996-2007). Clinical and laboratory findings were collected and analyzed in order to assess their ability to predict in-hospital death. <br />Results: Twelve patients were identified (mean age 59 <span style="text-decoration: underline;">+</span> 22 years; 58% were women). Five (42%) died during the hospitalization, and the variables significantly associated with this outcome were: a delay between diagnosis and symptom onset (HR 1.36; 95% CI 1.04-1.78; p < 0.05); a higher severity score (HR 1.48; 95%CI 1,23-3.86; p < 0.05); hemodynamic instability with hypotension and/or shock (HR 3.35; 95%CI 3.02-9.26; p < 0.01); a higher schistocyte count on blood smear (HR 1.84; 95%CI 1.04-3.27; p < 0.05); and higher lactate values (HR 1.85; 95%CI 1.08- 3.16; p < 0.05). <br />Conclusions: TTP is a rare and potentially fatal disease with protean manifestations. Delayed diagnosis after symptom onset is a major determinant of poor outcome. Hypotension and shock are also prognostically unfavourable. Laboratory evidence of cardiocirculatory compromise (i.e., elevated lactate levels) and extension of the disease process (i.e., schistocyte count > 3) are predictive of in-hospital death, independently of the hemodynamic profile on admission.http://www.italjmed.org/index.php/ijm/article/view/114Thrombotic thrombocytopenic purpurahemolytic anemialactic acid. |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Filippo Pieralli Antonio Mancini Alberto Camaiti Giancarlo Berni Carlo Nozzoli |
spellingShingle |
Filippo Pieralli Antonio Mancini Alberto Camaiti Giancarlo Berni Carlo Nozzoli The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department Italian Journal of Medicine Thrombotic thrombocytopenic purpura hemolytic anemia lactic acid. |
author_facet |
Filippo Pieralli Antonio Mancini Alberto Camaiti Giancarlo Berni Carlo Nozzoli |
author_sort |
Filippo Pieralli |
title |
The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department |
title_short |
The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department |
title_full |
The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department |
title_fullStr |
The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department |
title_full_unstemmed |
The ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department |
title_sort |
ability of clinical and laboratory findings to predict in-hospital death in patients with thrombotic thrombocytopenic purpura in an internal and emergency medicine department |
publisher |
PAGEPress Publications |
series |
Italian Journal of Medicine |
issn |
1877-9344 1877-9352 |
publishDate |
2012-01-01 |
description |
Introduction: Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening syndrome characterized by microangiopathic anemia, thrombocytopenia, diffuse microvascular thrombosis, and ischemia. It is associated with very low levels of ADAMTS-13. Measurement of ADAMTS-13 levels is used for diagnostic and prognostic purposes, but in every-day clinical practice, this type of analysis is not always readily available. In this retrospective study, we evaluated prognostic value of clinical and laboratory findings in patients with TTP. <br />Materials and methods: We retrospectively investigated patients with clinically diagnosed TTP treated in a unit of Internal and Emergency Medicine (1996-2007). Clinical and laboratory findings were collected and analyzed in order to assess their ability to predict in-hospital death. <br />Results: Twelve patients were identified (mean age 59 <span style="text-decoration: underline;">+</span> 22 years; 58% were women). Five (42%) died during the hospitalization, and the variables significantly associated with this outcome were: a delay between diagnosis and symptom onset (HR 1.36; 95% CI 1.04-1.78; p < 0.05); a higher severity score (HR 1.48; 95%CI 1,23-3.86; p < 0.05); hemodynamic instability with hypotension and/or shock (HR 3.35; 95%CI 3.02-9.26; p < 0.01); a higher schistocyte count on blood smear (HR 1.84; 95%CI 1.04-3.27; p < 0.05); and higher lactate values (HR 1.85; 95%CI 1.08- 3.16; p < 0.05). <br />Conclusions: TTP is a rare and potentially fatal disease with protean manifestations. Delayed diagnosis after symptom onset is a major determinant of poor outcome. Hypotension and shock are also prognostically unfavourable. Laboratory evidence of cardiocirculatory compromise (i.e., elevated lactate levels) and extension of the disease process (i.e., schistocyte count > 3) are predictive of in-hospital death, independently of the hemodynamic profile on admission. |
topic |
Thrombotic thrombocytopenic purpura hemolytic anemia lactic acid. |
url |
http://www.italjmed.org/index.php/ijm/article/view/114 |
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