Pulmonary Tumor Thrombotic Microangiopathy: Report of 3 Cases and Review of the Literature

Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare clinical entity where tumor cell embolisms in pulmonary circulation induce thrombotic microangiopathy (TMA), respiratory failure, and subacute cor pulmonale. We describe 3 cases of PTTM that presented as the initial manifestation of metasta...

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Autores principales: Gainza, Eukene, Fernández, Sara, Martínez, Daniel, Castro, Pedro, Bosch, Xavier, Ramírez, José, Pereira, Arturo, Cibeira, María T., Esteve, Jordi, Nicolás, Josep M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer Health 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4602432/
https://www.ncbi.nlm.nih.gov/pubmed/25500705
http://dx.doi.org/10.1097/MD.0000000000000219
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author Gainza, Eukene
Fernández, Sara
Martínez, Daniel
Castro, Pedro
Bosch, Xavier
Ramírez, José
Pereira, Arturo
Cibeira, María T.
Esteve, Jordi
Nicolás, Josep M.
author_facet Gainza, Eukene
Fernández, Sara
Martínez, Daniel
Castro, Pedro
Bosch, Xavier
Ramírez, José
Pereira, Arturo
Cibeira, María T.
Esteve, Jordi
Nicolás, Josep M.
author_sort Gainza, Eukene
collection PubMed
description Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare clinical entity where tumor cell embolisms in pulmonary circulation induce thrombotic microangiopathy (TMA), respiratory failure, and subacute cor pulmonale. We describe 3 cases of PTTM that presented as the initial manifestation of metastatic gastric adenocarcinoma with TMA and pulmonary infiltrates. All 3 cases had similar clinical and laboratory features, which included moderate thrombocytopenia without renal failure, hemolysis with extremely high serum lactate dehydrogenase levels, leukoerythroblastosis in peripheral blood smear, altered coagulation tests, lymphadenopathies, and interstitial pulmonary infiltrates. All patients died within 2 weeks of diagnosis. Two cases were initially misdiagnosed as idiopathic thrombotic thrombocytopenic purpura and treated with plasma exchange with no response. One patient had bone marrow infiltration by malignant cells. Autopsies revealed PTTM associated with gastric disseminated adenocarcinoma (signet-ring cell type in 2 patients and poorly differentiated type in 1). PTTM should be considered in the differential diagnosis of patients with fulminant microangiopathic hemolytic anemia, such as atypical thrombotic thrombocytopenic purpura, mainly those with pulmonary infiltrates, disseminated intravascular coagulation, or Trousseau syndrome.
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spelling pubmed-46024322015-10-27 Pulmonary Tumor Thrombotic Microangiopathy: Report of 3 Cases and Review of the Literature Gainza, Eukene Fernández, Sara Martínez, Daniel Castro, Pedro Bosch, Xavier Ramírez, José Pereira, Arturo Cibeira, María T. Esteve, Jordi Nicolás, Josep M. Medicine (Baltimore) Article Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare clinical entity where tumor cell embolisms in pulmonary circulation induce thrombotic microangiopathy (TMA), respiratory failure, and subacute cor pulmonale. We describe 3 cases of PTTM that presented as the initial manifestation of metastatic gastric adenocarcinoma with TMA and pulmonary infiltrates. All 3 cases had similar clinical and laboratory features, which included moderate thrombocytopenia without renal failure, hemolysis with extremely high serum lactate dehydrogenase levels, leukoerythroblastosis in peripheral blood smear, altered coagulation tests, lymphadenopathies, and interstitial pulmonary infiltrates. All patients died within 2 weeks of diagnosis. Two cases were initially misdiagnosed as idiopathic thrombotic thrombocytopenic purpura and treated with plasma exchange with no response. One patient had bone marrow infiltration by malignant cells. Autopsies revealed PTTM associated with gastric disseminated adenocarcinoma (signet-ring cell type in 2 patients and poorly differentiated type in 1). PTTM should be considered in the differential diagnosis of patients with fulminant microangiopathic hemolytic anemia, such as atypical thrombotic thrombocytopenic purpura, mainly those with pulmonary infiltrates, disseminated intravascular coagulation, or Trousseau syndrome. Wolters Kluwer Health 2014-11-02 /pmc/articles/PMC4602432/ /pubmed/25500705 http://dx.doi.org/10.1097/MD.0000000000000219 Text en © 2014 Wolters Kluwer Health | Lippincott Williams & Wilkins
spellingShingle Article
Gainza, Eukene
Fernández, Sara
Martínez, Daniel
Castro, Pedro
Bosch, Xavier
Ramírez, José
Pereira, Arturo
Cibeira, María T.
Esteve, Jordi
Nicolás, Josep M.
Pulmonary Tumor Thrombotic Microangiopathy: Report of 3 Cases and Review of the Literature
title Pulmonary Tumor Thrombotic Microangiopathy: Report of 3 Cases and Review of the Literature
title_full Pulmonary Tumor Thrombotic Microangiopathy: Report of 3 Cases and Review of the Literature
title_fullStr Pulmonary Tumor Thrombotic Microangiopathy: Report of 3 Cases and Review of the Literature
title_full_unstemmed Pulmonary Tumor Thrombotic Microangiopathy: Report of 3 Cases and Review of the Literature
title_short Pulmonary Tumor Thrombotic Microangiopathy: Report of 3 Cases and Review of the Literature
title_sort pulmonary tumor thrombotic microangiopathy: report of 3 cases and review of the literature
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4602432/
https://www.ncbi.nlm.nih.gov/pubmed/25500705
http://dx.doi.org/10.1097/MD.0000000000000219
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