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

机译:肺肿瘤血栓性微血管病3例报告并文献复习

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Abstract: 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.
机译:摘要:肺肿瘤血栓性微血管病(PTTM)是一种罕见的临床实体,其中肺循环中的肿瘤细胞栓塞会诱发血栓性微血管病(TMA),呼吸衰竭和亚急性肺动脉疾病。我们描述了3例PTTM,这些病例表现为TMA和肺浸润转移性胃腺癌的初始表现。所有3例病例均具有相似的临床和实验室特征,包括中度血小板减少症而无肾功能衰竭,溶血,血清乳酸脱氢酶水平极高,外周血涂片白细胞成纤维细胞增多,凝血试验改变,淋巴腺病和间质性肺浸润。所有患者在诊断后2周内死亡。最初将2例误诊为特发性血栓性血小板减少性紫癜,并用血浆置换治疗无反应。一名患者的恶性细胞浸润了骨髓。尸检显示PTTM与胃弥漫性腺癌有关(2例患者的信号环细胞类型和1例分化差的类型)。鉴别诊断为暴发性微血管性溶血性贫血,例如非典型性血栓性血小板减少性紫癜,主要是肺部浸润,弥散性血管内凝血或Trousseau综合征的患者,应鉴别诊断为PTTM。

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