Alonso Joaquín Valle, Fonseca Javier, Lopera Elisa Lopera, Aguayo Miguel Ángel, Montes Yelda Hernandez, Llamas Jose Carlos
Departament of Emergency and Critical care Medicine, Hospital Valle de los Pedroches, Pozoblanco, Córdoba, Spain.
Hematol Rep. 2011 Aug 31;3(2):e14. doi: 10.4081/hr.2011.e14. Epub 2011 Oct 7.
Thrombotic microangiopathies (TMAs) represent a heterogeneous group of diseases characterized by a microangiopathic hemolytic anemia, peripheral thrombocytopenia, and organ failure of variable severity. TMAs encompass thrombotic thrombocytopenic purpura (TTP), typically characterized by fever, central nervous system manifestations and hemolytic uremic syndrome (HUS), in which renal failure is the prominent abnormality. In patients with cancer TMAs may be related to various antineoplastic drugs or to the malignant disease itself. The reported series of patients with TMAs directly related to cancer are usually heterogeneous, retrospective, and encompass patients with hematologic malignancies with solid tumors or receiving chemotherapy, each of which may have distinct presentations and pathophysiological mechanisms. Patients with disseminated malignancy who present with microangiopathic hemolytic anemia and thrombocytopenia may be misdiagnosed as thrombotic thrombocytopenic purpura (TTP) Only a few cases of TTP secondary to metastatic adenocarcinoma are known in the literature. We present a case of a 34-year-old man with TTP syndrome secondary to metastatic small-bowel adenocarcinoma. Patients with disseminated malignancy had a longer duration of symptoms, more frequent presence of respiratory symptoms, higher lactate dehydrogenase levels, and more often failed to respond to plasma exchange treatment. A search for systemic malignancy, including a bone marrow biopsy, is appropriate when patients with TTP have atypical clinical features or fail to respond to plasma exchange.
血栓性微血管病(TMA)是一组异质性疾病,其特征为微血管病性溶血性贫血、外周血小板减少以及严重程度各异的器官衰竭。TMA包括血栓性血小板减少性紫癜(TTP),其典型特征为发热、中枢神经系统表现和溶血性尿毒症综合征(HUS),其中肾衰竭是主要异常表现。在癌症患者中,TMA可能与各种抗肿瘤药物或恶性疾病本身有关。报道的与癌症直接相关的TMA患者系列通常是异质性的、回顾性的,涵盖患有实体瘤或接受化疗的血液系统恶性肿瘤患者,其中每一类患者可能有不同的表现和病理生理机制。出现微血管病性溶血性贫血和血小板减少的播散性恶性肿瘤患者可能被误诊为血栓性血小板减少性紫癜(TTP)。文献中仅知少数几例继发于转移性腺癌的TTP病例。我们报告一例34岁男性继发于转移性小肠腺癌的TTP综合征病例。播散性恶性肿瘤患者的症状持续时间更长,呼吸道症状出现更频繁,乳酸脱氢酶水平更高,且对血浆置换治疗无反应的情况更常见。当TTP患者具有非典型临床特征或对血浆置换无反应时,进行包括骨髓活检在内的系统性恶性肿瘤筛查是合适的。