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[血栓性血小板减少性紫癜伴发癌症:附2例报告]

[Thrombotic thrombocytopenic purpura disclosing cancer: apropos of 2 cases].

作者信息

Girard P, Tardy B, Page Y, Mosnier J F, Tardy-Poncet B, Bertrand J C

机构信息

Service d'Urgences, médicales, Hôpital de Bellevue, Saint-Etienne.

出版信息

Presse Med. 1995 Jan 21;24(3):175-8.

PMID:7899357
Abstract

Thrombotic thrombocytopenic purpura (TTP) causes severe haemolytic anaemia, thrombopenia, fever and neurological and renal involvement. Currently five large aetiologic groups have been identified: viral or bacterial infection, drugs, conjunctive tissue diseases, pregnancy and solid tumours. We observed two cases resulting from an adenocarcinoma. In the first case, a 71-year-old man with chronic silicosis, the presenting signs were asthenia, fever, epistaxis with diffus purpura and spontaneous haematomas of the lower limbs. Diagnosis of TTP was based on routine laboratory tests and the patient responded well to fresh frozen plasma. On the 5th day of treatment, haemoglobin level dropped sharply and melana occurred. Upper digestive tract endoscopy revealed a tumoural formation of the antrum-fundic junction and histology examination of the biopsy confirmed the diagnosis of adenocarcinoma. Ten months after gastrectomy the patient was in excellent health with no relapse of the TTP. In the second case, the presenting signs included spontaneous haematomas, rectorrhagia and low grade fever. Microscopic haematuria and renal failure were observed in addition to the biological syndrome of TTP. The patient responded poorly to fresh frozen plasma and packed cell transfusions. Plasma exchange was equally unsuccessful. The disease continued a fulminant course and the diagnosis of adenocarcinoma located in a pulmonary lymph nodes was made at autopsy. These rare cases of TTP caused by cancer emphasize the importance of a thorough aetiological research. Plasma exchange has been shown to be effective but mortality at 1 year approximately 85% in cancer related cases. Early diagnosis and specific anti-cancer therapy might improve prognosis. We report our personal experience with 16 other similar cases.

摘要

血栓性血小板减少性紫癜(TTP)可导致严重的溶血性贫血、血小板减少、发热以及神经和肾脏受累。目前已确定五个主要病因组:病毒或细菌感染、药物、结缔组织病、妊娠和实体肿瘤。我们观察到两例由腺癌引起的病例。第一例中,一名患有慢性矽肺的71岁男性,其表现症状为乏力、发热、鼻出血伴弥漫性紫癜以及下肢自发性血肿。TTP的诊断基于常规实验室检查,患者对新鲜冷冻血浆反应良好。在治疗的第5天,血红蛋白水平急剧下降并出现黑便。上消化道内镜检查显示胃窦-胃底交界处有肿瘤形成,活检的组织学检查证实为腺癌。胃切除术后十个月,患者健康状况良好,TTP未复发。第二例中,表现症状包括自发性血肿、直肠出血和低热。除了TTP的生物学综合征外,还观察到镜下血尿和肾衰竭。患者对新鲜冷冻血浆和红细胞悬液输血反应不佳。血浆置换同样未成功。疾病呈暴发性发展,尸检时诊断为位于肺门淋巴结的腺癌。这些由癌症引起的罕见TTP病例强调了进行全面病因研究的重要性。血浆置换已被证明是有效的,但在与癌症相关的病例中,1年死亡率约为85%。早期诊断和特异性抗癌治疗可能会改善预后。我们报告了我们个人对其他16例类似病例的经验。

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