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一名早期T细胞前体急性淋巴细胞白血病/淋巴瘤患者因获得性低纤维蛋白原血症导致严重出血素质:病例报告

Severe haemorrhagic diathesis due to acquired hypofibrinogenemia in a patient with early T-cell precursor acute lymphoblastic leukaemia/lymphoma: a case report.

作者信息

Spiezia Luca, Riva Marcello, Gurrieri Carmela, Campello Elena, Simioni Paolo

机构信息

General Internal Medicine & Thrombotic and Haemorrhagic Diseases Unit, Department of Medicine, Padova University School of Medicine, Padova, Italy.

Haematology and Clinical Immunology Unit, Department of Medicine, Padova University School of Medicine, Padova, Italy.

出版信息

Front Cardiovasc Med. 2024 Jan 8;10:1335296. doi: 10.3389/fcvm.2023.1335296. eCollection 2023.

Abstract

The most frequent haematological malignancy associated with acquired hypo/dysfibrinogenemia is multiple myeloma. We present an unusual case of severe haemorrhagic diathesis due to acquired hypofibrinogenemia in a patient with early T-cell precursor acute lymphoblastic leukaemia/lymphoma (ETP-ALL/LBL). A 57-year-old male was admitted to the General Internal Medicine Department of Padova University Hospital for acute massive haematomas of the left lower extremity associated with macrohaematuria. Coagulation tests showed prolonged prothrombin time, activated partial thromboplastin time and thrombin time due to isolated severe hypofibrinogenemia (antigen 0.70 g/L and activity 26%). The radiological workup showed a bulky lesion located in the anterior mediastinum, and a biopsy led to the diagnosis of ETP-ALL/LBL. Fibrinogen replacement therapy failed to correct the bleeding diathesis and we were able to exclude other frequent causes of acquired hypofibrinogenemia (i.e., liver dysfunction, fibrinogen-specific antibody or drug toxicity); therefore, we hypothesised that hypofibrinogenemia might stem either from enhanced removal of fibrinogen from the circulation or consumptive coagulopathy. Notably, only after initiating a specific chemotherapy treatment did the patient start showing improvement in bleeding symptoms and achieve normal fibrinogen levels.

摘要

与获得性低纤维蛋白原血症/异常纤维蛋白原血症相关的最常见血液系统恶性肿瘤是多发性骨髓瘤。我们报告了一例罕见病例,一名患有早期T细胞前体急性淋巴细胞白血病/淋巴瘤(ETP-ALL/LBL)的患者因获得性低纤维蛋白原血症出现严重出血倾向。一名57岁男性因左下肢急性大量血肿伴肉眼血尿入住帕多瓦大学医院普通内科。凝血检查显示,由于单纯严重低纤维蛋白原血症(抗原0.70 g/L,活性26%),凝血酶原时间、活化部分凝血活酶时间和凝血酶时间延长。影像学检查显示前纵隔有一个巨大肿块,活检确诊为ETP-ALL/LBL。纤维蛋白原替代疗法未能纠正出血倾向,我们排除了获得性低纤维蛋白原血症的其他常见原因(即肝功能障碍、纤维蛋白原特异性抗体或药物毒性);因此,我们推测低纤维蛋白原血症可能源于循环中纤维蛋白原清除增加或消耗性凝血病。值得注意的是,仅在开始特定化疗后,患者的出血症状才开始改善,纤维蛋白原水平恢复正常。

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