Acquired factor VII inhibitor associated with primary central nervous system Lymphoma: A case report.
Acquired factor VII inhibitor associated with primary central nervous system Lymphoma: A case report.
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DOI：
10.1002/jha2.482
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发表时间：
2022-08
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副肿瘤性凝固性病变在淋巴瘤患者中并不常见。我们报告第一例获得性凝血障碍的患者，在孤立的原发性中枢神经系统淋巴瘤(PCNSL)显示大细胞组织学。在我们的患者中，尽管新鲜的冰冻血浆和灭活的凝血酶原复合体浓缩物，副肿瘤因子VII抑制剂仍显著延迟了诊断性腰椎穿刺术。虽然她的凝血障碍通过重组激活因子VIIa和随后的淋巴瘤指导治疗得到了有效的克服，但她的延迟诊断可能导致了糟糕的结果。我们的病例强调了当怀疑PCNSL时，快速识别和纠正副肿瘤性凝固性疾病的重要性。
Paraneoplastic coagulopathies are uncommon in patients with lymphoma. We report the first case of an acquired coagulopathy in a patient with isolated primary central nervous system lymphoma (PCNSL) demonstrating large‐cell histology. In our patient, a paraneoplastic factor VII inhibitor significantly delayed a diagnostic lumbar puncture despite fresh frozen plasma and inactivated prothrombin complex concentrate. While her coagulopathy was effectively overcome with recombinant activated factor VIIa and subsequently with lymphoma‐directed therapy, her delayed diagnosis likely contributed to a poor outcome. Our case highlights the importance of rapidly identifying and correcting paraneoplastic coagulopathies when PCNSL is suspected.