以肝衰竭为首要表现的恶性血液系统疾病——附7例报道  

Malignant hematological diseases with liver failure as the primary manifestation: a report of 7 cases

作　　者：赖曼[1] 王苏丹[2] 沈琳[3] 刘海霞[1] LAI Man;WANG Su-dan;SHEN Lin;LIU Hai-xia(Intensive Care Unit,Beijing Youan Hospital,Capital Medical University,Beijing 100069,China;Department of Image,Clinical Laboratory,Beijing Youan Hospital,Capital Medical University,Beijing 100069,China)

机构地区：[1]首都医科大学附属北京佑安医院重症医学科,100069 [2]首都医科大学附属北京佑安医院影像科,100069 [3]首都医科大学附属北京佑安医院检验科,100069

出　　处：《肝脏》2023年第1期33-36,49,共5页Chinese Hepatology

摘　　要：目的 观察以肝衰竭为首要表现的恶性血液系统疾病的临床特点及预后。方法 回顾性分析首都医科大学附属北京佑安医院2017年1月1日至2022年1月1日收治的7例恶性血液系统疾病患者的临床资料。结果 7例患者均为女性,年龄7~65岁,均有肝衰竭的典型症状及体征,发热7例,皮疹4例,淋巴结肿大4例,脾大6例。7例患者在病程中均有血细胞进行性下降。影像学表现为淋巴结肿大、脾大、骨质侵犯等。骨髓细胞学检查提示噬血细胞综合征、淋巴瘤等恶性疾病。结论 血液系统恶性肿瘤患者可以肝衰竭为首要表现,这类患者缺乏血液系统恶性疾病的典型临床表现,误诊率高,应尽早完善骨髓细胞学检查。Objective To investigate the clinical features of malignant hematological diseases with liver failure as the initial manifestation. Methods The clinical data including clinical symptoms, laboratory markers, imaging features of 7 malignant hematological cases with acute or subacute liver failure as the initial manifestation diagnosed at our hospital from January 1, 2017 to January 1, 2022 were analyzed retrospectively. Results All the 7 patients were female, with an average age of 7-65 years. They all had typical symptoms and signs of liver failure. Seven cases had fever in the course of the disease, 4 patients had rash, 4 patients had lymphadenopathy, and 6 patients had splenomegaly. During the course of the disease, leukopenia, anemia or thrombocytopenia occurred in 7 cases. Imaging findings were lymphadenopathy, splenomegaly, bone invasion and so on. Bone marrow cytology indicated hemophagocytic syndrome, lymphoma cell. Conclusion Liver failure might be the initial manifestation of patients with hematological malignancies. There is a high misdiagnosis rate in these patients because of the nontypical clinical representations. Cytological examination of bone marrow should be performed as soon as possible in suspicious patients.

关 键 词：血液系统恶性肿瘤 肝衰竭 噬血细胞综合征 淋巴瘤 恶性组织细胞病 

分 类 号：R575.3[医药卫生—消化系统]