以肝衰竭为首要表现的恶性血液系统疾病——附7例报道

肝脏 ›› 2023, Vol. 28 ›› Issue (1): 33-36.

以肝衰竭为首要表现的恶性血液系统疾病——附7例报道

赖曼, 王苏丹, 沈琳, 刘海霞

100069 首都医科大学附属北京佑安医院重症医学科(赖曼,刘海霞),影像科(王苏丹),检验科(沈琳)

收稿日期:2022-06-30 出版日期:2023-01-31 发布日期:2023-02-21
通讯作者: 刘海霞,Email: lhx52@126.com

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

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

Received:2022-06-30 Online:2023-01-31 Published:2023-02-21
Contact: LIU Hai-xia, Email: lhx52@126.com

摘要/Abstract

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。

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

Abstract: 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.

Key words: Hematological malignancy, Liver failure, Hemophagocytic syndrome, Lymphoma, Malignant histiocytosis

赖曼, 王苏丹, 沈琳, 刘海霞. 以肝衰竭为首要表现的恶性血液系统疾病——附7例报道[J]. 肝脏, 2023, 28(1): 33-36.

LAI Man, WANG Su-dan, SHEN Lin, LIU Hai-xia. Malignant hematological diseases with liver failure as the primary manifestation: a report of 7 cases[J]. Chinese Hepatolgy, 2023, 28(1): 33-36.

参考文献

[1] 中华医学会感染病学分会肝衰竭及人工肝学组,中华医学会肝病学分会重型肝病与人工肝学组. 肝衰竭诊治指南(2018年版). 临床肝胆病杂志, 2019, 35(01):38-44.
[2] Singh MM, Pockros PJ. Hematologic and oncologic diseases and the liver. Clin Liver Dis,2011, 15(1):69-87.
[3] Rich NE, Sanders C, Hughes RS, et al. Malignant infiltration of the liver presenting as acute liver failure. Clin Gastroenterol Hepatol, 2015, 13(5):1025-1028.
[4] Meier JA, Bokemeyer A, Cordes F, et al. Serum levels of ferritin and transferrin serve as prognostic factors for mortality and survival in patients with end-stage liver disease: a propensity score-matched cohort study. United European Gastroenterol J, 2020, 8(3):332-339.
[5] Minoia F, Davì S, Horne A, et al. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients. Arthritis Rheumatol, 2014, 66(11):3160-3169.
[6] Allen CE, Yu X, Kozinetz CA, et al. Highly elevated ferritin levels and the diagnosis of hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer, 2008, 50(6):1227-1235.
[7] Emmenegger U, Frey U, Reimers A, et al. Hyperferritinemia as indicator for intravenous immunoglobulin treatment in reactive macrophage activation syndromes. Am J Hematol, 2001, 68(1):4-10.
[8] Lin TF, Ferlic-Stark LL, Allen CE, et al. Rate of decline of ferritin in patients with hemophagocytic lymphohistiocytosis as a prognostic variable for mortality. Pediatr Blood Cancer, 2011, 56(1):154-155.
[9] Emmenegger U, Schaer DJ, Larroche C, et al. Haemophagocytic syndromes in adults: current concepts and challenges ahead. Swiss Med Wkly, 2005, 135(21-22):299-314.
[10] Carter SJ, Tattersall RS, Ramanan AV. Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment. Rheumatology (Oxford), 2019, 58(1):5-17.
[11] Al-Samkari H, Berliner N. Hemophagocytic Lymphohistiocytosis. Annu Rev Pathol, 2018, 13:27-49.
[12] Larroche C. Hemophagocytic lymphohistiocytosis in adults: diagnosis and treatment. Joint bone spine, 2012, 79(4):356-361.
[13] Tang WP, Zhong N, Chen ZW, et al. Clinical characteristics of liver dysfunction in patients with hemophagocytic syndrome. Zhongguo Shi Yan Xue Ye Xue Za Zhi, 2019, 27(4):1297-1304.
[14] Valade S, Azoulay E, Galicier L, et al. Coagulation disorders and bleedings in critically Ill patients with hemophagocytic lymphohistiocytosis. Medicine (Baltimore), 2015, 94(40):e1692.
[15] 王昭. 恶性组织细胞病的再认识. 中华血液学杂志, 2017, 38(09):806-808.
[16] Kheyri Z, Ali Asgari A, Zare Mehrjerdi A, et al. Fulminant hepatic failure due to primary hepatic lymphoma: a case report. Middle East J Dig Dis, 2013, 5(3):168-170.
[17] Romacho López L, León Díaz FJ, Sánchez Pérez B, et al. Acute liver failure caused by primary non-hodgkin's lymphoma of the liver. Transplant Proc, 2016, 48(9):3000-3002.
[18] Shah I, Vyas N, Reynolds JA. Natural killer-like T-cell lymphoma: a rare cause of acute liver failure. ACG Case Rep J, 2019, 6(7):e00145.
[19] Hafner A, Eaton DB. Acute liver failure with severe lactic acidosis secondary to infiltrative diffuse large B-cell lymphoma: an imaging-negative presentation. Cureus, 2020, 12(8):e10110.
[20] Shibata J, Kurahashi S, Naito T, et al. Diffuse large B cell lymphoma primarily presenting as acute liver failure in a surviving patient. J Community Hosp Intern Med Perspect, 2019, 9(2):135-139.
[21] El Nouwar R, El Murr T. Primary hepatic diffuse large B-cell lymphoma mimicking acute fulminant hepatitis: a case report and review of the literature. Eur J Case Rep Intern Med, 2018, 5(6):000878.