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Abstract：
BACKGROUND: Malignant infiltration accounts for 0.5% of acute liver failure cases, with non-Hodgkin\'s lymphoma the predominant cause. Adult T-cell lymphoma/leukemia (ATLL) is a rarer source of acute hepatitis, with only 3 cases reported and all resulting in immediate deterioration with death. ATLL rises from human T-lymphocytic virus-1 (HTLV-1), commonly found in Japan (southern and northern islands), the Caribbean, Central and South America, intertropical Africa, Romania, and northern Iran. In Micronesia, HTLV-1 infection amongst native-born is absent or exceedingly rare.
METHODS: A 77-year-old Marshallese man presented to the emergency department with a 1-week history of generalized weakness, fatigue, and nausea. The physical exam revealed a cervical papulonodular exanthem and scleral icterus.
METHODS: Laboratory studies were remarkable for aspartate-aminotransferase of 230 IU/L (reference range [RR]: 0-40), alanine-aminotransferase of 227 IU/L (RR: 0-41), alkaline phosphatase of 133 IU/L (RR: 35-129), and total bilirubin of 4.7 mg/dL (RR: 0-1.2), supporting acute liver injury. Platelet count was 11.6x104/μL (RR: 15.1-42.4 × 104), hemoglobin was 13.8 g/dL (RR: 13.7-17.5), and white blood cell count was 7570/μL (RR: 3800-10,800) with 81.8% neutrophils (RR: 34.0-72.0) and 10.4% lymphocytes (RR: 12.0-44.0). The peripheral blood smear demonstrated abnormal lymphocytes with occasional flower cell morphology. HTLV-1/2 antibody tested positive. The skin and liver biopsies confirmed atypical T-cell infiltrate. The diagnosis of ATLL was established.
METHODS: The patient elected for palliative chemotherapy with cyclophosphamide, vincristine, and prednisone (CVP). He began antiviral treatment with zidovudine 250 mg bis in die (BID) indefinitely. Ursodiol and cholestyramine were added for his hyperbilirubinemia.
RESULTS: Four weeks from admission, the patient returned to near baseline functional status and was discharged home.
CONCLUSIONS: This case highlights that ATLL can initially present as isolated acute hepatitis, and how careful examination of peripheral blood-smear may elucidate hepatitis etiology. We also present support for utilizing ursodiol with cholestyramine for treating a hyperbilirubinemia. Moreover, unlike prior reports of ATLL presenting as liver dysfunction, combined antiviral and CVP chemotherapy was effective in this case. Lastly, there are seldom demographic reports of HTLV-1 infection from the Micronesian area, and our case represents the first indexed case of HTLV-1-associated-ATLL presenting as acute liver failure in a Marshallese patient.
METHODS: A 77-year-old Marshallese man presented to the emergency department with a 1-week history of generalized weakness, fatigue, and nausea. The physical exam revealed a cervical papulonodular exanthem and scleral icterus.
METHODS: Laboratory studies were remarkable for aspartate-aminotransferase of 230 IU/L (reference range [RR]: 0-40), alanine-aminotransferase of 227 IU/L (RR: 0-41), alkaline phosphatase of 133 IU/L (RR: 35-129), and total bilirubin of 4.7 mg/dL (RR: 0-1.2), supporting acute liver injury. Platelet count was 11.6x104/μL (RR: 15.1-42.4 × 104), hemoglobin was 13.8 g/dL (RR: 13.7-17.5), and white blood cell count was 7570/μL (RR: 3800-10,800) with 81.8% neutrophils (RR: 34.0-72.0) and 10.4% lymphocytes (RR: 12.0-44.0). The peripheral blood smear demonstrated abnormal lymphocytes with occasional flower cell morphology. HTLV-1/2 antibody tested positive. The skin and liver biopsies confirmed atypical T-cell infiltrate. The diagnosis of ATLL was established.
METHODS: The patient elected for palliative chemotherapy with cyclophosphamide, vincristine, and prednisone (CVP). He began antiviral treatment with zidovudine 250 mg bis in die (BID) indefinitely. Ursodiol and cholestyramine were added for his hyperbilirubinemia.
RESULTS: Four weeks from admission, the patient returned to near baseline functional status and was discharged home.
CONCLUSIONS: This case highlights that ATLL can initially present as isolated acute hepatitis, and how careful examination of peripheral blood-smear may elucidate hepatitis etiology. We also present support for utilizing ursodiol with cholestyramine for treating a hyperbilirubinemia. Moreover, unlike prior reports of ATLL presenting as liver dysfunction, combined antiviral and CVP chemotherapy was effective in this case. Lastly, there are seldom demographic reports of HTLV-1 infection from the Micronesian area, and our case represents the first indexed case of HTLV-1-associated-ATLL presenting as acute liver failure in a Marshallese patient.
摘要：
背景：恶性浸润占急性肝衰竭病例的0.5%，非霍奇金淋巴瘤是主要原因。成人T细胞淋巴瘤/白血病（ATLL）是急性肝炎的罕见来源，仅报告了3例病例，所有病例都立即恶化并死亡。ATLL从人类T淋巴细胞病毒1（HTLV-1）上升，常见于日本（南部和北部岛屿），加勒比海,中美洲和南美洲，热带非洲，罗马尼亚,伊朗北部。在密克罗尼西亚,本地出生的HTLV-1感染不存在或极为罕见。
方法：一名77岁的马绍尔人出现在急诊科，有1周的全身无力史，疲劳,和恶心。体格检查显示宫颈丘疹样结节和巩膜黄疸。
方法：对于230IU/L的天冬氨酸转氨酶（参考范围[RR]：0-40），丙氨酸转氨酶227IU/L（RR：0-41），133IU/L的碱性磷酸酶（RR：35-129），总胆红素为4.7mg/dL（RR：0-1.2），支持急性肝损伤。血小板计数为11.6x104/μL（RR：15.1-42.4×104），血红蛋白为13.8g/dL（RR：13.7-17.5），白细胞计数为7570/μL（RR：3800-10,800），中性粒细胞占81.8％（RR：34.0-72.0），淋巴细胞占10.4％（RR：12.0-44.0）。外周血涂片显示淋巴细胞异常，偶有花细胞形态。HTLV-1/2抗体检测呈阳性。皮肤和肝脏活检证实非典型T细胞浸润。确定了ATLL的诊断。
方法：选择环磷酰胺姑息化疗的患者，长春新碱,和泼尼松（CVP）。他开始无限期地使用齐多夫定250mgbisindie（BID）进行抗病毒治疗。Ursodiol和胆甾胺用于治疗他的高胆红素血症。
结果：入院四周，患者恢复到接近基线的功能状态并出院回家.
结论：该病例突出表明，ATLL最初可以表现为孤立的急性肝炎，以及仔细的外周血涂片检查如何阐明肝炎的病因。我们还提供了使用熊儿二醇和胆甾胺治疗高胆红素血症的支持。此外，与以前关于ATLL表现为肝功能障碍的报道不同，在这种情况下，联合抗病毒和CVP化疗是有效的。最后,密克罗尼西亚地区很少有HTLV-1感染的人口统计报告，我们的病例代表了马绍尔患者中出现急性肝衰竭的首例HTLV-1相关ATLL的索引病例。
方法：一名77岁的马绍尔人出现在急诊科，有1周的全身无力史，疲劳,和恶心。体格检查显示宫颈丘疹样结节和巩膜黄疸。
方法：对于230IU/L的天冬氨酸转氨酶（参考范围[RR]：0-40），丙氨酸转氨酶227IU/L（RR：0-41），133IU/L的碱性磷酸酶（RR：35-129），总胆红素为4.7mg/dL（RR：0-1.2），支持急性肝损伤。血小板计数为11.6x104/μL（RR：15.1-42.4×104），血红蛋白为13.8g/dL（RR：13.7-17.5），白细胞计数为7570/μL（RR：3800-10,800），中性粒细胞占81.8％（RR：34.0-72.0），淋巴细胞占10.4％（RR：12.0-44.0）。外周血涂片显示淋巴细胞异常，偶有花细胞形态。HTLV-1/2抗体检测呈阳性。皮肤和肝脏活检证实非典型T细胞浸润。确定了ATLL的诊断。
方法：选择环磷酰胺姑息化疗的患者，长春新碱,和泼尼松（CVP）。他开始无限期地使用齐多夫定250mgbisindie（BID）进行抗病毒治疗。Ursodiol和胆甾胺用于治疗他的高胆红素血症。
结果：入院四周，患者恢复到接近基线的功能状态并出院回家.
结论：该病例突出表明，ATLL最初可以表现为孤立的急性肝炎，以及仔细的外周血涂片检查如何阐明肝炎的病因。我们还提供了使用熊儿二醇和胆甾胺治疗高胆红素血症的支持。此外，与以前关于ATLL表现为肝功能障碍的报道不同，在这种情况下，联合抗病毒和CVP化疗是有效的。最后,密克罗尼西亚地区很少有HTLV-1感染的人口统计报告，我们的病例代表了马绍尔患者中出现急性肝衰竭的首例HTLV-1相关ATLL的索引病例。
