Prophylactic heparin does not prevent liver veno-occlusive disease following autologous bone marrow transplantation.

Veno-occlusive disease (VOD) is a major cause of toxic death after autologous bone marrow transplantation (ABMT). We studied the potential role of continuous administration of low-dose heparin for VOD prevention in 234 consecutive patients who underwent ABMT in our institution. The population consisted of 98 patients autografted before October 1984 who did not receive heparin, and a series of 136 patients autografted from October 1984 to March 1989 containing 98 patients included in a randomized trial comparing heparin administration (n = 52) vs no heparin (n = 46), and an additional group of 38 patients who received non-randomized heparin in view of high-risk criteria to develop VOD (n = 31) or other reasons unrelated to VOD (n = 7). Overall, 90 patients (38%) received heparin and 144 (62%) did not. The global incidence of VOD was 13/234 (5-5%). Heparin did not reduce the risk of VOD in all subgroups studied. In particular, in the randomized trial, the incidence of VOD was 2.2% in the group without heparin vs 7-7% in the group receiving heparin. We analyzed in depth the 13 patients who developed VOD and we compared them to a control group of 13 patients pair-matched for age, sex, diagnosis and preparative regimen, who did not develop VOD. We found that abnormal LFT before ABMT predisposed patients to VOD; refractoriness to platelet transfusion was observed in 85% of the patients in the VOD group vs 15% in the control group (p less than 0.05). VOD patients had an increased requirement for red cells and platelet transfusions, a lower recovery (R less than 25%) after the second and third platelet transfusion, and shorter intervals separating the first four platelet transfusions. Further, the platelet reconstitution after ABMT in the VOD group was slower in comparison to the control group (p less than 0.01). Again, in this pair-matched analysis continuous infusion of low-dose heparin did not prevent VOD.     

Defibrotide for the treatment of hepatic veno-occlusive disease: results of the European compassionate-use study

Severe hepatic veno-occlusive disease (VOD) is a recognized complication of autologous and allogeneic stem cell transplantation (SCT) that is often fatal. Defibrotide (DF) is a polydeoxyribonucleotide that has been found to have anti-thrombotic, anti-ischaemic and thrombolytic properties without causing significant anticoagulation. Preliminary studies have demonstrated activity for DF in the treatment of VOD, with minimal associated toxicity. In the present study, 40 patients who fulfilled established criteria for VOD were treated with DF on compassionate grounds in 19 European centres; 28 patients met risk criteria predicting progression of VOD and fatality or had evidence of multiorgan failure (MOF), and were defined as 'poor-risk'. DF was commenced intravenously at a median of 14 d (range, -2 d to 53 d) post SCT at doses ranging from 10 to 40 mg/kg. The median duration of therapy was 18 d (range, 2--71 d). Twenty-two patients showed a complete response (CR) (bilirubin < 34.2 micromol/l and resolution of signs/symptoms of VOD and end-organ dysfunction) [CR = 55%, confidence interval (CI) 40--70%] and 17 patients (43%) are alive beyond d +100. Ten poor-risk patients showed a complete response (CR = 36%, CI 21--51%). These results demonstrate that DF is an active treatment for VOD following SCT and a randomized trial is now underway in order to further evaluate its role.     

以关节痛为首发症状的肝小静脉闭塞症1例报告

肝小静脉闭塞症(hepatic veno-occlusive disease,HVOD)指肝小叶中央静脉和肝小叶下静脉狭窄甚至闭塞,继而发生肝细胞萎缩、弥漫性肝纤维化.HVOD的病因多见于野百合碱中毒,近年来造血干细胞移植、化疗、放疗等也成为常见病因.临床可表现为肝脏肿大、疼痛、腹水等.该病临床诊断较为困难,病理检查、影像学检查具有特征性.我科近期明确诊断1例以关节痛为首发表现的HVOD患者,现报告如下.     

A case report of hepatic veno-occlusive disease after ingesting dainties

INTRODUCTION Hepatic veno-occlusive disease (HVOD) is a rarely encountered ailment in the literature[1,2]. The establishment of the diagnosis of this condition can be very difficult because there is no specificity in the clinical manifestations and some c…     

造血干细胞移植并发肝静脉闭塞症23例

肝静脉闭塞症（HVOD）是造血干细胞移植（HSCT）后一种严重且危险的并发症，一般表现为移植后3周内即出现黄疸、肝肿大、肝区疼痛、腹腔积液及液体潴留和不明原因的体重增加。现将我院240例HSCT患者中并发HVOD23例报道如下。     

Hepatic veno-occlusive disease induced by Gymura segetum: report of two cases

BACKGROUND: Hepatic veno-occlusive disease (VOD) or sinusoidal obstruction syndrome is associated with a high mortality because of its severity. Gymura segetum, a Chinese herbal medicine, is always used to cure injury and bleeding in rural areas in China. This study was undertaken to better understand VOD and its relations to the effect of Gymura segetum. METHODS: Between 2000 and 2002,two patients were admitted to our department because of VOD. Before admission, both of them had been injured and taken oral decoction of patent drug Gymura segetum. We analyzed the clinical manifestations, diagnosis and therapy of the two patients. RESULTS: Pyrrolizidine in Panax notginseng was proved to induce VOD. The diagnosis of VOD depended on hepatic biopsy. CONCLUSION: Gymura segetum can induce VOD. More attention should be paid to its unsuscepted side effects.     

原位肝移植治疗肝小静脉闭塞病1例

1病例资料患者,男,57岁,因腹胀、腹痛1个月余入院。1个月前,患者无明显诱因出现腹胀、右上腹痛,发现肝脏肿大和腹水到眉山市人民医院治疗。有饮酒史30年,每日摄入乙醇约120 g。查体：一般情况差,皮肤巩膜无黄染,可见肝掌,未见蜘蛛痣,心肺无异常,腹膨隆,腹软,中上腹轻压痛,无反跳痛及肌紧张,     

造血干细胞移植后肝静脉闭塞病的临床观察

目的 探讨造血干细胞移植（HSCT）后肝静脉闭塞病（HVOD）的临床特征、相关危险因素.方法 用回顾性分析方法对145例HSCT患者移植后HVOD的发病情况进行临床分析.结果 145例患者中,发生HVOD 8例,发生率为5.5%.对HVOD的相关危险因素分析发现,HSCT患者移植前乙型肝炎表面抗原（HbsAg）阳性或HBV-DNA阳性患者HVOD发生率显著增高,是HVOD发生的高危因素（P＜0.01）;PGE1脂质微球（Lipo-PGE1）预防HVOD的效果显著优于PGE1组（P＜0.05）,原因可能与本组病例PGE1预防剂量相对不足有关.结论 HVOD是HSCT常见并发症之一,移植前患者感染乙型肝炎病毒是其发生的高危因素;Lipo-PGE1对HVOD具有较好的预防效果.     

Successful treatment of disseminated Nocardia farcinica infection in a living-donor liver transplantation recipient

Nocardiosis is a serious infection with high mortality. We report a case of subcutaneous and neural lesions due to Nocardia farcinica infection after living-donor liver transplantation. The neural lesion was cured with antibiotics without drainage.     

Gynura root induces hepatic veno-occlusive disease： A case report and review of the literature

Gynura root has been used extensively in Chinese folk medicine and plays a role in promoting microcirculation and relieving pain.However,its hepatic toxicity should not be neglected.Recently,we admitted a 62-year old female who developed hepatic veno-occlusive disease(HVOD)after ingestion of Gynura root.Only a few articles on HVOD induced by Gynura root have been reported in the literature.It is suspected that pyrrolizidine alkaloids in Gynura root might be responsible for HVOD.In this paper,we report a case of HVOD and review the literature.     

Case report of acute liver injury caused by the eszopiclone in a patient with chronic liver disease

Rationale:Eszopiclone, sold under the brand name Lunesta, is a new type of non-benzodiazepine hypnotic. Eszopiclone is a zopiclone dextrorotation, which is classified as a cyclopyrrolone. It functions by binding gamma-aminobutyric acid (GABA) receptors. Compared with benzodiazepines hypnotics, eszopiclone has higher selectivity for certain subunits of the GABA(A) receptor. So far, there are no reports about the elevation of serum enzymes or severe liver injury caused by eszopiclone. Here, we present a case report of acute liver injury following eszopiclone treatment in a patient with chronic hepatitis B virus (HBV).Patient concerns:The patient was a 53-year-old female with a 36-year history of positive HBV markers. Due to poor sleep, the patient took trazodone hydrochloride orally for 1 year. After hospital admission for positive hepatitis B pathogenic markers, abdominal distension, fatigue, and aggravation, she was treated with eszopiclone under the guidance of the mental health department.Diagnoses:Her transaminase levels increased abnormally after eszopiclone treatment and rapidly decreased after drug withdrawal. This was determined to be an acute liver injury event. liver-protecting treatment was maintained. Considering the patient''s anxiety and depression, the patient''s family members refused a liver biopsy.Outcomes:Transaminase levels decreased rapidly within one week, and the patient continued to take trazodone hydrochloride after discharge. No adverse events occurred in the follow-up period.Lessons:Sleep disorders are more common in patients with chronic diseases, especially patients with chronic liver disease. Recently, it has become common for patients with hepatitis B and C to use antidepressants along with antiviral treatment. Patients with chronic hepatitis B or C may have a threefold risk of liver dysfunction after receiving antituberculosis treatment.^[1,2] A proinflammatory environment induced by actively replicating the hepatitis virus may alter the detoxication process and increase drug toxicity.^[3] At this time, the safety of other drugs should be reevaluated. Although hepatitis and liver injury are listed as rare adverse reactions of eszopiclone, this case is the first to report the eszopiclone-involved acute liver injury.     

Living-donor liver transplantation in Budd-Chiari syndrome with inferior vena cava complete thrombosis: A case report and review of the literature

BACKGROUND Budd-Chiari syndrome(BCS) is a challenging indication for liver transplantation(LT) due to a combination of massive liver,increased bleeding,retroperitoneal fibrosis and frequently presents with stenosis of the inferior vena cava(IVC).Occasionally,it may be totally thrombosed,increasing the complexity of the procedure,as it should also be resected.The challenge is even greater when performing living-donor LT as the graft does not contain the retrohepatic IVC;thus,it may be necessary to reconstruct it.CASE SUMMARY A 35-year-old male patient with liver cirrhosis due to BCS and hepatocellular carcinoma beyond the Milan criteria underwent living-donor LT with IVC reconstruction.It was necessary to remove the IVC as its retrohepatic portion was completely thrombosed,up to almost the right atrium.A right-lobe graft was retrieved from his sister,with outflow reconstruction including the right hepatic vein and the branches of segment V and VIII to the middle hepatic vein.Owing to massive subcutaneous collaterals in the abdominal wall,venovenous bypass was implemented before incising the skin.The right atrium was reached via a transdiaphragramatic approach.Hepatectomy was performed en bloc with the retrohepatic vena cava.It was reconstructed with an infra-hepatic vena cava graft obtained from a deceased donor.The patient remains well on outpatient clinic follow-up 25 mo after the procedure,under an anticoagulation protocol with warfarin.CONCLUSION Living-donor LT in BCS with IVC thrombosis is feasible using a meticulous surgical technique and tailored strategies.     

Subcapsular hematoma after right-lobe living-donor liver transplantation

Because right-lobe living-donor liver transplantation was introduced in adult-to-adult liver transplantation to mitigate the problems of small-for-size grafts, some technical controversies have been reported. This report describes a case of graft subcapsular hematoma due to parenchymal injury. A 53-year-old woman underwent a right-lobe living-donor liver transplantation for acute-on-chronic liver failure due to primary biliary cirrhosis. A huge subcapsular hematoma was discovered by routine Doppler echogram examination on the first posttransplantation day. Relaparotomy findings revealed that rotation of the graft for the hemostasis procedure during the transplant operation had induced a compression injury to the graft by the xiphoid process. It was speculated that a small laceration in the graft parenchyma led to the major subcapsular hematoma. This experience suggests that the graft liver must be handled with special care to prevent potential mechanical injury.     

Successful treatment with tocilizumab for refractory anemia and slowly progressive renal glomerulosclerosis in multicentric Castleman disease: A case report

Rationale:Multicentric Castleman disease (MCD) is a rare lymphoproliferative disorder accompanied by systemic symptoms characterized by polyclonal hypergammaglobulinemia and chronic inflammation due to overexpression of interleukin-6. Histological heterogeneity of renal involvement in MCD has been described, although the number of reports is limited. Tocilizumab, a humanized anti-interleukin-6 receptor antibody, has been reported to be effective for MCD.Patent concerns:A 64-year-old man experienced refractory anemia and slowly progressive renal dysfunction with proteinuria, accompanied by persistent inflammation for 11 years.Diagnosis:Two renal biopsies were obtained. The first biopsy performed 7 years before admission revealed non-specific interstitial inflammation, whereas the second biopsy demonstrated global sclerosis in most glomeruli and interstitial fibrosis. The patient had multiple lymphadenopathies. Cervical lymph node biopsy histological findings were compatible with plasma cell type Castleman disease. The patient had no evidence of human hepatitis virus-8 infection.Intervention:The patient was treated with 60 mg/d prednisolone followed by 8 mg/kg intravenous tocilizumab every 2 weeks.Outcome:His anemia significantly improved, as well as a marked reduction in proteinuria and stabilization of renal function. He did not experience renal function during the 2-years follow-up period.Lessons:The heterogeneity of the renal manifestations of MCD sometimes makes early diagnosis difficult. We need to interpret the histological findings of the renal biopsy carefully. For advanced-stage renal diseases, tocilizumab might be an effective treatment strategy for MCD.     

肝素联合前列腺素E1预防造血干细胞移植后肝静脉阻塞症

目的 探讨在异基因造血干细胞移植(Allo-HSCT)中使用肝素联合前列腺素E1(PGE1)预防肝静脉阻塞症(HVOD)的疗效和不良反应.方法 100例造血干细胞移植患者,男62人,女38人,其中位年龄30岁(2～62岁),其中血液系统恶性肿瘤85例,重型再生障碍性贫血13例,重症肌无力2例.所有患者均采用肝素联合PGE1预防HVOD.结果 100例造血干细胞移植患者中除3例非血缘脐血移植患者未植入外其余患者均获得植入,本组HVOD的发生率2.0%.结论 肝素联合PGE1可用于造血干细胞移植中预防HVOD,疗效好,副作用小,价格低廉,是一个安全,值得推广的预防方案.     

A first report of leptospirosis after liver transplantation

Leptospirosis has been rarely reported in solid organ transplant recipients. We report the first case to our knowledge of leptospirosis in a liver transplant recipient who developed jaundice and renal insufficiency. We describe his favorable clinical progression and discuss the possible mechanisms involved in the more benign disease course. We also review the previously published cases of leptospirosis in solid organ transplant recipients. Although this disease does not appear to present any particularities in this context, we highlight the importance of clinical suspicion in this setting, particularly after liver transplantation.     

Metronidazole-induced hepatotoxicity in a patient with xeroderma pigmentosum: A case report

Rationale:Whereas metronidazole-induced hepatotoxicity is quite rare in the general population, in individuals carrying a nucleotide excision repair disorder, namely Cockayne syndrome, there is a high risk of developing this complication.Patient concerns:We report the case of a 44-year-old man, affected by xeroderma pigmentosum, who was admitted to the hospital presenting aspiration pneumoniae caused by worsening dysphagia and with severe hepatotoxicity during the hospitalization.Diagnoses:Acute hepatitis, which was leading to acute liver failure, occurred during antibiotic treatment with metronidazole and ceftazidime with an elevation of liver enzymes consistent with hepatocellular damage pattern.Interventions:Hydration with glucose 5% solution, pantoprazole and vitamin K were administered, meanwhile other causes of hepatitis were ruled out and the ongoing antibiotic treatment was stopped suspecting a drug-induced liver injury.Outcomes:Liver function nearly completely recovered 1 month later with a first rapid improvement, within few days, of aminotransferases and coagulation studies, and slower of cholestatic enzymes.Lessons:We describe the first case available in the literature of hepatotoxicity associated with metronidazole treatment in a xeroderma pigmentosum patient. Clinicians therefore, based on this report and according to the possible underlying mechanism shared by other genetic diseases characterized by alterations in the pathway of DNA-repair, should consider such adverse event also in patients affected by this rare disease.     

肝小静脉闭塞病2例报道

一、临床资料 1．患者1，男，49岁。入院1个月前无明显诱因出现腹胀， 饮食减少，尿量减少。当地医院诊断“肝硬化”，经多次放腹水 及补充白蛋白，利尿等治疗后症状无明显缓解，1个月内体重增 加3kg。于2004年8月20日收住我科。入院查体：皮肤巩膜轻 度黄染、无出血点，无水肿，可见肝掌，未见蜘蛛痣。心肺体 检未见异常。腹部膨隆，未见脐周和侧腹壁静脉曲张，腹部无     

A case of pulmonary veno-occlusive disease associated with systemic sclerosis

The case of a patient with pulmonary veno-occlusive disease associated with systemic sclerosis is reported. The patient presented with progressive dyspnoea. Echocardiography and cardiac catheterization study demonstrated right-sided heart failure. The CXR suggested pulmonary hypertension and interstitial pulmonary oedema. We suspected pulmonary veno-occlusive disease based on radiological and haemodynamic findings. Treatment with prednisolone resulted in a reduction in pulmonary arterial pressure and CXR findings improved 2 months later, but no further effect was observed. The patient died 7 months later and at autopsy the lungs showed prominent thickening of the interlobular septa and small branches of pulmonary veins showed intimal thickening.