Hemophagocytic syndrome after living-related liver transplantation

Hemophagocytic syndrome (HPS) is a life-threatening hematological disorder in immunocompromised patients. Although the number of patients with HPS following liver transplantation is scarce the outcome is usually fatal. We report a patient who developed HPS following living-related liver transplantation (LRLT) and was treated successfully by a combination of intravenous (IV) immunoglobulin (Ig) and granulocyte colony-stimulating factor (GCSF).     

Recurrent nephrotic syndrome after living-related renal transplantation resistant to plasma exchange: report of two cases

Abstract:  We encountered two patients of recurrent nephrotic syndrome (NS) after renal transplantation that was resistant to plasma exchange (PEX). Case 1 was a 34-year-old man with a living-related renal transplant for type-I membranoproliferative glomerulonephritis (MPGN) related end-stage renal disease (ESRD). He developed overt proteinuria 7 months post-transplant and presented with NS 5 months later. Biopsy of the transplant kidney revealed recurrent type I MPGN, but no features of acute rejection (AR) or chronic allograft nephropathy (CAN). He was treated with cyclophosphamide (CP), oral prednisolone (40 mg/d), an anti-platelet agent, heparin sulfate, and PEX, but the nephrotic state persisted and renal function was deteriorated. He recommenced hemodialysis 3 yr and 9 months after renal transplant. Case 2 was a 47-year-old male who underwent living-related renal transplant for ESRD due to focal segmental glomerulosclerosis (FSGS). He presented with proteinuria shortly after renal transplantation. He also had frequent episodes of AR. Graft biopsy revealed recurrent FSGS. Treatment of pulse methylprednisolone and PEX was transiently effective, but NS relapsed shortly after PEX. Graft biopsy at our hospital showed features of CAN with moderate interstitial fibrosis and tubular atrophy, presence of intraglomerular foam cells but no segmental sclerosis. Treatment with 12 courses of low-density lipoprotein apheresis (LDL-A) reduced proteinuria from 9.6 to 2.0 g/d, and incomplete remission has been maintained for more than 1 yr after LDL-A with slowly progressive renal dysfunction. Despite recent therapeutic advances, including the use of immunosuppressants and PEX, treatment of recurrent disease remains difficult. The LDL-A might be useful in cases with recurrent FSGS resistant to PEX.     

Differential diagnosis between graft-versus-host disease and hemophagocytic syndrome after living-related liver transplantation by mixed lymphocyte reaction assay.

Graft-versus-host disease (GVHD) after liver transplantation is uncommon but is a serious complication that can be fatal. Hemophagocytic syndrome (HPS), which is caused by activation of autologous T lymphocytes, is also a serious complication that can occur after liver transplantation. Because these complications share the clinical triad of skin rash, marrow failure, and diarrhea, differential diagnosis is difficult. We describe a case of severe GVHD resembling HPS in clinical features that occurred after living-related liver transplantation. The patient who had undergone the transplantation had high fever, pancytopenia, and skin rash 3 wk after the operation. Examination of a bone-marrow biopsy sample revealed the presence of abundant monocytes with phagocytosis, suggesting either GVHD or HPS. Donor human leukocyte antigens were detected in the peripheral blood of the patient by polymerase chain reaction, but this finding is not specific for GVHD. A definitive diagnosis was made by demonstration of remarkable anti-self response and undetectable anti-donor response in a mixed lymphocyte reaction assay using carboxyfluorescein diacetate succinimidyl ester.     

Hemophagocytic syndrome after liver transplantation: Report of two cases

We report two cases of hemophagocytic syndrome (HPS), a rare but fatal complication after living-donor liver transplantation (LDLT). Despite their recovery from pancytopenia following treatment with steroid pulse therapy, granulocyte stimulating factor, and intravenous γ-globulin, both patients died. The outcomes reported in cases published in English are devastating, with only 4 survivors among the total 14 patients including ours. Pancytopenia is frequently recognized postoperatively in liver transplant recipients, although its cause is difficult to establish. When pancytopenia accompanying persistent high fever is recognized in LDLT recipients, HPS should be suspected and bone marrow aspiration performed as promptly as possible because of the poor prognosis of this syndrome. There is still no optimal treatment for HPS after liver transplantation.     

Massive bleeding due to hyperfibrinolysis during living-related liver transplantation for terminal liver cirrhosis; report of two cases

We reported two cases of massive bleeding due to critical hyperfibrinolysis during living-related liver transplantation (LRLT) for end stage liver cirrhosis. The total volume of bleeding amounted to 57930 ml with the case 1, and amounted to 55980 ml with the case 2. TEG was useful for diagnosis of the hyperfibrinolysis. We administrated large amounts of FFPs, MAPs, PLTs, and gabexate mesilate. By rapid transfusion, we could manage to finish the procedures without hypotension, and complications were not observed at the early postoperative stage. We thought that the cause of the hyperfibrinolysis is the increasing blood tissue plasminogen activator (t-PA) due to long-anhepatic stage and small graft size. During anesthesia, since the functional start of a transplant liver is indispensable to it, in order to support a transplant liver for an improvement of hyperfibrinolysis, it is important to keep the homeostasis, such as body temperature, blood pressure.     

Idiopathic thrombocytopenic purpura after a living-related liver transplantation

BACKGROUND: Idiopathic thrombocytopenic purpura (ITP) is a rare complication after liver transplantation. We describe three cases of ITP in pediatric patients after a living-related liver transplantation (LRLT). METHODS: Of 266 patients who underwent an LRLT between June 1990 and June 1996, severe thrombocytopenia developed in three pediatric patients after transplantation, and ITP was also diagnosed. The original disease was biliary atresia in all cases, and the patients were given a partial liver graft from a living-related mother and subsequently treated with tacrolimus and low-dose steroids as an immunosuppressive regimen. RESULTS: The duration until the onset of ITP after transplantation in the three cases was 1 day, 3 months, and 13 months, respectively. The platelet-associated IgG levels increased in all cases. A preceding viral infection was suspected in two of the three cases. All patients were treated with intravenous gamma globulin with a transient recovery of thrombocytopenia in two cases and a sustained recovery in another. CONCLUSIONS: Transplant clinicians need to be aware of the possibility of ITP complication because a sudden onset of severe thrombocytopenia can occur even in patients who are apparently doing well after undergoing an LRLT.     

胆道闭锁婴儿亲属活体肝移植术后空肠穿孔四例

目的 分析胆道闭锁婴儿亲属活体肝移植术后空肠穿孔的原因,总结治疗经验.方法 胆道闭锁婴儿行亲属活体肝移植者28例,术后应用环孢素A、糖皮质激素预防排斥反应,部分患儿加用吗替麦考酚酯.结果28例中4例(14.3%)发生空肠穿孔,共发生7次,发生时间平均为术后11 d(8～13 d).4例的穿孔部位均在空肠,其中3例在肠吻合口丝线缝合线脚处,1例在肠袢臂固定丝线线头处.肠吻合口丝线缝合线脚处穿孔的3例经丝线缝合修补穿孔后,其中2例(67%)再次出现穿孔,用prolene线修补后愈合.无患儿因空肠穿孔而死亡.结论 胆道闭锁婴儿行亲属活体肝移植后发生空肠穿孔可能与用丝线吻合肠道有关,可换用Prolene线吻合肠道或修补穿孔.早期诊断和早期剖腹探查对空肠穿孔的治疗至关重要.

Abstract：

Objective To investigate the cause of jejunum perforation after infantile livingrelated liver transplantation (ILRLT) and summarize the experience of treatment. Methods The clinical data of 28 infants with biliary atresia who underwent ILRLT were analyzed and 4 of 28 infantile recipients (14. 3%) developed jejunum perforation after ILDLT. Results Four patients had 7 episodes of jejunum perforation after transplantation among 28 infantile recipients who underwent ILRLT because of biliary atresia. The median time between transplantation and perforation was 11 days.Perforation occurred at the point of silk in jejunum stoma (n = 3) and the Roux-en-Y limb (n = 1 ).None had a history of prior operation including Kasai in 4 patients. Clinical manifestation included fever, increased heart rate, abdominal distention, leukocytosis, and no free air on abdominal roentgenograrns. A simple repair was performed in three infants with silk: two developed recurrent perforation (67%) and underwent a re-exploration,and another had a third perforation and underwent a third repair because of re-perforation. Another child underwent a simple repair with prolene, and there was no recurrence. None died from the perforation in our study. Conclusion The occurrence and location of jejunum perforation after ILDLT suggests that the cause of the perforation is related to the jejunal anastomosis with silk, and the jejunum perforation may be avoided in the jejunal anastomosis with prolene. Early diagnosis and exploration may ensure better survival.     

肝移植术后癫痫发作三例报告

癫痫发作是原位肝移植术后神经系统主要并发症之一,最常发生于肝脏移植术后前2周[1].本文结合我们行肝移植以来遇到的3例并发癫痫病人并参考相关文献,就肝移植术后癫痫发作的病因及防治措施进行讨论.     

Endovascular management of arterial conduit pseudoaneurysm after liver transplantation: a report of two cases

We describe two cases of pseudoaneurysms in liver-transplant iliac artery conduits, which were successfully treated with endovascular stent grafting.     

Postreperfusion syndrome during living-related donor renal transplantation: a case report

Herein we report a case of postreperfusion syndrome (PRS) occurring during renal transplant. PRS, which is defined as a drop in mean arterial blood pressure by at least 30% for a minimum of 1 minute within 5 minutes of reperfusion and classically includes braydycardia and high pulmonary filling pressures, was first described in liver transplantation. Surprisingly, no case of PRS has been previously reported during renal transplantation. CASE REPORT: A 66-year-old woman underwent a living-related renal transplant. Upon completing the vascular anastomosis, arterial and venous clamps were removed to restore kidney perfusion. Subsequently, the patient developed persistent sinus bradycardia at 30 bpm with simultaneous hypotension that lasted for approximately 2 minutes. Although saline boluses, ephedrine, atropine, and 100 microg of epinephrine were administered, the patient's hemodynamics were not restored until an additional 300 microg of epinephrine were administered. CONCLUSION: This case confirms the hypothesis of previous authors who predicted that PRS likely occurs in non-liver transplantation.     

Pontine myelinolysis following liver transplantation: a report of two cases

Two cases of central pontine myelinolysis (CPM) following orthotopic liver transplantation (OLT) are reported. Several months after the onset of this neurological syndrome, the two patients are still alive but with severe neurological sequelae. Some patients undergoing emergency OLT present a high risk of CPM because of pre-existing malnutrition, encephalopathy, and hepatic insufficiency. All of these are associated with an inevitable abrupt rise in sodium serum concentration due to intraoperative compensation of blood losses with high-sodium content blood products. Whenever the renal capacity to excrete sodium is impaired by the surgical procedure, continuous intraoperative venouvenous hemofiltration is recommended.     

Life-threatening veno-occlusive disease after living-related liver transplantation

Veno-occlusive disease (VOD) can develop in association with the administration of cytotoxic chemotherapeutic agents and irradiation. In solid-organ transplant settings, azathioprine has been implicated as a predisposing factor. VOD with fatal outcome occurred in a post liver-transplant recipient who had never been exposed to any agents that have the potential to induce VOD. At onset, the disease manifested clinically as gross ascites and progressive jaundice and was observed after clinically diagnosed acute graft rejection. The disease was confirmed by histologic examinations. Histologic studies of biopsy samples from this patient revealed that most small hepatic veins less than 300 microm in diameter were affected, exhibiting concentric intimal thickening with sparse inflammatory cells. A few of the hepatic veins exhibited active endotheliitis with occasional extension of inflammation to neighboring centrilobular areas. Despite intensified immunosuppression, the observed fibrous obliterative changes were irreversible. Although the cause of VOD in this patient is tentative, the damage to the endothelium, associated with acute rejection, is likely to be attributable. VOD deserves recognition as one of the causes for liver dysfunction and persistent ascites after liver transplantation.     

肝、肾移植后多尿症两例报道

目的 探讨器官移植后发生多尿症原因。方法 1例肝癌患者和1例肾功能衰竭患者分别接受肝移植和肾移植，肝移植患者术后采用他克莫司（FK506）与小剂量甲泼尼龙进行免疫抑制治疗，肾移植患者因肾功能延迟恢复，于术后2周将环孢素A改为FK506，同时服用霉酚酸酯和泼尼松进行免疫抑制治疗。结果 2例患者均在使用FK506后出现多尿、高尿钠和低钠血症，补液和补充氯化钠均无法纠正水、电解质紊乱；当血中FK506浓度升高到10μg/L时出现上述症状，停用FK506后自愈，在此期间血肌酐一直正常。结论 在血中FK506浓度较高时会出现肾小管毒性，导致钠离子的重吸收和分泌障碍，此2例的多尿可能与血中FK506的浓度较高有关。     

桥式背驮式肝移植治疗布加综合征:附六例次临床病例分析

目的探讨肝移植治疗布加综合征的外科新技术。方法1999年以来笔者共为6例布加综合征病人施行了桥式背驮式肝移植,通过血管架桥将供肝的肝上下腔静脉与受者的右心房吻合,供肝的肝下下腔静脉与受者的下腔静脉吻合。结果6例次肝移植术均获成功,未发生手术技术并发症。其中1例术后3个月死于多器官功能衰竭,其他5例仍在随访中,1例已存活近7年,术后均未出现布加综合征复发。结论桥式背驮式肝移植为临床治疗布加综合征提供了一种新的技术途径,减少了技术并发症和布加综合征复发可能。     

肝移植术后门静脉瘤2例并文献复习

目的  总结肝移植术后门静脉瘤的诊疗经验。 方法  回顾性分析2例肝移植术后门静脉瘤患者的临床资料,结合文献复习总结其临床特点、诊断、治疗及预后。 结果  两例肝移植术后门静脉瘤均为肝内型,同时合并门静脉血栓、门静脉高压表现。例1患者给予针对性内科保守治疗,患者拒绝行再次肝移植手术,出院后病情加重,最终因移植肝衰竭、肾衰竭、肺部感染、感染性休克死亡。例2患者给予大剂量糖皮质激素冲击治疗,患者肝功能无改善,接受二次肝移植后恢复顺利。 结论  肝移植术后远期合并门静脉瘤（尤其是肝内型）可能预示预后不良,需正确认识、密切随访、积极处理,适时考虑再次肝移植是可供选择的治疗方案。