A case of intraductal tubulopapillary neoplasm of the pancreas in a branch duct: a rare case report and literature review.

BACKGROUND: Intraductal tubulopapillary neoplasm (ITPN) of the pancreas is a new disease concept defined by the World Health Organization in 2010. ITPN progresses with tubulopapillary growth in the pancreatic duct and is known to have a fair prognosis. Localization in the main pancreatic duct (MPD) is one characteristic. There are few case reports of ITPN in a branch of the pancreatic duct (BD). CASE PRESENTATION: We encountered a case of ITPN localized in BD. An 85-year-old man was followed after colonic surgery for rectal carcinoma. An abdominal computed tomography scan revealed a cystic mass in the pancreatic head and further examination was done. A T2 weighted intension picture in magnetic resonance imaging showed a 20 mm cystic lesion with an internal mass of 15 mm. Duodenal papilla were slightly open and endoscopic retrograde pancreatography revealed mild and diffuse dilatation of the main pancreatic duct and mucin in the MPD. In consideration with the image examinations, we diagnosed the tumor as an intraductal papillary mucinous neoplasm with carcinoma because of its large mural nodule (> 10 mm in size) in a cyst. Consequently, a pancreaticoduodenectomy was performed. Macroscopically, a white solid tumor sized 2.5 × 1.8 × 1.0 was identified in the head of the pancreas. The cut surface of the resected pancreas showed a side-branch type intraductal tumor with tubulopapillary architecture without mucin secretion. Immunohistochemical staining was positive for MUC1, and negative for MUC2 and MUC5AC. The final diagnosis was determined to be pancreatic ITPN from BD. At the time of this report (48 months post-surgery), the patient remains disease-free without evidence of recurrence. CONCLUSION: ITPNs localized in BD are rare and diagnosis prior to surgery is difficult. In our case, the shape was round, not papillary, and with little fluid. These characteristics are different from a branch duct type IPMN and can be a clue to suspect ITPN in BD.

A case of Turcot's syndrome type 1 with loss of immunoexpression of MSH6 in colon cancer and liver metastasis due to secondary somatic mutation in coding mononucleotide (C)8 tract: a case report.

BACKGROUND: Lynch syndrome (LS), which is known as a hereditary cancer syndrome, is distinguished by microsatellite instability, represented by the altered number of repetitive sequences in the coding and/or non-coding region. Immunohistochemical staining (IHC) of DNA mismatch repair (MMR) proteins (e.g., MLH1, MSH2, MSH6, and PMS2) has been recognized as an useful technique for screening of LS. Previous study has shown that the assessment of IHC, however, requires specific caution due to variable staining patterns even without germline mutations in MMR genes. CASE PRESENTATION: A 48-year-old man, who had been treated for anaplastic astrocytoma, was referred to our department for the precise examination of progressing anemia. Whole-body examination revealed two advanced carcinomas in descending colon and stomach. A hypo-vascular mass lesion was detected in liver as well. Pathological diagnosis (on surgical specimens) was poorly differentiated adenocarcinoma in descending colon, moderately differentiated tubular adenocarcinoma in stomach, and liver metastasis, which is possibly from colon. It was suspected that this case would be Turcot's syndrome-type-1 due to its specific family history having two cases of colon cancer within the second relatives. Pathogenic frameshift mutations in codon 618 of MLH1 gene was identified. Immunohistochemical analyses (IHC) demonstrated complete loss of MLH1 immuno-expression as well as of PMS2 except for those in brain tumor. Although frameshift mutation was not found in MSH6 gene, histological expression of MSH6 was patchy in primary colon carcinoma and was completely lost in the metastatic site in liver. MSH6 expression in gastric carcinoma, a coincidental cancer in this case, was intact. An abnormal (C)8 region was identified by the cloned PCR of colon and liver tumors but not from gastric cancer. Frameshift mutation in a (C)8 tract in exon 5 of the MSH6 gene was also detected in liver metastasis. CONCLUSION: This case supports a plausible mechanism, proposed by a previous literature, for the reduced expression of MSH6 in a somatic mutation manner, which might preferentially happen in colon cancer rather than in stomach carcinoma in MLH1/PMS2-deficient type of Turcot's syndrome type 1.

Feasibility and efficacy of repeat laparoscopic liver resection for recurrent hepatocellular carcinoma.

BACKGROUND: Repeat hepatectomy is an acceptable treatment for recurrent hepatocellular carcinoma (HCC). However, repeat laparoscopic liver resection (LLR) has not been widely adopted due to its technical difficulty. This study aimed to assess the feasibility and efficacy of repeat LLR compared with repeat open liver resection (OLR) for recurrent HCC. METHODS: We performed 42 repeat OLR and 30 repeat LLR for cases of recurrent HCC between January 2007 and March 2018. This study retrospectively compared the patients' clinicopathological characteristics and operative and short-term outcomes including surgical time, intraoperative blood loss, duration of hospital stay, and postoperative complications between the two groups. RESULTS: There were no significant differences in patient characteristics between the two groups except in terms of Child-Pugh grade. The repeat LLR group had lower median intraoperative blood loss (100 mL vs. 435 mL; P = 0.001) and shorter median postoperative hospital stay (10 days vs. 14.5 days; P = 0.002). The other results including postoperative complications were comparable between the two groups. Further, comparison of two subpopulations of the repeat LLR group stratified by previous hepatectomy type (open or laparoscopic) or tumor location (segments 7 and 8 or other) revealed no significant differences in the postoperative clinical characteristics between them, although the morbidity rate tended to be higher in patients who underwent open hepatectomy for primary HCC than in patients who underwent laparoscopic hepatectomy. CONCLUSIONS: Repeat LLR for recurrent HCC is feasible and useful with good short-term outcomes although an appropriate patient selection seems to be necessary.

[MSI-H Small Bowel Cancer with Peritoneal Dissemination Successfully Treated with Pembrolizumab-A Case Report].

A 52-year-old man was diagnosed with small bowel adenocarcinoma(T4aN1M0, Stage ⅢA, according to the Japanese colorectal cancer classification)and treated with partial resection of the small bowel in June 2014. He also received adjuvant chemotherapy(XELOX: 8 courses)after surgery. Three and a half years after the operation, peritoneal dissemination recurred, and he received bevacizumab plus XELOX therapy. The regimen was adjusted to a total of 11 courses because of the disease progression. The primary lesion showed MSI-H. The patient was started on pembrolizumab therapy in April 2019. The tumor responded well to pembrolizumab(maximum therapeutic effect: PR, 31% reduction), but a new lesion appeared 6 months after the start of this regimen. He continued pembrolizumab therapy for 14 months without adverse events since it appeared to be clinically effective. Although MSI-H small bowel cancers are rare, accurate screening is essential to not miss the opportunity to administer pembrolizumab.

[A Case of Sigmoid Colon Cancer Recurrence at the Hand-Sewn Anastomosis Site Originated from Tumor Implantation].

We report a case of anastomotic recurrence following laparoscopic sigmoidectomy with hand-sewn anastomosis, which was attributable to the implantation of exfoliated cancer cells. A 78-year-old man diagnosed with early colon cancer underwent endoscopic submucosal dissection(ESD); however, ESD was suspended due to infiltrated muscle fibers. Subsequently, he underwent laparoscopic sigmoidectomy with hand-sewn anastomosis, accompanied by D3 lymph node dissection. Histopathological findings revealed a well-differentiated tubular adenocarcinoma, pT2(MP), tub1>tub2>por2, ly0, v1, PM0, DM0, RM0, N0M0, pStage Ⅰ. The follow-up CT 6 months after surgery, showed enhanced wall thickening and irregular surface of the sigmoid colon. Colonoscopy revealed a type 2 tumor located on the anastomotic line. Based on the diagnosis of anastomotic recurrence, the patient underwent partial colectomy. Histopathological findings were similar to those of the primary tumor and suggested implantation of exfoliated cancer cells as the origin of anastomotic recurrence. Cancer cells had infiltrated all layers. In conclusion, we recommend the performance of appropriate operative procedures to prevent anastomotic recurrence, such as the cleaning of the anastomosed intestinal tract. Careful follow-up in colon cancer patients is of the utmost importance and the risk of anastomotic recurrence should always be considered.

Cotransplantation of preactivated mesenchymal stem cells improves intraportal engraftment of islets by inhibiting liver natural killer cells in mice.

The activation of natural killer (NK) cells in the liver inhibits engraftment of intraportally transplanted islets. We attempted to modulate the activity of NK cells by cotransplanting mesenchymal stem cells (MSCs) with islets in mice. We first investigated the ability of MSCs to secrete prostaglandin E2 , a predominant inhibitor of NK cell function, in various combinations of inflammatory cytokines. Notably, we found that prostaglandin E2 production was partially delayed in MSCs activated by inflammatory cytokines in vitro, whereas liver NK cells were activated early after islet transplant in vivo. Accordingly, preactivated MSCs, but not naive MSCs, substantially suppressed the expression of activation markers in liver NK cells after cotransplant with islets. Similarly, cotransplant with preactivated MSCs, but not naive MSCs, markedly improved the survival of islet grafts. These results highlight MSC cotransplant as an effective and clinically feasible method for enhancing engraftment efficiency.

[Two Cases of Colorectal Liver Metastasis with Localized Biliary Dilatation].

We encountered 2 cases of colorectal liver metastasis with biliarydilatation mimicking cholangiocarcinoma. Case 1: A 70- year-old male patient, who was diagnosed with colorectal cancer and underwent transverse colectomy3 years prior, was preoperativelydiagnosed with cholangiocarcinoma with biliarydilatation of the medial and lateral segments. He underwent left hemi-hepatectomy. The pathological diagnosis was colorectal liver metastasis with intra-biliarytumor thrombosis. Case 2: A 67-year-old male patient was diagnosed with descending colon cancer and cholangiocarcinoma with biliarydilatation of the medial segment. He underwent left hemi-colectomyand left hemi-hepatectomy. The pathological diagnosis was descending colon cancer and colorectal liver metastasis with biliaryinfiltration. The immunopathological findings showed double positivityfor CK20 and CDX2 antibodies and negativityfor CK7 antibodyin these cancer lesions.

[A Case of Advanced Gastric Cancer with Para-Aortic Lymph Node Metastasis Treated with Conversion Surgery after S-1 plus Oxaliplatin Chemotherapy].

A 71-year-old man was diagnosed as having Type 2 gastric cancer(tub2, HER2-negative). Abdominal computed tomography( CT)revealed bulky metastatic lymph nodes around the stomach and para-aorta(No. 16a2, b1). Our clinical diagnosis was cT4a(SE)N+M1(PAN), cStage Ⅳb, and SOX therapy was immediately administered. After 3 courses of chemotherapy, the treatment effect was PR, and after 6 courses, the patient was diagnosed with ycT2(MP)N0M0, ycStageⅠB. No Grade 2 or higher adverse events were observed during chemotherapy. At this stage, we determined that radical resection was feasible; thus, distal gastrectomy and D3 dissection(para-aortic lymph node dissection)were performed. No cancer cells were found in the primary lesion on histopathology. The histological response of the primary lesion was Grade 3, and the lymph node was Grade 2b. On follow-up observation, the patient is alive without tumor recurrence at 1 year postoperatively.

[Surgical Resection of Hepatic Portal Lymph Node Metastasis after Repeated Treatment for Hepatocellular Carcinoma Recurrence].

We encountered a case of hepatic portal lymph node metastasis after repeated treatment for hepatocellular carcinoma (HCC)recurrence. A 73-year-old male patient underwent partial gastrectomy following rupture of a gastrointestinal stromal tumor 8 years ago. A 70mm tumor was simultaneously revealed in the posterior segment of the liver, and imatinib treatment was initiated based on the diagnosis of a metastatic liver tumor. Due to the absence of an increasing tendency in the tumor, extended posterior segmentectomy was performed, and the pathological diagnosis was moderately differentiated HCC. During observation, transcatheter arterial chemoembolization(TACE)plus radiofrequency ablation(RFA)therapy was performed twice, and partial resection of the liver was performed once again for HCC recurrence. Recently, PIVKA-Ⅱ showed a high value of 1,720mAU/mL, and follow-up computed tomography showed HCC recurrence in S4/8 and hepatic portal lymph node metastasis. TACE was administered for recurrent lesions in S4/8, and surgical resection of the hepatic portal lymph node was performed together. The pathological diagnosis revealed extensive liver tissue necrosis and moderately-topoorly differentiated HCC in the excised lymph nodes. Lymph node metastasis of HCC is rare, and in this case, a change in lymph flow caused by repeated treatment for HCC recurrence was considered a factor influencing the course.

Asunaprevir and daclatasvir for recurrent hepatitis C after liver transplantation: A Japanese multicenter experience.

The safety and efficacy of an IFN-free regimen using asunaprevir (ASV) and daclatasvir (DCV) for recurrent hepatitis C virus (HCV) infection after liver transplantation (LT) have not been evaluated in Japan. A multicenter study of LT recipients (n = 74) with recurrent HCV genotype 1b infection treated with ASV-DCV for 24 weeks was performed. Medical history was positive for pegylated interferon and ribavirin (Peg-IFN/RBV) in 40 (54.1%) patients, and for simeprevir (SMV) with Peg-IFN/RBV in 12 (16.2%) patients. Resistance-associated variants (RAVs) were positive at D168 (n = 1) in the NS3, and at L31 (n = 4), Y93 (n = 4), and L31/Y93 (n = 1) in the NS5A region of the HCV genome. Sixty-one (82.4%) patients completed the 24-week treatment protocol. Although sustained viral response (SVR) was achieved in 49 (80.3%) patients, it was achieved in only two (16.7%) patients among those with histories of receiving SMV (n = 12). Univariate analysis showed that a history of SMV (P < .01) and the presence of mutations in NS5A (P = .02) were the significant factors for no-SVR. By excluding the patients with either a history of SMV-based treatment or RAVs in NS3/NS5A, the SVR rate was 96.4%. By excluding the patients with a history of SMV and those with RAVs in NS3/NS5A, viral clearance of ASV-DCV was favorable, with a high SVR rate.

Perforation of the gallbladder in a patient with acute cytomegalovirus cholecystitis shortly following renal transplantation.

A 74-year-old man with end-stage renal failure secondary to diabetes received a living donor renal transplant (cytomegalovirus [CMV]-seropositive recipient from a CMV-seropositive donor). Computed tomography scan revealed a gallbladder with hemorrhage. On postoperative day 27, cholecystography revealed gallbladder perforation; he underwent an emergency operation. Histological examination of the gallbladder wall was positive for multiple viral inclusion bodies. We report a very rare case of both hemorrhagic and perforated CMV cholecystitis within a month following renal transplantation.

Prediction of recurrence following hepatectomy in patients with hepatitis C virus infection-related hepatocellular carcinoma who achieved a sustained virological response.

AIM: The risk of hepatitis C virus infection-related hepatocellular carcinoma (HCC) is lower, with a better prognosis, in patients who achieve a sustained virological response (SVR) than in those who do not. We aimed to identify risk factors of post-hepatectomy HCC recurrence in patients who achieved a SVR. METHODS: This retrospective study included 349 HCC patients who underwent an initial radical hepatectomy at our institution between January 2005 and December 2014. Sixty-eight patients had achieved a SVR (the SVR group) and 281 patients had not (the non-SVR group). Clinical characteristics and long-term outcomes were compared between the two groups. Univariate and multivariate analyses identified variables associated with recurrence-free survival in the SVR group. RESULTS: Post-hepatectomy overall and recurrence-free survival rates were significantly higher in the SVR group than the non-SVR group (P < 0.01 and <0.05, respectively). Univariate analysis of post-hepatectomy recurrence-free survival in the SVR group revealed multiple significant factors: aspartate aminotransferase, 25 IU/L or more (P = 0.01); indocyanine green retention rate at 15 min, 20.0% or less (P < 0.05); hepatic vascular invasion (P < 0.05); and an interval of months or less between achieving a SVR and hepatectomy (P < 0.01). Multivariate analysis confirmed an interval of 30 months or less between achieving a SVR and hepatectomy as an independent prognostic factor of recurrence-free survival (hazard ratio, 2.30; 95.0% confidence interval, 1.04-5.13; P < 0.05). CONCLUSION: The interval between achieving a SVR and hepatectomy is an important predictor of recurrence in hepatitis C virus infection-related HCC patients who achieved a SVR.

Hybrid Surgery for Portosystemic Encephalopathy in a Patient with Liver Cirrhosis: a case report.

Regarding the treatment for a portosystemic shunt, surgical or interventional radiological closure of the shunt was established. Interventional radiology including balloon-occluded retrograde transvenous obliteration can worsen portal hypertension and create a large thrombus close to the major venous system in the case of a huge portosystemic shunt. In contrast, it is also difficult to treat some cases through surgery alone when huge complicated shunts exist very deep in the body. Herein, we report a successful case of surgical shunt ligation for portosystemic encephalopathy in a hybrid operation room that enabled intraoperative angiography and computed tomography. A 62-year-old woman with chronic hepatitis C was referred to our hospital due to high levels of serum ammonia and hepatic encephalopathy. She had a massive, complicated portosystemic shunt from the inferior mesenteric vein to the left renal vein but did not have esophageal or gastric varices. It was difficult to occlude the portosystemic shunt by interventional radiologic techniques because the shunt had an extremely large amount of blood flow and many collateral routes. We performed the shunt ligation in the hybrid operation room. Intraoperative angiography provided detailed information about the portosystemic shunt, such as direction or volume of blood flow and collateral routes in real time. Her encephalopathy disappeared completely and she remains healthy with improved liver functional reserve to date. In conclusion, this is a successful case of a hybrid operation for an extremely large and complicated portosystemic shunt, providing for intraoperative angiography as a safe and reliable surgical treatment for portosystemic encephalopathy in patients with liver cirrhosis.

Prediction of long-term survival by using the Glasgow Prognostic Score in patients with hepatocellular carcinoma after liver transplantation.

AIM: This study aimed to validate the prognostic value of the Glasgow Prognostic Score (GPS) in patients who underwent living donor liver transplantation (LDLT) for hepatocellular carcinoma (HCC). METHODS: Data were retrospectively collected for HCC patients who underwent LDLT. Univariate and multivariate analyses were performed to identify variables associated with overall and recurrence-free survival. Patients were separated into two groups based on the GPS. The predictive ability of the GPS was compared with other variable scores (modified GPS and hepatic GPS) to identify the most accurate test for this population. RESULTS: Univariate analysis identified a GPS of 1 or 2 (P = 0.018), multiple tumors (P = 0.032) and HCC beyond the Milan criteria (P = 0.015) as factors significantly associated with poor overall survival. Hepatitis B negative status (P = 0.034), a GPS of 1 or 2 (P = 0.030), α-fetoprotein of 20.0 ng/mL or more (P = 0.042) and HCC beyond the Milan criteria (P = 0.002) were identified as factors significantly associated with poor recurrence-free survival. Multivariate analysis indicated that HCC beyond the Milan criteria and a GPS of 1 or 2 were independent risk factors for long-term outcomes. Among the scoring systems evaluated in this study, the GPS was the best predictive system in our population. Moreover, a new scoring system that combined the GPS and the Milan criteria showed the highest prognostic values. CONCLUSION: Novel prediction scores combining the GPS and the Milan criteria provide a precise, objective, and quick approach for selecting appropriate HCC patients for LDLT.

Simeprevir or telaprevir with peginterferon and ribavirin for recurrent hepatitis C after living-donor liver transplantation: A Japanese multicenter experience.

AIM: This study aimed to clarify the efficacy and safety of simeprevir, a second-generation NS3/4A inhibitor, with peginterferon and ribavirin for recurrent hepatitis C after liver transplantation. METHODS: A retrospective cohort study of living-donor liver transplant recipients with recurrent hepatitis C with the hepatitis C virus genotype 1 treated with either simeprevir- or telaprevir-based triple therapy was carried out at eight Japanese liver transplant centers. RESULTS: Simeprevir- and telaprevir-based triple therapies were given to 79 and 36 patients, respectively. Of the 79 patients treated with simeprevir-based triple therapy, 44 (56%) achieved sustained virological response 12 weeks (SVR12) after treatment ended, and there was no significant difference in the SVR12 between the simeprevir- and telaprevir-based triple therapy groups (69%). The rates of adverse events were not significantly different between the simeprevir- and telaprevir-based triple therapy groups, although the rate of patients who received blood cell transfusion and erythropoietin due to anemia and had renal insufficiency were significantly higher in the telaprevir group than in the simeprevir group. Three baseline factors, the presence of prior dual therapy with peginterferon and ribavirin (P = 0.001), a non-responder to the prior dual therapy (P < 0.001), and male sex (P = 0.040), were identified as significant predictive factors for non-SVR with simeprevir-based triple therapy. CONCLUSION: Simeprevir-based triple therapy for recurrent hepatitis C after living-donor liver transplantation resulted in a high SVR rate and good tolerability, especially in treatment-naïve patients.

Efficacy and Feasibility of Salvage Living Donor Liver Transplantation after Initial Liver Resection in Patients with Hepatocellular Carcinoma.

BACKGROUND/AIMS: Liver transplantation (LT) is promising method of treatment for hepatocellular carcinoma (HCC) patients, but is limited by donor organ shortages and tumor progression during long wait periods. This study investigated the efficacy of salvage living donor LT (LDLT) after initial liver resection (LR) in HCC patients. METHODS: Sixty patients with HCC who underwent primary LDLT (n = 45) or salvage LDLT after initial LR (n = 15) were enrolled. Significant prognostic variables determined by univariate analysis were subjected to multivariate analysis using a Cox proportional hazard regression model. Cox proportional hazards models with inverse probability of treatment weighting (IPTW) based on propensity score were used to adjust for selection bias between groups. RESULTS: The salvage group had significantly higher Child-Pugh class A (p = 0.003), ≥3 pretransplant treatments (p = 0.007), and reoperation rates for postoperative bleeding (p = 0.032) than the primary LDLT group, whereas overall and recurrence-free survival rates were comparable. After IPTW matching, the salvage LDLT group had significantly more reoperations for postoperative bleeding (hazard ratio 7.948, p = 0.017). CONCLUSIONS: First-line LR followed by salvage LDLT allows survival equal to that of primary LDLT. Salvage LDLT following primary LR could be an effective therapy.

Successful resolution of very severe hepatopulmonary syndrome following adult-to-adult living donor liver transplantation: Report of two cases.

Hepatopulmonary syndrome (HPS) is a severe complication in patients with chronic liver disease with poor prognosis. Liver transplantation (LT) is a promising treatment for HPS; however, very severe HPS, which is defined by an arterial oxygen pressure (PaO2 ) of less than 50 mmHg and a right-left intrapulmonary shunt rate of more than 20%, may be a contraindication to LT, including living donor LT (LDLT). Here, we report two cases of decompensated liver cirrhosis with very severe HPS which were resolved after adult-to-adult LDLT including ABO-incompatible LDLT. Both patients required oxygen supportive therapy in combination with specialized respiratory care postoperatively, followed by improvement of oxygenation and substantial decreases of intrapulmonary shunt rate. These findings suggest very severe HPS can be resolved by LDLT, including ABO-incompatible LDLT, and reduced graft volume did not impede the reversal of intrapulmonary shunting. Our current report may indicate that adult-to-adult LDLT, including ABO-incompatible LDLT, is becoming an effective therapeutic method and prompt a review of previous reports as well as our own files with particular regard to the indication of LDLT for decompensated liver cirrhosis with very severe HPS.

Sustained virological response to antiviral therapy improves survival rate in patients with recurrent hepatitis C virus infection after liver transplantation.

AIM: Previous European and North American studies analyzed the relationship between survival rate and sustained virological response (SVR) to interferon (IFN) therapy in patients with recurrent hepatitis C viral (HCV) infection after liver transplantation (LT). The present study was designed to define the same relationship in Japanese patients who had undergone LT. METHODS: Forty-seven patients (genotype 1, 40; genotype 2, 7) with recurrent HCV after LT were treated with pegylated interferon (PEG IFN) or IFN/ribavirin (RBV). In possible, within 3 months after LT, patients started treatment with PEG IFN-α-2b or IFN-α-2b s.c. once weekly combined with RBV (200 mg/day). RESULTS: The SVR rate was 51% (24/47) for all patients, 42.5% (17/40) for genotype 1 and 100% (7/7) for genotype 2. The median follow-up period was 71 months (range, 24-152). The survival rate of 24 patients who achieved SVR was 95% at 5 years and 92% at 10 years. These rates were significantly better than those of 23 patients who did not achieve SVR (82% at 5 years, 58% at 10 years) (P = 0.027). Two patients of the SVR group died during follow up (due to hepatocellular carcinoma in one and chronic rejection in one), while six non-SVR patients died during the same period (three died due to liver failure by recurrent HCV). CONCLUSION: SVR following IFN therapy contributes to improvement of survival rate in patients with recurrent post-LT HCV infection.

Treatment of hepatic amyloid light-chain amyloidosis with bortezomib and dexamethasone in a liver transplant patient.

Hepatic amyloid light-chain (AL) amyloidosis is characterized by abnormal deposition of amyloid fibrils in the liver. As this precursor protein is produced by a proliferative plasma cell clone in the bone marrow, liver transplantation (LT) does not affect the disease's progression. Here, we describe the successful treatment using bortezomib- and dexamethasone-based chemotherapy, following LT, of hepatic AL amyloidosis in a 65-year-old woman with progressive liver failure. The patient presented with progressive hepatic dysfunction accompanied by hepatorenal syndrome requiring hemodialysis, and living donor LT was successfully performed. Histology revealed amyloid deposits in the liver and stomach, and serum immunofixation revealed AL amyloidosis (κ-type). The patient began chemotherapy on day 45 after the LT, and remission was achieved after one course. She was subsequently discharged 83 days after the LT, with normal liver and renal function, and no clinical evidence of recurrent disease was observed at the latest follow up (22 months post-LT).

Low skeletal muscle mass index is independently associated with low bone mineral density in kidney transplant recipients: a retrospective observational cohort study.

BACKGROUND: Osteoporosis and osteopenia are more frequent in patients who have received kidney transplants than in healthy individuals. Although osteoporosis and sarcopenia are closely related, only few studies have considered them in the post-transplantation period. We aimed to investigate the relationship between lower bone mineral density and skeletal muscle in kidney transplant recipients. METHODS: We included 371 patients in the maintenance phase of kidney transplantation (> 6 months after transplantation) followed-up at our institution from January to December 2019. The primary endpoint was the association between bone mineral density and skeletal muscle mass index. As secondary endpoints, in addition to skeletal muscle mass index, we investigated other factors associated with low bone mineral density, including kidney function and 25-hydroxy vitamin D (25(OH)D) concentration. Considering the possibility that factors affecting bone mineral density differ between men and women, we explored these factors separately for both sexes. RESULTS: Of the 371 participants, 243 (65.4%) were men. The median age and time after transplantation were 52 and 14 years, respectively. Univariate analysis showed that age, female sex, time since transplantation, cystatin C-based estimated glomerular filtration rate (eGFRcysC), 25(OH)D, and skeletal muscle mass index were associated with bone mineral density. Multivariate analysis showed associations of bone mineral density with eGFRcysC, 25(OH)D, and skeletal muscle mass index. Multivariate analysis by sex showed significant associations with eGFRcysC, hemoglobin, and skeletal muscle mass index in men and with age, eGFRcysC, albumin, and skeletal muscle mass index in women. Bone mineral density was not associated with history of dialysis prior to transplantation or time since transplantation. CONCLUSIONS: In kidney transplant recipients, an independent association between lower bone mineral density and skeletal muscle mass index was observed in both sexes.