Treatment of sporadic Burkitt lymphoma in adults,a retrospective comparison of four treatment regimens

Burkitt lymphoma is an aggressive B cell malignancy accounting for 1–2% of all adult lymphomas. Treatment with dose-intensive, multi-agent chemotherapy is effective but associated with considerable toxicity. In this observational study, we compared real-world efficacy, toxicity, and costs of four frequently employed treatment strategies for Burkitt lymphoma: the Lymphome Malins B (LMB), the Berlin-Frankfurt-Münster (BFM), the HOVON, and the CODOX-M/IVAC regimens. We collected data from 147 adult patients treated in eight referral centers. Following central pathology assessment, 105 of these cases were accepted as Burkitt lymphoma, resulting in the following treatment groups: LMB 36 patients, BFM 19 patients, HOVON 29 patients, and CODOX-M/IVAC 21 patients (median age 39 years, range 14–74; mean duration of follow-up 47 months). There was no significant difference between age, sex ratio, disease stage, or percentage HIV-positive patients between the treatment groups. Five-year progression-free survival (69%, p = 0.966) and 5-year overall survival (69%, p = 0.981) were comparable for all treatment groups. Treatment-related toxicity was also comparable with only hepatotoxicity seen more frequently in the CODOX/M-IVAC group (p = 0.004). Costs were determined by the number of rituximab gifts and the number of inpatients days. Overall, CODOX-M/IVAC had the most beneficial profile with regards to costs, treatment duration, and percentage of patients completing planned treatment. We conclude that the four treatment protocols for Burkitt lymphoma yield nearly identical results with regards to efficacy and safety but differ in treatment duration and costs. These differences may help guide future choice of treatment.     

Primary extrahepatic portal vein obstruction in adults: A single center experience

Background

Extrahepatic portal venous obstruction (EHPVO) is a heterogenous disease with regards to etiology, pathogenesis, age, and geographical location. This study analyzed our experience with EHPVO in adults aged >20 years. EHPVO associated with pancreatitis and abdominal lymph node tuberculosis compressing the portal vein was excluded.

Methods

Retrospective analysis of the hospital database from January 2000 to December 2009 was done. All patients with liver disease who are attending our department were also prospectively evaluated with Doppler ultrasound for the portal venous system to study the prevalence of EHPVO. Clinical, biochemical, and imaging findings; work up for thrombophilia; treatment given; and follow up were evaluated.

Result

In the retrospective analysis, primary EHPVO in adults was seen in 108/10,095 (1 %), and in the prospective analysis, it was seen in 16/2,188 (0.73 %). The main clinical presentations were abdominal pain and variceal bleed. Imaging findings included portal cavernoma, portal biliopathy, gallbladder calculi, collaterals, and ascites. The major causative factors identified were hyperhomocysteinemia, antiphospholipid antibodies, and myeloproliferative disorders, while, in a third of patients, none of these risk factors could be identified. Twelve patients were subjected to surgery, while the remaining patients were managed either by medical, endoscopic, or interventional radiological techniques. More than 2 years of follow up was available in 90 patients; two patients died due to uncontrolled bleeding, seven patients required surgery, seven patients showed deterioration in liver function, and one patient developed hepatocellular carcinoma. In the prospective study, three patients were subjected to surgery, and the others were managed medically.

Conclusion

Primary EHPVO is an uncommon cause of portal hypertension in adults in India, and its etiological spectrum is comparable to the West.     

Outcome of cardiac surgery in adults with congenital heart disease: A single center experience

ObjectivesAdult survivors with congenital heart diseases represent a large and growing population, yet the published data does not represent the magnitude of their needs specifically in the Middle East. We aimed to review our experience at King Faisal Heart Center, Riyadh, Saudi Arabia for the outcome of adult patients with congenital heart disease who underwent either primary or redo surgery.MethodsA retrospective study at a tertiary care hospital. All patients who underwent surgery either as the first surgery or as a reoperation for congenital heart disease aged >16 years old at the time of cardiac surgery in the period between January 1, 2008 and January 1, 2013. We looked for incidence of postoperative bleeding, arrhythmias, acute kidney injury, neurological complications, duration of mechanical ventilation, hospital and intensive care unit (ICU) stay. Additionally, we assessed the mortality and 1- and 5-year survival.ResultsNinety-eight patients were included in our study. Fifty-two (53%) were females and 46 (47%) were males, with a mean age of 26 ± 8.4 years and a mean weight of 62 ± 22.8 kg. Forty-nine patients (50%) required redo surgery. Ten patients (10%) suffered from postoperative bleeding. Eight patients (8%) had postoperative arrhythmias, of which two patients required permanent pacemaker insertion. Three patients (3%) had postoperative acute kidney injury and seven patients (7%) suffered from neurological complications. The mean duration of ventilation was 1.3 ± 2 days, with a mean ICU and hospital stay of 3.7 ± 3 days, and 10 ± 7 days, respectively. The overall mortality rate in our series was 4% with a 1–5-year survival of 96%.ConclusionAdult patients with congenital heart disease are prone to immediate postoperative multisystem complications, yet the majority of them are reversible. Their 1- and 5-year survival rate is excellent. Further follow up studies are required.     

Clinical characteristics of a group of adults with nodular lymphoid hyperplasia： A single center experience

AIM:To describe the clinical and histological charac-teristics of a group of adults with small-bowel nodularlymphoid hyperplasia(NLH).METHODS:Patients were searched for five years inpathology records of our institution.The biopsy mate-rial was reassessed using strict histopathological criteria.Clinical data were obtained from medical records.RESULTS:Small-bowel NLH was diagnosed in 18 cases.The female:male ratio was 2:1.The most frequentsymptoms were diarrhea(72%),involuntary weight loss(72%)and abdominal pain(61%).Nine patients(50%)had immunodeficiency.Small-bowel bacterial overgrowthwas found in three(17%)cases.At small-bowel NLHdiagnosis,three(17%)had associated lymphoma:twointestinal and one extra-intestinal lymphomas.In twopatients with villous atrophy and anti-endomysial anti-bodies the diagnosis of celiac disease was established.Giardia lamblia infection was found in only one patientwith hypogammaglobulinemia(Herman's syndrome).CONCLUSIONS:NLH is uncommon in adult patients.Associated diseases are immunodeficiency and lymphoidtissue malignancies.     

Clinical features and treatment outcomes of lymphoplasmacytic lymphoma: a single center experience in Korea

Lymphoplasmacytic lymphoma (LPL) constitutes less than 5% of all non-Hodgkin lymphomas, and little is known about clinical features and treatment outcomes for patients with LPL in East Asia. In this study, we summarize our experiences managing patients diagnosed with LPL in Korea. A retrospective analysis was performed using data for 22 patients with LPL diagnosed at Samsung Medical Center. LPL was more common among males (77.3%), with a median age of diagnosis of 63 years (range 26-86). The most common presenting symptom was fatigue related to anemia (59.1%), and the bone marrow was commonly involved at diagnosis (90.9%). IgM paraproteinemia was found in 15 patients, and only one patient had anti-hepatitis C virus. Although some patients could be observed without treatment, the majority of patients required systemic treatment. Chlorambucil alone and cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) or CHOP-like combination chemotherapy was frequently used as a first-line treatment, and a fludarabine-based regimen was commonly used as salvage therapy. However, responses to those treatments were not satisfactory. Even patients who could be monitored without therapy became refractory to salvage therapies once their disease progressed. Eight patients died due to disease progression, and the median overall survival was 70.8 months (95% CI: 31.4-109.2 months). This study describes the clinical features and treatment outcome of LPL in Korea. The treatment approach was too heterogeneous to draw firm conclusions, however, and treatment recommendations in the future should utilize a uniform treatment strategy.     

Autologous stem cell transplantation in advanced follicular lymphoma. A single center experience

BACKGROUND AND OBJECTIVE: The use of intensive therapy supported by autologous stem cell transplantation (ASCT) is being investigated as treatment for poor-prognosis follicular lymphomas (FL). A single-center experience is herein reported. DESIGN AND METHODS: From September 1990 to October 1997, 30 consecutive patients (pts) with advanced FL received transplants, 8 of bone marrow and 22 of peripheral blood. Thirteen harvests were purged by an immunomagnetic method using anti-B antibodies. Twenty-seven patients received salvage chemotherapy (CT) before ASCT with the objective of reaching this procedure in the best possible response. The disease status at ASCT was: 1(st) CR in 7 pts, > or =2(nd) CR in 6 pts, PR in 10 pts, untreated relapse in 2 pts and chemoresistant disease in 5 pts. RESULTS: There was only one transplant-related death (one month after ASTC). With a median follow-up of 19 (1-89) months, 27 pts are alive, 8 pts have relapsed/progressed at a median time of 11 (6-22) months after ASCT. The estimated 2-year PFS and OS are 57% (95% CI, 34-81%) and 83% (95% CI, 64-100%). When comparing the progression-free interval (PFI) before salvage CT and ASCT and the PFI after ASCT, of 17 evaluable pts, 10 had a PFI after ASCT longer than the previous interval, and 5 additional pts remain in CR/PR with a follow-up that has not yet reached the duration of pre-transplant response. By contrast, 2 pts had a short post-transplant response. INTERPRETATION AND CONCLUSIONS: High-dose therapy followed by ASCT obtains a high rate of responses, frequently longer than any previous PFI. Additional follow-up is necessary to determine whether there is any "plateau" in response duration and to define what proportion of pts may be cured with ASCT in this setting.     

Duodenal GIST: a single center experience

Purpose

The duodenum as primary site for gastrointestinal stromal tumors (GISTs) is rare and mitotic rate, tumor size, type of mutation and number of chromosomal aberrations have prognostic implications.

Methods

We analyzed the outcome of 13 patients with duodenal GISTs who underwent surgical tumor resection. Either segmental duodenectomy or pylorus-preserving duodenopancreatectomy was performed. The tumors were histopathologically examined and the risk of progression was assessed based on tumor size and mitotic count. Additionally, mutation analysis of the KIT and PDGFRA receptor tyrosine kinase genes and comparative genomic hybridization (CGH) were performed in all cases.

Results

Eight patients underwent segmental duodenectomy and five patients were treated with pylorus-preserving duodenopancreatectomy. None of the five GISTs with low or no risk for malignancy according to the Miettinen classification developed tumor progress. In contrast, five of eight cases (62.5%) with high-risk tumors revealed tumor progress, and four of these patients died (50%). The median overall survival for all patients was 66 months, and the median disease-free survival 41 months. The operative procedure and type of mutation did not correlate with long-term survival. CGH analysis displayed ?15q in 12/13 tumors, and ?1p in 11/13 cases as characteristic chromosomal aberrations for intestinal origin. Notably, ?22q was present in three of four cases with tumor progress.

Conclusions

Both segmental duodenectomy and pylorus-preserving duodenopancreatectomy are appropriate options to treat duodenal GIST and should be implemented depending on resectability and the patient's performing state. The Miettinen classification and CGH findings correlate with the clinical course.     

Microscopic colitides: a single center experience in Mexico

Background/aim Microscopic colitis is characterized by chronic diarrhea and specific microscopic changes in a macroscopically normal colonic mucosa. In this paper, we report the clinical and histological features of a group of Mexican patients with microscopic colitis. Materials and methods Patients were identified from 10 years of data in the pathology database of our institution. A pathologist reassessed all biopsy material. We included only patients who met strict histopathological criteria. Clinical data were obtained from medical records. Results Microscopic colitis was diagnosed in 26 cases. The female/male ratio was 1.8:1. The median age at diagnosis was 56.5 ± 15.7 (range, 26–85) years. The most frequent symptoms were diarrhea in all, weight loss in 22 (84%), and abdominal pain in 18 (69%) patients. Sixteen (61%) patients reported associated diseases; autoimmune thyroid disorders were the most common. Drug-induced microscopic colitis was suspected in 12 (46%) patients. More than 80% of the treated patients improved or had clinical remission. Conclusion Microscopic colitis is an uncommon colonic disease at our institution, representing less than 1% of the final diagnoses in mucosal colonic biopsies. It is the largest case series of microscopic colitis in the Mexican population.     

Recurrence patterns of mucose-associated lymphoid tissue lymphoma after definitive radiation treatment: A single center experience

Objectives: To evaluate the treatment outcomes in non-gastric and non-nodal mucose-associated lymphoid tissue (MALT) lymphoma patients treated by definitive radiation therapy (RT).

Methods: A total of 134 patients were analyzed. The RT dose was 30.6 or 36?Gy.

Results: The median follow-up duration for all patients was 51.1 months (range, 3.0–132.4 months). Among the 88 orbital MALT lymphoma patients, 12 had disease recurrence. There were 23 head and neck tumor patients. Two patients experienced relapse, all at out-of-field locations. Gastrointestinal MALT lymphoma was diagnosed in 13 patients, and three showed local (n?=?1), distant (n?=?1), or local/distant (n?=?1) relapse.

Conclusion: RT alone is highly effective in achieving local control and long-term survival in localized MALT lymphoma. In orbital MALT, excellent local control is achieved, and relapse is predominantly observed in the contralateral eye. Other head and neck lymphomas are also well controlled.     

应用激光鞘拔除电极导线(附五例报道)

目的探讨应用激光鞘拔除电极导线的安全性、可行性。方法回顾分析北京大学人民医院应用激光鞘拔除电极导线的5例患者资料,总结电极导线拔除原因、电极导线特点、手术相关并发症、手术时间、曝光时间、曝光量等。结果 5例患者(2例女性),年龄(62.8±16)岁,其中1例为Ⅱa类适应证,4例为I类适应证,共拔除电极导线8根(2根除颤电极导线),均成功拔除,无并发症发生,手术时间、曝光时间、曝光量的中位数分别为4 min、1min 35 s、1.305 mgy。结论对于大的、有经验的中心,应用激光鞘拔除电极导线是安全、可行的。     

Pneumatosis cystoides intestinalis: a single center experience

AIM: To share our experience of the management and outcomes of patients with pneumatosis cystoides intestinalis (PCI).METHODS: The charts of seven patients who underwent surgery for PCI between 2001 and 2009 were reviewed retrospectively. Clinical features, diagnoses and surgical interventions of patients with PCI are discussed.RESULTS: Seven patients with PCI (3 males, 4 females; mean age, 50 ± 16.1 years; range, 29-74 years) were analyzed. In three of the patients, abdominal pain was the only complaint, whereas additional vomiting and/or constipation occurred in four. Leukocytosis was detected in four patients, whereas it was within normal limits in three. Subdiaphragmatic free air was observed radiologically in four patients but not in three. Six of the patients underwent an applied laparotomy, whereas one underwent an applied explorative laparoscopy. PCI localized to the small intestine only was detected in four patients, whereas it was localized to the small intestine and the colon in three. Three patients underwent a partial small intestine resection and four did not after PCI was diagnosed. Five patients were diagnosed with secondary PCI and two with primary PCI when the surgical findings and medical history were assessed together. Gastric atony developed in one case only, as a complication during a postoperative follow-up of 5-14 d.CONCLUSION: Although rare, PCI should be considered in the differential diagnosis of acute abdomen. Diagnostic laparoscopy and preoperative radiological tests, including computed tomography, play an important role in confirming the diagnosis.     

Head and neck extranodal lymphoma in a single institute: a 17-year retrospective analysis

The study's purposes are to identify patient characteristics, treatment response and survival rate, and to describe the important prognostic factors for our patients with extranodal head and neck lymphoma. Furthermore, no study has systemically discussed the overall figure of this disease in Taiwan and we analyzed our data on this topic. A retrospective review was performed for 86 patients with extranodal head and neck lymphoma, diagnosed in Kaohsiung Medical University Hospital, between 1990 and 2007. We evaluated the medical records and analyzed the possible factors affecting treatment outcomes, survival rate, and free-from-disease (FFD) survival rate. Forty-nine male and 37 female patients were included with a male:female ratio of 1.32:1. The most frequent histologic type was diffuse large B cell lymphoma, accounting for 41.9% of the total. The most common primary site involved with extranodal head and neck non-Hodgkin's lymphoma was a tonsil with 27 cases (31.4%). Stage, international prognostic index (IPI) score, B symptoms, lactate dehydrogenase (LDH) level, and lymph node status significantly affected treatment response. The overall 5- and 10-year survival rates were 68.0% and 57.8%, respectively. The FFD survival rate was 53.6% and 49.3% at 5 and 10 years, respectively. Factors including stage, lymph node status, LDH level, and IPI score produced significant differences in both overall survival and FFD survival. Our analyzed information is similar to other previously presented studies. Stage, IPI score, B symptoms, LDH level, and neck nodal status can be used to evaluate the treatment outcomes. Neck nodal status and stage are the two significant prognostic factors for overall survival.     

Gastrointestinal manifestations of melioidosis: A single center experience

Melioidosis, being increasing, is reported from India. Gastrointestinal manifestations are typically reported as unusual cause of liver and/or splenic abscess. We aimed to describe various gastrointestinal manifestation of melioidosis in the present study. We retrospectively collected data of culture positive melioidosis cases from hospital database during August 2014–October 2016 at Asian Institute of Gastroenterology, Hyderabad. A total of nine culture positive cases (8 male) of melioidosis with median age of 40 years (range 23–66) were analyzed. Median duration of symptoms was 45 days. Two patients were being treated as tuberculosis. Three patients presented with liver abscess with two of them having simultaneous splenic abscess, and one had prostatic abscess. Three patients (43%) with history of acute pancreatitis had infected pancreatic collection, and one patient had left empyema with splenic abscess. One patient had wound infection with left lower limb cellulitis, presented as acute in chronic liver failure and another as spontaneous bacterial peritonitis (SBP). Diabetes and/or alcoholism was present in all patients. Seven patients had disseminated organ involvement. Seven patients underwent percutaneous intervention for drainage of abscess. Induction therapy as ceftazidime (n=4) or meropenem (n=5) followed by continuation therapy as oral cotrimoxazole (n=6) and doxycycline (n=1) was given. Six patients completed therapy and asymptomatic at end of follow up. Two patients died in the study period. One patient died due to acute-on-chronic liver failure (ACLF) with acute kidney injury and the other due to cardiac failure. One patient with SBP had lost to follow up. Apart from being unusual cause of liver/splenic abscess, melioidosis can present with infection of pancreatic collection, SBP, and infection in a compensated cirrhosis which can precipitate ACLF. Early recognition and specific therapy can improve prognosis.     

Outcomes of Burkitt lymphoma with central nervous system involvement: evidence from a large multicenter cohort study

Central nervous system (CNS) involvement in Burkitt lymphoma poses a major therapeutic challenge, and the relative ability of contemporary regimens to treat CNS involvement remains uncertain. We describe the prognostic significance of CNS involvement and the incidence of CNS recurrence/progression after contemporary immunochemotherapy using real-world clinicopathological data from adults with Burkitt lymphoma diagnosed between 2009 and 2018 in 30 institutions in the USA. We examined associations between baseline CNS involvement, patients’ characteristics, complete response rates, and survival. We also examined risk factors for CNS recurrence. Of 641 patients (aged 18 to 88 years), 120 (19%) had CNS involvement. CNS involvement was independently associated with human immunodeficiency virus infection, poor performance status, involvement of ≥2 extranodal sites, and bone marrow involvement. Selection of the first-line treatment regimen was unaffected by CNS involvement (P=0.93). Patients with CNS disease had significantly lower rates of complete response (59% vs. 77% for patients with and without CNS involvement, respectively; P<0.001), worse 3-year progression-free survival (adjusted hazard ratio [aHR]=1.53, 95% confidence interval [95% CI]: 1.14-2.06; P=0.004) and overall survival (aHR=1.62, 95% CI: 1.18-2.22; P=0.003). The 3-year cumulative incidence of CNS recurrence was 6% (95% CI: 4-8%) and was significantly lower among patients receiving other regimens (CODOX-M/IVAC, 4%, or hyperCVAD/MA, 3%) compared with DA-EPOCH-R (13%; adjusted sub-distribution HR=4.38, 95% CI:, 2.16-8.87; P<0.001). Baseline CNS involvement in Burkitt lymphoma is relatively common and portends inferior prognosis independently of the first-line treatment regimen selected. In real-world practice, regimens including intravenous systemic agents with pronounced CNS penetrance were associated with a lower risk of CNS recurrence. This finding may be influenced by observed suboptimal adherence to the strict CNS staging and intrathecal therapy procedures incorporated in the DA-EPOCH-R regimen.     

起搏器更换相关并发症的单中心分析

目的 回顾性分析阜外心血管病医院起搏器更换手术相关并发症的发生情况.方法 分析2008年6月至2011年6月期间在阜外心血管病医院接受起搏器更换的714例患者.术后3、6、12个月时工作人员通过常规门诊随访或电话随访的方式进行随访,之后改为每年1次,并以电话随访方式进行非定期随访.通过术后定期门诊随访或电话随访了解患者并发症发生情况,平均随访(19.5±9.7)个月.所有与手术相关的并发症均纳入本次研究.结果 随访期间共22例(3.1％)患者出现手术相关的并发症其中,13例(1.8％)出现次要并发症,包括4例起搏器囊袋血肿,2例起搏器移位,6例伤口愈合不良和1例局限性气胸；9例(1.3％)出现主要并发症,包括3例起搏导线脱位,3例严重的囊袋血肿,起搏器囊袋调整1例和2例严重的感染.9例主要并发症患者均接受了再次手术.接受起搏器导线相关操作的患者并发症发生率有增高的趋势.服用抗凝和抗血小板药物并不增加患者术后并发症的风险.结论 尽管由经验丰富的医师进行操作能够降低起搏器更换的并发症发生率,但手术操作仍存在不能忽略的风险.我国植入型心律转复除颤器和心脏再同步治疗起搏器的更换数量较少,对此类起搏器更换术后的并发症情况仍需要进一步观察.