Myelodysplastic Syndrome (MDS) and Myeloproliferative Disease (MPD) in Children: A Prospective Registration of 222 Cases

Abstract MDS and MPD are rare in children, however, they are important because the diagnosis is difficult and the prognosis is generally very poor. We conducted a prospective registration of patients suspected of having MDS employing pathological central review through the MDS Committee of the Japanese Society of Pediatric Hematology. Of total, 222 children were enrolled between 1999 and 2006: 96 cases of primary MDS (RA 52, RAEB 25, RAEB-T 19, by FAB classification; RA 28, RCMD 24, RAEB-1 11, RAEB-2 28, AML 5 by WHO classification), 71 cases of JMML (excluding patients with Noonan syndrome and NF1), 1 case of CMML, 19 cases of therapy-related MDS (t-MDS), and 35 cases with constitutional predisposition to MDS. There were128 male and 94 female. The median age at diagnosis was 4.5 years (range, 0.0–19.2 years): primary MDS, 6.8y (0.3–17.4); JMML, 1.5y (0.1–7.1); t-MDS, 11.9y (2.8–19.2); Constitutional MDS, 2.1y (0.0–14.7).The median follow up for 144 surviving patients was 39 months (range 1 to 118 months) after diagnosis. The 3-year overall survival (OS) estimated by Kaplan-Meier analysis, for primary MDS, JMML, t-MDS and constitutional MDS was 73.8%, 62.4%, 69.7% and 65.1% respectively. In primary MDS classified by FAB, OS for RA, RAEB and RAEB-T was 95.9%, 44.7% and 39.5% respectively, while in primary MDS classified by WHO, OS for RA, RCMD, RAEB-1 and RAEB-2 was 100%, 91.0%, 61.4% and 35.0%. Thus, the WHO system could also be employed in children. The data of karyotype were available in 214 of 222 patients (96%). Cytogenetic abnormality was noticed in 55% of primary MDS (40% RA, 72% RAEB, 72% RAEB-T), 27% of JMML and 83% of t-MDS cases. Totally-7/7q-was the most frequent aberration, followed by complex (more than 3 aberrations) and trisomy 8. The del(5q), del(20q), and –Y, which were frequent chromosomal aberrations in adult MDS were rare in children. −7/7q-were observed in primary MDS (27% of abnormal karyotypes) and in JMML (53%). In primary MDS, OS for −7/7q-, complex, others and normal were 50.0%, 25.0%, 79.9% and 86.0%. Thus, the cytogenetic classification defined by the IPSS was useful to predict outcome in childhood primary MDS, however, it was not the case in patients with JMML. Regarding treatment, patients with RA or RCMD were divided into 3 categories: HSCT in 25 (including HSCT after the immunosuppressive therapy [IST] in 6), IST in 15 and no treatment in 10. OS for these categories were 91.6%, 100% and 100%. There were 8 cases of RA with −7/7q-. Six of the 8 cases underwent HSCT but 4 died. The other two received IST and they are long term survivors. The IST therapy could be an option for those with −7/7q-. Patients with RAEB/RAEB-T were divided into 3 categories: HSCT in 12, the AML-type chemotherapy followed by HSCT in 22, and the AML-type chemotherapy in 4. OS for these categories were 53.6%, 28.7% and 37.5%. In HSCT group (n=12), 4 had −7/7q- or complex karyotype. Regarding the donor type, related donors were used in 7 cases. In AML/HSCT group (n=22), 5 had −7/7q- or complex karyotype. Related donors were used only in 6 cases. Chemotherapy induced complete remission (CR) in 11 patients in this group. OS for the patients who attained CR was 40.5% while OS for the others was only 16.0%. Thus, AML-type chemotherapy seemed essential to cure a subset of patients with RAEB/RAEB-T although HSCT without AML-type chemotherapy maybe justified when related donors are available. Regarding JMML, OS (62.4%) was as good as that in Europe. Although we obtained important information, we could not define an optimal management for patients in each subtype of the diseases. Since MDS and MPD in childhood are very rare, an international cooperation is urgently needed to improve treatment outcome.

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Predictors of radiation necrosis in long-term survivors after Gamma Knife stereotactic radiosurgery for brain metastases

Neuro-Oncology Practice ◽

10.1093/nop/npz067 ◽

2019 ◽

Vol 7 (4) ◽

pp. 400-408

Author(s):

Zaid A Siddiqui ◽

Bryan S Squires ◽

Matt D Johnson ◽

Andrew M Baschnagel ◽

Peter Y Chen ◽

...

Keyword(s):

Brain Metastases ◽

Surgical Resection ◽

Radiation Necrosis ◽

Factors Associated ◽

Kaplan Meier ◽

Target Volumes ◽

Gamma Knife Stereotactic Radiosurgery ◽

Long Term Survivors

Abstract Background The long-term risk of necrosis after radiosurgery for brain metastases is uncertain. We aimed to investigate incidence and predictors of radiation necrosis for individuals with more than 1 year of survival after radiosurgery for brain metastases. Methods Patients who had a diagnosis of brain metastases treated between December 2006 and December 2014, who had at least 1 year of survival after first radiosurgery were retrospectively reviewed. Survival was analyzed using the Kaplan-Meier estimator, and the incidence of radiation necrosis was estimated with death or surgical resection as competing risks. Patient and treatment factors associated with radiation necrosis were also analyzed. Results A total of 198 patients with 732 lesions were analyzed. Thirty-four lesions required salvage radiosurgery and 10 required salvage surgical resection. Median follow-up was 24 months. The estimated median survival for this population was 25.4 months. The estimated per-lesion incidence of radiation necrosis at 4 years was 6.8%. Medical or surgical therapy was required for 60% of necrosis events. Tumor volume and male sex were significant factors associated with radiation necrosis. The per-lesions incidence of necrosis for patients undergoing repeat radiosurgery was 33.3% at 4 years. Conclusions In this large series of patients undergoing radiosurgery for brain metastases, patients continued to be at risk for radiation necrosis throughout their first 4 years of survival. Repeat radiosurgery of recurrent lesions greatly exacerbates the risk of radiation necrosis, whereas treatment of larger target volumes increases the risk modestly.

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Prevalence and Characteristics of Survivors from Adult Acute Myeloid Leukemia (AML) in Sweden 2014

Blood ◽

10.1182/blood.v126.23.4888.4888 ◽

2015 ◽

Vol 126 (23) ◽

pp. 4888-4888

Author(s):

Gunnar Juliusson ◽

Vladimir Lazarevic ◽

Petar Antunovic ◽

Asa Rangert Derolf ◽

Hege Garelius ◽

...

Keyword(s):

Conflicts Of Interest ◽

Normal Karyotype ◽

Molecular Data ◽

Complex Karyotype ◽

Monosomy 7 ◽

Previous Diagnosis ◽

Long Term Survivors ◽

Good Risk

Abstract The prevalence of survivors of adult AML has previously been calculated, but the prevalent population has not been characterized. We analysed all 6289 patients diagnosed with adult AML from 1997-2013 as reported in the Swedish AML registry, with a complete survival follow-up. We found 1148 patients, 563 males and 585 females, who were alive on January 1, 2014, constituting 18% of the total, and of them 204 (18%) were diagnosed during 2013. Patients who had their initial treatment at pediatric departments, and people diagnosed with AML before 1997 are not included. The mean age of survivors was 53.4 years at diagnosis and 59.3 years in 2014, and 303 (26%) were 70 years or older in 2014. The overall prevalence of patients diagnosed at age 20 or more was 15.2 per 100,000 (males 15.0 and females 15.4 per 100,000). There were 124 with prior acute promyelocytic leukemia (APL) (11%), 54 with inv(16) (5%), 44 with t(8;21) (4%), and 82 with NPM1 mut/FLT3 wt (7%), 75 with FLT3 -ITD (7%), and 82 with complex karyotype (7%). Karyotype results were not available in 10%, and molecular data only since 2007. Four hundred two (35%) had undergone allogeneic stem cell transplantation, out of which 32 (8%) had had complex karyotype, 43 (11%) FLT3-ITD, 136 (34%) normal karyortype, and 41 (10%) good risk but were transplanted after relapse. Among the 426 non-transplanted prevalent patients who had survived at least 3 years, 94 (22%) had prior APL, 25 (6%) t(8;21), 24 (6%) inv(16), 126 (30%) normal karyotype, and 11 (3%) complex karyotype, including eight monosomal karyotypes and two with del(7) and one del(5q). Among non-complex karyotypes there were four MLL abnormalities, two del(5q) and two monosomy 7. Although long-term survivors more often have pretreatment good-risk features, this patient group is still heterogeneous, and also contains older people and those with intermediate/high risk cyogenetics. Figure 1. Prevalence in January 1, 2014 of Swedish people with a previous diagnosis of AML by sex and current age per 100,000 inhabitants in 2014, and mean incidence 1997-2014 per 100,000 inhabitants in Sweden 2005. Figure 1. Prevalence in January 1, 2014 of Swedish people with a previous diagnosis of AML by sex and current age per 100,000 inhabitants in 2014, and mean incidence 1997-2014 per 100,000 inhabitants in Sweden 2005. Disclosures No relevant conflicts of interest to declare.

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Adult outcome after treatment of pediatric posterior fossa ependymoma: long-term follow-up of a single consecutive institutional series of 22 patients with more than 5 years of survival

Journal of Neurosurgery Pediatrics ◽

10.3171/2020.1.peds19700 ◽

2020 ◽

Vol 26 (1) ◽

pp. 22-26 ◽

Cited By ~ 1

Author(s):

Tryggve Lundar ◽

Bernt Johan Due-Tønnessen ◽

Radek Frič ◽

Petter Brandal ◽

Paulina Due-Tønnessen

Keyword(s):

Proton Beam ◽

Posterior Fossa ◽

Adjuvant Treatment ◽

Tumor Control ◽

Full Time ◽

Time Work ◽

Long Term Follow Up ◽

Long Term Survivors

OBJECTIVEEpendymoma is the third most common posterior fossa tumor in children; however, there is a lack of long-term follow-up data on outcomes after surgical treatment of posterior fossa ependymoma (PFE) in pediatric patients. Therefore, the authors sought to investigate the long-term outcomes of children treated for PFE at their institution.METHODSThe authors performed a retrospective analysis of outcome data from children who underwent treatment for PFE and survived for at least 5 years.RESULTSThe authors identified 22 children (median age at the time of surgery 3 years, range 0–18 years) who underwent primary tumor resection of PFE during the period from 1945 to 2014 and who had at least 5 years of observed survival. None of these 22 patients were lost to follow-up, and they represent the long-term survivors (38%) from a total of 58 pediatric PFE patients treated. Nine (26%) of the 34 children treated during the pre-MRI era (1945–1986) were long-term survivors, while the observed 5-year survival rate in the children treated during the MRI era (1987–2014) was 13 (54%) of 24 patients. The majority of patients (n = 16) received adjuvant radiotherapy, and 4 of these received proton-beam irradiation. Six children had either no adjuvant treatment (n = 3) or only chemotherapy as adjuvant treatment (n = 3). Fourteen patients were alive at the time of this report. According to MRI findings, all of these patients were tumor free except 1 patient (age 78 years) with a known residual tumor after 65 years of event-free survival.Repeat resections for residual or recurrent tumor were performed in 9 patients, mostly for local residual disease with progressive clinical symptoms; 4 patients underwent only 1 repeated resection, whereas 5 patients each had 3 or more resections within 15 years after their initial surgery. At further follow-up, 5 of the patients who underwent a second surgery were found to be dead from the disease with or without undergoing additional resections, which were performed from 6 to 13 years after the second procedure. The other 4 patients, however, were tumor free on the latest follow-up MRI, performed from 6 to 27 years after the last resection. Hence, repeated surgery appears to increase the chance of tumor control in some patients, along with modern (proton-beam) radiotherapy. Six of 8 patients with more than 20 years of survival are in a good clinical condition, 5 of them in full-time work and 1 in part-time work.CONCLUSIONSPediatric PFE occurs mostly in young children, and there is marked risk for local recurrence among 5-year survivors even after gross-total resection and postoperative radiotherapy. Repeated resections are therefore an important part of treatment and may lead to persistent tumor control. Even though the majority of children with PFE die from their tumor disease, some patients survive for more than 50 years with excellent functional outcome and working capacity.

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Microvascular Transposition Without Teflon: A Single Institution's 17-Year Experience Treating Trigeminal Neuralgia

Operative Neurosurgery ◽

10.1093/ons/opaa413 ◽

2021 ◽

Vol 20 (4) ◽

pp. 397-405

Author(s):

Andrew R Pines ◽

Richard J Butterfield ◽

Evelyn L Turcotte ◽

Jose O Garcia ◽

Noel De Lucia ◽

...

Keyword(s):

Multiple Sclerosis ◽

Postoperative Pain ◽

Trigeminal Neuralgia ◽

Fibrin Glue ◽

Microvascular Decompression ◽

Inclusion Criteria ◽

Kaplan Meier ◽

Trigeminal Nerve Root

Abstract BACKGROUND Trigeminal neuralgia (TN) refractory to medical management is often treated with microvascular decompression (MVD) involving the intracranial placement of Teflon. The placement of Teflon is an effective treatment, but does apply distributed pressure to the nerve and has been associated with pain recurrence. OBJECTIVE To report the rate of postoperative pain recurrence in TN patients who underwent MVD surgery using a transposition technique with fibrin glue without Teflon. METHODS Patients were eligible for our study if they were diagnosed with TN, did not have multiple sclerosis, and had an offending vessel that was identified and transposed with fibrin glue at our institution. All eligible patients were given a follow-up survey. We used a Kaplan-Meier (KM) model to estimate overall pain recurrence. RESULTS A total of 102 patients met inclusion criteria, of which 85 (83%) responded to our survey. Overall, 76 (89.4%) participants responded as having no pain recurrence. Approximately 1-yr pain-free KM estimates were 94.1% (n = 83), 5-yr pain-free KM estimates were 94.1% (n = 53), and 10-yr pain-free KM estimates were 83.0% (n = 23). CONCLUSION Treatment for TN with an MVD transposition technique using fibrin glue may avoid some cases of pain recurrence. The percentage of patients in our cohort who remained pain free at a maximum of 17 yr follow-up is on the high end of pain-free rates reported by MVD studies using Teflon. These results indicate that a transposition technique that emphasizes removing any compression near the trigeminal nerve root provides long-term pain-free rates for patients with TN.

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Coronary artery aneurysms: outcomes following medical, percutaneous interventional and surgical management

Open Heart ◽

10.1136/openhrt-2020-001440 ◽

2021 ◽

Vol 8 (1) ◽

pp. e001440

Author(s):

Shameer Khubber ◽

Rajdeep Chana ◽

Chandramohan Meenakshisundaram ◽

Kamal Dhaliwal ◽

Mohomed Gad ◽

...

Keyword(s):

Coronary Artery ◽

Surgical Management ◽

Medical Management ◽

Cox Regression ◽

Artery Bypass ◽

Management Strategies ◽

Coronary Artery Aneurysms ◽

Kaplan Meier

BackgroundCoronary artery aneurysms (CAAs) are increasingly diagnosed on coronary angiography; however, controversies persist regarding their optimal management. In the present study, we analysed the long-term outcomes of patients with CAAs following three different management strategies.MethodsWe performed a retrospective review of patient records with documented CAA diagnosis between 2000 and 2005. Patients were divided into three groups: medical management versus percutaneous coronary intervention (PCI) versus coronary artery bypass grafting (CABG). We analysed the rate of major cardiovascular and cerebrovascular events (MACCEs) over a period of 10 years.ResultsWe identified 458 patients with CAAs (mean age 78±10.5 years, 74.5% men) who received medical therapy (N=230) or underwent PCI (N=52) or CABG (N=176). The incidence of CAAs was 0.7% of the total catheterisation reports. The left anterior descending was the most common coronary artery involved (38%). The median follow-up time was 62 months. The total number of MACCE during follow-up was 155 (33.8%); 91 (39.6%) in the medical management group vs 46 (26.1%) in the CABG group vs 18 (34.6%) in the PCI group (p=0.02). Kaplan-Meier survival analysis showed that CABG was associated with better MACCE-free survival (p log-rank=0.03) than medical management. These results were confirmed on univariate Cox regression, but not multivariate regression (OR 0.773 (0.526 to 1.136); p=0.19). Both Kaplan-Meier survival and regression analyses showed that dual antiplatelet therapy (DAPT) and anticoagulation were not associated with significant improvement in MACCE rates.ConclusionOur analysis showed similar long-term MACCE risks in patients with CAA undergoing medical, percutaneous and surgical management. Further, DAPT and anticoagulation were not associated with significant benefits in terms of MACCE rates. These results should be interpreted with caution considering the small size and potential for selection bias and should be confirmed in large, randomised trials.

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Cumulative incidence and risk factors for radiation induced leukoencephalopathy in high grade glioma long term survivors

Scientific Reports ◽

10.1038/s41598-021-89216-1 ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Robert Terziev ◽

Dimitri Psimaras ◽

Yannick Marie ◽

Loic Feuvret ◽

Giulia Berzero ◽

...

Keyword(s):

Risk Factors ◽

Cumulative Incidence ◽

High Grade Glioma ◽

High Grade ◽

Nucleotide Polymorphisms ◽

Increased Risk ◽

Radiation Induced ◽

Long Term Survivors

AbstractThe incidence and risk factors associated with radiation-induced leukoencephalopathy (RIL) in long-term survivors of high-grade glioma (HGG) are still poorly investigated. We performed a retrospective research in our institutional database for patients with supratentorial HGG treated with focal radiotherapy, having a progression-free overall survival > 30 months and available germline DNA. We reviewed MRI scans for signs of leukoencephalopathy on T2/FLAIR sequences, and medical records for information on cerebrovascular risk factors and neurological symptoms. We investigated a panel of candidate single nucleotide polymorphisms (SNPs) to assess genetic risk. Eighty-one HGG patients (18 grade IV and 63 grade III, 50M/31F) were included in the study. The median age at the time of radiotherapy was 48 years old (range 18–69). The median follow-up after the completion of radiotherapy was 79 months. A total of 44 patients (44/81, 54.3%) developed RIL during follow-up. Twenty-nine of the 44 patients developed consistent symptoms such as subcortical dementia (n = 28), gait disturbances (n = 12), and urinary incontinence (n = 9). The cumulative incidence of RIL was 21% at 12 months, 42% at 36 months, and 48% at 60 months. Age > 60 years, smoking, and the germline SNP rs2120825 (PPARg locus) were associated with an increased risk of RIL. Our study identified potential risk factors for the development of RIL (age, smoking, and the germline SNP rs2120825) and established the rationale for testing PPARg agonists in the prevention and management of late-delayed radiation-induced neurotoxicity.

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Correctable biliary atresia and cholangiocarcinoma: a case report of a 63-year-old patient

Surgical Case Reports ◽

10.1186/s40792-019-0748-9 ◽

2019 ◽

Vol 5 (1) ◽

Cited By ~ 1

Author(s):

Masaki Nio ◽

Motoshi Wada ◽

Hideyuki Sasaki ◽

Hiromu Tanaka ◽

Masatoshi Hashimoto ◽

...

Keyword(s):

Biliary Atresia ◽

Liver Tumors ◽

Malignant Tumors ◽

Porta Hepatis ◽

Case Presentation ◽

Cancer Occurrence ◽

Long Term Survivors ◽

Near Future

Abstract Background Although cancer occurrence following surgery for biliary atresia has gradually increased, the development of cholangiocarcinoma in a native liver survivor of biliary atresia is extremely rare. Case presentation A 3-month-old female patient with the correctable type of biliary atresia underwent a cystoduodenostomy. At 16 years of age, she underwent multiple surgeries including lysis of intestinal adhesions, ileostomy, and gastrojejunostomy at another hospital. At 54 years of age, she underwent lithotomy at the porta hepatis, resection of the residual cystic bile duct with gallbladder, and hepaticojejunostomy in Roux-en-Y fashion. As she approached the age of 63, her computed tomography scan showed no liver tumors. In the following year, she developed cholangiocarcinoma at the porta hepatis and underwent chemotherapy. However, the cancer progressed, and she died before she reached the age of 64 years. Conclusions Cholangiocarcinoma is extremely rare in patients with biliary atresia. However, physicians should follow up patients with biliary atresia as closely as possible, as malignant tumors secondary to biliary atresia may increase in number in the near future because of the growing number of long-term survivors with biliary atresia.

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Atrial fibrillation associated with increased risk of venous thromboembolism

Thrombosis and Haemostasis ◽

10.1160/th14-05-0405 ◽

2015 ◽

Vol 113 (01) ◽

pp. 185-192 ◽

Cited By ~ 23

Author(s):

Chun-Cheng Wang ◽

Cheng-Li Lin ◽

Guei-Jane Wang ◽

Chiz-Tzung Chang ◽

Fung-Chang Sung ◽

...

Keyword(s):

Atrial Fibrillation ◽

Venous Thromboembolism ◽

Incidence Rates ◽

Vein Thrombosis ◽

Kaplan Meier ◽

Increased Risk ◽

Long Term Follow Up ◽

Deep Vein

SummaryWhether atrial fibrillation (AF) is associated with an increased risk of venous thromboembolism (VTE) remains controversial. From Longitudinal Health Insurance Database 2000 (LHID2000), we identified 11,458 patients newly diagnosed with AF. The comparison group comprised 45,637 patients without AF. Both cohorts were followed up to measure the incidence of deep-vein thrombosis (DVT) and pulmonary embolism (PE). Univariable and multivariable competing-risks regression model and Kaplan-Meier analyses with the use of Aelon-Johansen estimator were used to measure the differences of cumulative incidences of DVT and PE, respectively. The overall incidence rates (per 1,000 person-years) of DVT and PE between the AF group and non-AF groups were 2.69 vs 1.12 (crude hazard ratio [HR] = 1.92; 95 % confidence interval [CI] = 1.54-2.39), 1.55 vs 0.46 (crude HR = 2.68; 95 % CI = 1.97-3.64), respectively. The baseline demographics indicated that the members of the AF group demonstrated a significantly older age and higher proportions of comorbidities than non-AF group. After adjusting for age, sex, and comorbidities, the risks of DVT and PE remained significantly elevated in the AF group compared with the non-AF group (adjusted HR = 1.74; 95 %CI = 1.36-2.24, adjusted HR = 2.18; 95 %CI = 1.51-3.15, respectively). The Kaplan-Meier curve with the use of Aelon-Johansen estimator indicated that the cumulative incidences of DVT and PE were both more significantly elevated in the AF group than in the non-AF group after a long-term follow-up period (p<0.01). In conclusion, the presence of AF is associated with increased risk of VTE after a long-term follow-up period.

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Clinical and Biological Characteristics and Outcomes of Richter Transformation : A French Multicenter Study from the Filo Group

Blood ◽

10.1182/blood-2019-128053 ◽

2019 ◽

Vol 134 (Supplement_1) ◽

pp. 4112-4112

Author(s):

Charline Moulin ◽

Romain Morizot ◽

Thomas Remen ◽

Hélène Augé ◽

Florian Bouclet ◽

...

Keyword(s):

Board Of Directors ◽

Research Funding ◽

Intermediate Risk ◽

Line Treatment ◽

First Line ◽

Advisory Committees ◽

Long Term Survivors ◽

First Line Treatment

Introduction: About 2 to 10% of patients (pts) diagnosed with Chronic Lymphocytic Leukemia (CLL) develop diffuse large B-cell lymphoma (DLBCL, so-called Richter transformation (RT)) over long-term follow-up. The outcomes of pts with RT are variable and poorly understood and there is no consensus on the best therapeutic approach. The aim of this study was to analyze the clinical characteristics, outcomes and factors predictive of survival in a large series of RT from the French Innovative Leukemia Organization (FILO). Methods: Biopsy-confirmed RT (limited to DLBCL and excluding Hodgkin lymphoma) diagnosed from 2001 to 2018 were identified from eight FILO centers. Clinical and biological characteristics of CLL and RT at diagnosis, including cytogenetics, clonal relation with the pre-existing CLL, Epstein-Barr virus (EBV) status, cell of origin (COO) analyzed by immunohistochemistry and RT score (Tsimberidou AM et al, J Clin Oncol, 2006) were analyzed as well as treatment and outcomes. Overall survivals (OS) were defined as time from CLL and RT diagnosis to death from any cause and analyzed using the Kaplan-Meier method. Statistical analyses were performed with SAS version 9.4. Results: A total of 70 CLL pts who developed RT were identified. The median age at CLL diagnosis was 62 years old (range 35-82), and 50 (71.4 %) were male. The median time to transformation was 5.5 years (range 0 to 22 years), with 12 simultaneous diagnosis of CLL and RT. Prior to RT, 20 (29%) pts had not been treated for CLL, 50 received one (n=21) or more (n= 29) line of treatment ; 6 pts had received a novel agent (ibrutinib, idelalisib or venetoclax). The median age at RT diagnosis was 68 years old (range 42-88). All biopsies were centrally reviewed; 38/58 pts (66%) had elevated LDH (>1.5N) ; 35/65 pts (54 %) had bulky disease (≥ 5 cm); 10/54 (18.5%) pts had del(17p) or TP53 mutation ; 9/42 pts (21%) had a complex karyotype (at least 3 abnormalities). The CLL and RT were clonally related in 27/27 (100%) tested pts. COO by Hans algorithm was non germinal center B cell-like (GCB) in 26/28 pts (93%). EBV was positive or detected in 5/40 (12.5%) pts. The median of Ki67 positivity was 70% (range 30% to 100%). The RT score (based at RT diagnosis on ECOG performance status 2-4, LDH >1.5 x normal, platelets<100 x 109/L, tumor size >5 cm and >1 prior therapy for CLL) was : low risk in 17 pts (31%), low-intermediate risk in 10 pts (19%), high-intermediate risk in 14 pts (25%) and high risk in 14 pts (25%). The most common first-line treatment of RT was immunochemotherapy (n=57, 87%) including R-CHOP-like regimen (n=48, 73%). Autologous or allogeneic transplantation was performed for 7 pts (11%). Response to first-line treatment was complete or partial response in 26 pts (40%), and stable disease or progression in 39 pts (60%). After a median follow-up of 8 years, 51/64 pts (80%) have died. The main causes of death were progressive DLBCL (n=36, 71%), infection (n=8, 16%) or progressive CLL (n=2, 4%). The median OS of the cohort from CLL and RT diagnosis (Figure 1) were 7.8 years and 9.5 months, respectively. In univariate analysis, patients with TP53 disruption at CLL stage, low platelets count, elevated LDH, elevated beta2-microglobulin, high ECOG score, high RT score, EBV positivity and absence of response to first-line RT treatment had worse OS. The ECOG score, platelets count and TP53 disruption remain significant in multivariate Cox-regression. Last, we compared the clinical and biological parameters of two Richter groups defined as: (i) short-term survivors (<12 months, n = 34) and (ii) long-term survivors (>48 months, n = 18). Long survival was significantly associated with elevated platelets count, low LDH, low ECOG, low RT score and response to RT first-line treatment. Discussion: The clinical outcomes of RT patients is poor and novel treatment options are needed. However, a group of long-term survivors was identified, characterized by elevated platelets count, low LDH, low ECOG, low RT score and response to immunochemotherapy. Disclosures Leblond: Astra Zeneca: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Gilead: Honoraria, Speakers Bureau; Roche: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Janssen: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Abbvie: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Speakers Bureau; Amgen: Honoraria, Speakers Bureau. Thieblemont:Roche: Honoraria, Research Funding; Gilead: Honoraria; Novartis: Honoraria; Kyte: Honoraria; Janssen: Honoraria; Celgene: Honoraria; Cellectis: Membership on an entity's Board of Directors or advisory committees. Cymbalista:Janssen: Honoraria; Gilead: Honoraria; AstraZeneca: Honoraria; Sunesis: Research Funding; Roche: Research Funding; Abbvie: Honoraria. Guièze:Abbvie: Honoraria; Janssen: Honoraria; Gilead: Honoraria; Roche: Honoraria. Broseus:Janssen: Honoraria; Gilead: Honoraria; Novartis: Research Funding. Feugier:gilead: Honoraria, Research Funding, Speakers Bureau; janssen: Honoraria, Research Funding, Speakers Bureau; abbvie: Honoraria, Research Funding, Speakers Bureau; roche: Honoraria, Research Funding, Speakers Bureau.

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Long-term outcomes of transvaginal mesh surgery for pelvic organ prolapse: a retrospective cohort study

BMC Women s Health ◽

10.1186/s12905-021-01505-z ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Xiaojuan Wang ◽

Yisong Chen ◽

Changdong Hu ◽

Keqin Hua

Keyword(s):

Pelvic Organ Prolapse ◽

Patient Characteristics ◽

Pelvic Organ ◽

Transvaginal Mesh ◽

Recurrence Rates ◽

Kaplan Meier ◽

Mesh Surgery ◽

Patient Global Impression

Abstract Background The objective of this study was to evaluate the overall outcomes and complications of transvaginal mesh (TVM) placement for the management of pelvic organ prolapse (POP) with different meshes with a greater than 10-years of follow-up. Methods We performed a retrospective review of patients with POP who underwent prolapse repair surgery with placement of transvaginal mesh (Prolift kit or self-cut Gynemesh) between January 2005 and December 2010. Baseline of patient characteristics were collected from the patients’ medical records. During follow-up, the anatomical outcomes were evaluated using the POP Quantification system, and the Patient Global Impression of Improvement (PGI-I) was used to assess the response of a condition to therapy. Overall postoperative satisfaction was assessed by the following question: “What is your overall postoperative satisfaction, on a scale from 0 to 10?”. Relapse-free survival was analyzed using Kaplan–Meier curves. Results In total, 134 patients were included. With a median 12-year (range 10–15) follow-up, 52 patients (38.8%) underwent TVM surgery with Prolift, and Gynemesh was used 82 (61.2%). 91% patients felt that POP symptom improved based on the PGI-I scores, and most satisfied after operation. The recurrence rates of anterior, apical and posterior compartment prolapse were 5.2%, 5.2%, and 2.2%, respectively. No significant differences in POP recurrence, mesh-associated complications and urinary incontinence were noted between TVM surgery with Prolift versus Gynemesh. Conclusions Treatment of POP by TVM surgery exhibited long-term effectiveness with acceptable morbidity. The outcomes of the mesh kit were the same as those for self-cutmesh.

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