scholarly journals P1693LONG-TERM OUTCOME OF KIDNEY TRANSPLANTATION IN PLASMA CELLS DYSCRASIAS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Fulvia Zappulo ◽  
Gabriele Donati ◽  
Giorgia Comai ◽  
Claudia Bini ◽  
Andrea Angeletti ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Light Chain ◽  
Graft Failure ◽  
Case Series ◽  
University Hospital ◽  
High Dose ◽  
Light Chain Deposition Disease ◽  
Light Chain Amyloidosis ◽  
First Case

Abstract Background and Aims Survival of patients with Multiple Myeloma (MM), Light Chain Amyloidosis (LCA) and Monoclonal Gammopathies of renal significance (MGRS) on chronic renal replacement therapy (RRT) is poor. The gold standand treatment of plasma cell dyscrasias (PCD) is high-dose chemotherapy followed by Autologous Stem Cell Transplantation (ASCT) which can induce complete remission and longer survival than chemotherapy alone. Kidney transplantation (KT) after ASCT could represent an option for patients with PCD and End Stage Renal Disease (ESRD). There is no evidence about the time of follow up required from MM remission and KT. Method We present a case series of 5 patients who underwent KT after ASCT and remission of MM among 2,500 transplant recipients followed at the Nephrology Dialysis and Renal Transplantation Unit of S.Orsola University Hospital from 1967 untill now. As in case of recovery from solid cancers, the feasibility of KT after MM was considered when no signs of relapse were assessed. In our cohort 3 patients were affected by Light Chain Deposition Disease (LCDD), 1 patient presented Myeloma Cast Nephropathy (MCN) and one patient Light Chain Amyloidosis (LCA). They all required RRT and underwent KT after ASCT. Results Time between ASCT and KT ranged from 3 and 11 years and clinical outcome was very different. The mean follow up period ranged from 2 to 4 years. In the first case (LCDD) KT was performed 11 years after ASCT, the graft failure occurred 6 years later because of chronic allograft nephropathy requiring RRT. In the second case (LCDD) patient received KT 3 years after ASCT. He developed Bence-Jones proteinuria requiring specific therapy with Dexametasone and Bortezomib determining progressive graft failure. In the third case (LCDD) KT was performed 4 years after ASCT and the 4 year follow up is negative for relapse of MM or ESRD. The fourth patient presented MCN and received KT 8 years after ASCT. MCN relapsed 6 years later; it caused ESRD requiring RRT. In the last patient (LCA) KT was performed 4 years after ASCT. No recurrence occurred in a 2-year follow up. Conclusion MM is the most frequent malignancy in dialytic population; the need for KT in MM remains high. ASCT improves the quality of life and offers higher survival in patients with myeloma/MGRS/amyloidosis-related ESRD. Therefore the combination of chemotherapy/ASCT and KT is pivotal to pursue renal restoring. Since high risk of recurrence larger study are required to clarify the better follow up period after MM remission and KT.

Treatment of Light Chain (AL) Amyloidosis and Light Chain Deposition Disease (LCDD) with the Combination of Bortezomib and Dexamethasone.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 191-191 ◽  
Author(s):  
Efstathios Kastritis ◽  
Athanasios Anagnostopoulos ◽  
Maria Roussou ◽  
Savvas Toumanidis ◽  
Constantinos Pamboukas ◽  
...  
Keyword(s):  
Median Time ◽  
Light Chain ◽  
Dose Adjustment ◽  
High Dose ◽  
Al Amyloidosis ◽  
Light Chain Deposition Disease ◽  
Hematologic Response ◽  
Organ Response ◽  
Light Chain Deposition

Abstract Background: Primary (AL) amyloidosis and light chain deposition disease (LCDD) are clonal plasma cells disorders characterized by deposition of either amyloid fibrils (in AL) or amorphous nodular non-congophilic deposits (in LCDD) derived from abnormal light chains, that cause failure of affected organs. Treatment of AL is based on steroids and standard dose or high dose melphalan with ASCT. Data on treatment of LCDD are limited. Bortezomib, a proteasome inhibitor, has significant activity in myeloma, which is enhanced by the addition of dexamethasone (BD) and can be given in myeloma patients with renal impairment. We evaluated this combination in patients with AL and LCDD. Methods: We treated consecutive patients with AL or LCDD with the combination of Bortezomib (1.3 mg/m2 days 1, 4, 8 and 11) and Dexamethasone (40 mg days 1–4), every 21 days, for up to 6 cycles. Dose modifications were made based on toxicity. Organ involvement and hematologic and organ response were assessed following standard criteria (Gertz et al, Am J Hematol 2005). Results: Since September 2005, 21 consecutive AL and 2 LCDD patients started treatment with BD. Eight patients (38%) had at least one prior therapy and 13 (62%) had ≥2 organs involved; kidneys and heart were affected in most patients. The majority had impaired performance status, high BNP values and 7 (33%) patients had creatinine>2 mg/dl. Among the 21 AL patients, 2 are early for evaluation, 4 had non-measurable disease and 15 patients are evaluable: 13 (87%) responded (CR+PR) and 7 (47%) achieved hematologic CR. All 5 patients refractory to high dose DEXA had a hematologic response and 3 had a CR. Two of 3 AL patients with abnormal FLC ratio but involved FLC<100 mg/L achieved normal FLC ratio. Both patients with LCDD responded- the patient who was refractory to VAD had a CR. Median time to hematologic response was 0.93 months; all responses occurred within 2 courses while all patients in hematologic CR maintain CR for a median of 10.5 months (range 4.6–21). So far, 6 (28%) AL patients had organ responses (3 renal and 3 cardiac) while 7 (44%) patients had a sustained >50% reduction in BNP. Median time to organ response in AL patients was 4 months (range 2–8). In both LCDD patients albuminuria was reduced by more than 50%. Median follow-up for all patients and for living patients is 9.5 months (range 1–23) and 12 months (range 4–23) respectively. In an intention to treat basis 8 (38%) patients have progressed, including 3 AL patients (14%) who died while on treatment (with two of them at hematologic PR at the time of their death- one died before she was assessable for response). Five AL patients had either hematologic or organ progression at a median of 6.8 months after treatment initiation. Peripheral neuropathy, fatigue, peripheral edema, constipation and exacerbation of postural hypotension were managed with appropriate dose adjustment; however 10 (47%) patients were not able to receive the planned 6 courses. Conclusions: The combination of BD is feasible for patients with AL amyloidosis and LCDD. Most patients achieve rapid hematologic response and toxicity can be managed with close follow-up and appropriate dose adjustment. This treatment may be a valid option for patients with severe heart or kidney impairment who are not candidates for other therapies.

High Dose Therapy Improves Survival in Systemic Light Chain Amyloidosis: 14 Year Follow up

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 750-750
Author(s):  
Simrit Parmar ◽  
Joshua Howell ◽  
Michael Wang ◽  
Mubeen A Khan ◽  
Qaiser Bashir ◽  
...  
Keyword(s):  
Light Chain ◽  
Troponin I ◽  
Induction Treatment ◽  
High Dose ◽  
Primary Amyloidosis ◽  
Cell Transplant ◽  
Term Survival ◽  
Light Chain Amyloidosis ◽  
Number Of Patients

Abstract Abstract 750 Background: Treatment remains a challenge for systemic light chain amyloidosis (AL). Autologous stem cell transplant (AutoSCT) has been associated with long term survival. However, a recent multicenter randomized study failed to show survival benefit for AutoSCT perhaps due to high non-relapse mortality (NRM). Here we present a comparison of AutoSCT to other conventional therapies in AL patients treated at our institution with a 14-year follow up. Methods: A total of 2018 cases were identified upon pathology review from 1998–2012. AL was confirmed in 264 patients; primary amyloidosis (PA) in 147 pts and multiple myeloma with amyloidosis (AM) in 110 patients; solitary amyloidoma in 7 patients. AutoSCT was performed in 126 patients (PA=79 and AM=47). Results: The day 100 NRM was 5% and 1-year NRM was 8%. With a follow up of 14 years in surviving patients, the 10-year overall survival (OS) of AL patients was significantly better in those undergoing AutoSCT (41% vs. 17%; p<0.0001; figure 1). Involvement of more than one organ (6-yr OS 36% vs. 55%; p=0.04) and cardiac involvement (2-yr OS of 57% vs. 78%; p=0.01) were associated with poor outcome. In the patients undergoing AutoSCT: PA vs. AM, Mayo staging, Boston University (BU) staging or bone marrow plasma cells >10 % at the time of autoSCT did not have an impact on OS. Cardiac biomarkers including NT-ProBNP and Troponin-I and T levels were available in a limited number of patients and were not analyzed for survival outcomes. In multivariate analysis, superior OS was associated with: age <60yrs (HR 2.1, p=0.022); and induction treatment before AutoSCT (HR 2.7, p=0.02). Involvement of kidney as the only end organ showed a trend toward improved survival (HR 1.6, p=0.06) (Table 1). Specifically for PA patients (n=79); treatment before autoSCT was associated with improved 3-yr OS: 85% vs. 66%; p=0.02. Conclusions: AL patients should be evaluated for AutoSCT and selected patients should undergo induction therapy to decrease amyloid burden prior to AutoSCT. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Exclusive endoscopic transcanal transpromontorial approach: a new perspective for internal auditory canal vestibular schwannoma treatment

2017 ◽  
Vol 126 (1) ◽  
pp. 98-105 ◽  
Author(s):  
Daniele Marchioni ◽  
Matteo Alicandri-Ciufelli ◽  
Alessia Rubini ◽  
Babara Masotto ◽  
Giacomo Pavesi ◽  
...  
Keyword(s):  
Skull Base ◽  
Tertiary Care ◽  
Internal Auditory Canal ◽  
Case Series ◽  
University Hospital ◽  
Cerebrospinal Fluid Leakage ◽  
Complication Rates ◽  
First Case ◽  
The University

OBJECTIVE The aim of this study was to describe the first case series in which an exclusive endoscopic transcanal transpromontorial approach (EETTA) was used to treat small vestibular schwannomas (VSs) and meningiomas of the internal auditory canal (IAC). METHODS The authors performed a retrospective review of patients who had undergone surgery using an EETTA to the IAC at 2 university tertiary care referral centers during the period from November 2011 to January 2015. RESULTS Ten patients underwent surgery via an EETTA for the treatment of VS in the IAC at the University Hospital of Modena or the University Hospital of Verona. The patients had Koos Grade I or II tumors and American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) Class D hearing status preoperatively. Gross-total resection was achieved in all patients. No major complications such as cerebrospinal fluid leakage or hemorrhage were reported. In 7 of 10 (70%) patients, facial nerve function was normal immediately after surgery (Rough Grading System [RGS] Grade I). Two patients presented with a transitory facial palsy immediately after surgery (RGS Grade II–III) but experienced complete recovery during the follow-up period. The mean follow-up was 10 months. CONCLUSIONS The EETTA proved to be successful for the removal of VS or meningioma involving the cochlea, fundus, and IAC, with possible lower complication rates and less invasive procedures than those for traditional microscopic approaches. The potential for the extensive and routine use of this approach in lateral and posterior skull base surgery will depend on the development of technology and surgical refinements and on the diffusion of skull base endoscopic skills among the otolaryngological and neurosurgical communities.

scholarly journals Shortened Immunosuppression Following Peripheral Blood (PB) Haploidentical (haplo) Transplantation with Post-Transplant Cyclophosphamide (PTCy) Is Associated with Tolerable Rates of Graft-Vs-Host Disease (GVHD)

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 3320-3320
Author(s):  
Amy E. DeZern ◽  
Marianna Zahurak ◽  
Javier Bolanos-Meade ◽  
Richard J. Jones
Keyword(s):  
Graft Failure ◽  
Acute Gvhd ◽  
Risk Index ◽  
Chronic Gvhd ◽  
Prospective Trial ◽  
Stopping Rules ◽  
High Dose ◽  
Gvhd Prophylaxis ◽  
Grade 3

With PTCy as GVHD prophylaxis, nonmyeloablative (NMA) HLA- haplo and HLA-matched blood or marrow (BMT) have comparable outcomes. Previous reports showed that discontinuation of immunosuppression (IST) as early as day 60 after infusion of bone marrow (BM) haplo allograft with PTCy is feasible. However, there are certain diseases in which PB may be favored over BM grafts to augment engraftment rates; however, given the higher rates of GVHD with PB, excessive GVHD becomes a concern with early discontinuation of IST. We present a completed, prospective single-center trial of stopping IST at days 90 and 60 after NMA haplo PB. (NCT02556931) From 12/2015-7/2018, 117 evaluable patients (pts) with hematologic malignancies associated with higher rates of graft failure with PTCy (MDS, MPN, overlap syndromes, 2o AML, AML with MRD, MM, and CLL) received NMA PB allografts on trial. Haplo donors were preferred, but in patients lacking suitable haplo relatives, unrelated donors were employed with 6 in each IST cohort. The primary objective was to evaluate the safety and feasibility of reduced‐duration IST (from Day 5 through Day 90 in cohort 1 and through Day 60 in cohort 2.) Transplant inclusion criteria were standard and the conditioning included Cy (14.5 mg/kg IV D -6 and -5), fludarabine (D -6 to -2), TBI (200 cGy D -1) and T-cell replete PB. GVHD prophylaxis consisted of high-dose PTCy (50 mg/kg IV D 3 and 4), mycophenolate mofetil (D 5-35) and IST (tacrolimus/sirolimus) from D 5 forward. Priot to transplantation, pts were assigned to stop IST early if eligible, as defined by having ≥ 5% donor T cells at ~D 56 onward, no relapse, and no grade 2-4 acute or significant chronic GVHD. If ineligible to discontinue IST early, it continued through D 180. Monitoring rules declared reduced IST feasible if ≥ 33% of pts stopped IST early as planned. Safety stopping rules for early IST cessation were based on ≥ 5% graft failure, ≥ 5% NRM, ≥ 50% relapse, and ≥ 10% combined grade 3-4 acute GVHD and severe chronic GVHD, measured from the IST stop date to ~D 180. Historical data from 55 haplo transplants for MDS, CLL, and MPNs at our center using the same regimen and PB grafts informed safety calculations. Of the 117 pts (median age 64 years, range 24-78), the most common diagnoses were MDS (33%), AML (with MRD or arising from antecedent disorder) (31%), MPNs (21%) myeloma (10%), and CLL (6%). By refined Disease Risk Index, 13% were low risk, 69% intermediate and 18% high. Shortened IST was feasible in 75 pts (64%) overall. Ineligibility for shortened IST was due most commonly to GVHD (17 pts), followed by early relapse (11 pts), NRM (7 pts), patient/ physician preference (4 pts) or graft failure (3 pts). Of the 57 patients in the D90 cohort (median follow up 35 mos), 33 (58%) stopped IST early as planned. Of the 60 patients in the D60 cohort (median follow up 20 mos), 42 (70%) stopped IST early as planned. The graft failure rate was 2.6%. NRM was very similar in the two arms, 12% at both 12 and 18 months in the D90 cohort and 10% and 13% at 12 and 18 months in the D60 cohort. Relapse in D90 cohort is 40% at 18 months compared to 33% at 18 months in the D60 cohort. Figure 1 shows cumulative incidence (CI) of acute grade 2-4 and grade 3-4 GVHD. Although the CI of grade 1-2 GVHD may be slightly higher in day 60 cohort, it is only 40% at D180. Severe chronic GVHD was 12% (D90) and 11% (D60) at 540 days. One year OS is 75% and 78% for the D90 and D60 cohorts, respectively. At 12 months PFS is 54% in the D90 group and 67% in the D60. At 12 months, the GRFS is 33% in the D90 group, and 38% in the D60 group. (Figure 2) These data suggest that reduced-duration IST in pts receiving NMA haplo PB with PTCy is feasible and carries an acceptable safety profile. Risks of acute GVHD, chronic GVHD, graft failure and NRM appear similar to historical outcomes with IST until D180 and between the two cohorts. When comparing the D90 and D60 arms, grade 3-4, severe chronic GVHD, GRFS, OS and PFS were similar. Although a larger, prospective trial would be needed to uncover potential small differences in outcomes based on IST duration, these data show that similar to our findings with BM, many PB pts (64% in this trial) can discontinue IST as early as D60 without undue toxicity. The favorable toxicity profile of the PTCy platform, coupled with the feasibility and safety of early IST cessation, provides an ideal setting to incorporate novel post-transplantation approaches for relapse reduction. Figure 1 Disclosures DeZern: Astex Pharmaceuticals, Inc.: Consultancy; Celgene: Consultancy. Bolanos-Meade:Incyte Corporation: Other: DSMB fees.

scholarly journals Investigation of SARS-CoV-2 RNA in Milk Produced by Women with COVID-19 and Follow-Up of Their Infants: A Preliminary Study

2021 ◽  
Author(s):  
TALAT KILIC ◽  
Sebnem Kilic ◽  
Nurcan Kirici Berber ◽  
Ayten Gunduz ◽  
Yasemin Ersoy
Keyword(s):  
Human Milk ◽  
Prospective Observational Study ◽  
Throat Swab ◽  
Case Series ◽  
The Other ◽  
Breastfed Infants ◽  
Milk Samples ◽  
First Case ◽  
Preliminary Study

Objectives: Studies have shown that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is primarily transmitted from person to person via airborne droplets. It is unclear whether it can be shed into human milk and transmitted to a child via breastfeeding.We investigated the presence of SARS-CoV-2 RNA in human milk samples of 15 mothers with coronavirus disease 19(COVID-19) and in the throat swab samples of their infants. Methods: This is a prospective observational study in which breast milk samples were collected from 15 mothers with COVID-19. The presence of SARS-CoV-2 RNA in the whole human milk samples of the patients was investigated using RT-qPCR. All of the infants underwent a clinical follow-up during their 14-day isolation and their throat swab samples were tested for SARS-CoV-2 RNA. Results: Of 15 mothers with COVID-19, SARS-CoV-2 RNA was detected in milk samples from 4 mothers. The throat swab samples from these mothers’ infants were found to be positive for SARS-CoV-2 RNA. Three of the four mothers were breastfeeding. In addition, during the 14-day isolation, all but three of the mothers breastfed their infants. Of the 12 breastfed infants, while the test for SARS-CoV-2 RNA in throat swab samples was negative in six of the infants, the other six infants, who had mild COVID-19 symptoms, tested positive for SARS-CoV-2 RNA.Clinical outcomes of all mothers and infants were uneventful. Conclusion: To our knowledge, this is the first case series with the largest number of cases with SARS-CoV-2 RNA positivity in human milk samples of mothers with COVID-19. However, we believe that the benefits of breastfeeding may outweigh the risk of SARS-CoV-2 infection in infants

Frequency of Success in Surgery of Vesicovaginal Fistula by Layered Closure with Graft Repair

2021 ◽  
Vol 15 (10) ◽  
pp. 3479-3481
Author(s):  
Anila Mujadid Qureshi ◽  
Azra Parveen Rajpar ◽  
Ishrat Saba Mari ◽  
Khalida Avesi ◽  
Kousar Fatima ◽  
...  
Keyword(s):  
Success Rate ◽  
Vesicovaginal Fistula ◽  
Case Series ◽  
University Hospital ◽  
Blue Dye ◽  
Urogenital Fistula ◽  
Vesicovaginal Fistulae ◽  
University Setting ◽  
Distressing Condition

Introduction: Vesicovaginal fistulae is abnormal communication between bladder and vagina that cause continous dribling of urine. It is physically, mentally and socially distressing condition. There are various approaches for surgeries of these urogenital fistulae with different success-rate that depend upon the experience of surgeon and surgical procedures. This study can help us to estimate the success rate of layered repair with graft in vaginal route to make stragedy to adopted in severe patient. Objective: To determine frequency of success in surgery on vesic-ovaginal fistulae by layered closured with graft repair procedures among patients admitted in Isra University. Setting: Obstetrics & Gynecology department in Isra university hospital Duration: 6 months from 10.2.2014 to 10.8.2014 Study Design: Case series Subject and methods: A total of 100 patients after having surgery for vesicovaginal fistula by layered closure with graft repair was included in this study. History and examination of all subjects were taken. The follow up visit was planned after 3 weeks of surgery. All women was questioned for recurrence of continuous urinary leakage and that without such symptoms proved by absence of leakage on methylene blue dye test was labeled as ‘success’. Results: - Frequency of success in surgery on vesic-ovaginal fistulae by layered closured with graft repair procedures was observed in 88% cases. Conclusion: The success rate of VVF repair by layered closured with graft repair procedures is high. It is concluded that obstetric urogenital fistula is a preventable condition. Keywords: Vesicovaginal fistulae, Layered closured, Graft repair, urogenital fistula

scholarly journals Nephrogenic Adenoma of the Urinary Bladder after Kidney Transplantation: Long-Term Follow-Up

2020 ◽  
Vol 2020 ◽  
pp. 1-3
Author(s):  
Iftikhar Khan ◽  
Mahmoud Obeid ◽  
Nasreen Hasan ◽  
Fayyad Jaradat ◽  
Bodhisatwa Sengupta ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Urinary Bladder ◽  
Histopathological Examination ◽  
Transitional Epithelium ◽  
Nephrogenic Adenoma ◽  
Term Outcome ◽  
Long Term Outcome ◽  
First Case

Nephrogenic adenoma is a rare lesion that consists of epithelial cells arranged in tubular form, resembling tubules in the renal medulla, and is found usually in the urinary bladder although it can occur anywhere in the transitional epithelium of the lower urinary tract. The first case of nephrogenic adenoma of the urinary bladder was reported before the first kidney transplantation, and the lesion has been reported in patients with and without renal transplantation. The origin of cells in nephrogenic adenoma is debated and has been postulated to arise from cells of embryonic origin or from metaplasia secondary to chronic irritation or from implantation of allograft cells in patients with kidney transplantation. The long-term outcome and potential to convert into malignancy are not established, and therefore, there are no recommendations on how to follow up these patients. We present a case of a patient who was found to have nephrogenic adenoma of the urinary bladder during his second kidney transplantation from a cadaveric donor. He had undergone living donor kidney transplantation previously which subsequently failed. The patient did not manifest any symptoms of nephrogenic adenoma. During a follow-up period of 5 years, he has not manifested any symptoms related to nephrogenic metaplasia. Histopathological examination 5 years after the second transplantation did not show any malignant change. It can be concluded that nephrogenic adenoma is likely to behave in benign fashion post kidney transplantation.

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