Lupus Nephritis in Children—A Review of 167 Patients

Background. Relatively few studies have been made of children with lupus nephritis. The prognosis of children with lupus nephritis is ominous for those with diffuse proliferative glomerulonephritis and active interstitial inflammation. Up to now few studies have been made on this subject. Objectives. To evaluate the clinical course, histopathology, and prognosis of lupus nephritis in children, to identify the risk factors for renal failure and mortality, and to share our experience in treating lupus nephritis in children. Methods. Retrospectively, 167 children under 18 years of age with lupus nephritis at Veterans General Hospital-Taipei, Taiwan from 1979 to 1991 were studied. All patients received renal biopsy and follow-up biopsies were performed in 36 children. The clinical and serologic parameters at the time of renal biopsy were recorded. Results. There were 55 (33%) patients with class II, 30 (18%) with class III, 69 (41.3%) with class IV, and 13 (7.8%) with class V nephritis based on initial biopsy. The mean follow-up time was 59 months. Follow-up biopsies were histologically stationary in 29 patients, progressive in five, and regressive in two. The results revealed that those with persistent hypertension, anemia, increased serum creatinine concentration, and decreased creatinine clearance rate at initial biopsy were more prone to develop renal failure. Low titer of CH50 hemolytic assay appeared to be a poor prognostic indicator. The overall renal and patient 5-year survival rates were 93.1% (135/145) and 91.08% (143/157), respectively. They were 87.7% (50/57) and 82% (55/67), respectively, of patients with class IV proliferative glomerulonephritis. Conclusions. The prognosis of children with class IV nephritis in this study was better than that reported previously. All children surviving without renal failure were maintaining their normal lives with little organ dysfunction. The improved results may be due to earlier renal biopsy for precise histopathologic definition, better supportive care, and selective use of aggressive therapy, including methylprednisolone pulse therapy, intravenous cyclophosphamide, intravenous prostaglandin E1 therapy, high-dose intravenous gammaglobulin therapy, and cyclosporin A for those with high risk factors.

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P0897RISK FACTORS FOR AVASCULAR BONE NECROSIS IN PATIENTS WITH LUPUS NEPHRITIS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0897 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Daniela Monova ◽

Simeon Monov ◽

Assen Kamenov ◽

Vladislava Milenova

Keyword(s):

Risk Factors ◽

Lupus Nephritis ◽

Avascular Necrosis ◽

Immunosuppressive Agents ◽

Individual Risk ◽

Control Group ◽

High Dose ◽

Increased Risk ◽

Avascular Necrosis Of Bone ◽

Class Iv

Abstract Background and Aims Avascular necrosis of bone (AVN) is an important complication of systemic lupus erythematosus (SLE) and often causes serious physical disability. The aim of this study was to investigate the risk factors for symptomatic avascular necrosis of bone (AVN) in lupus nephritis (LN) patients. Method The records of 374 patients (43 males, 331 females) with kidney biopsy-proven LN were reviewed retrospectively. Symptomatic AVN cases were defined as those with at least one diagnosis of AVN. The patients with LN who did not have AVN were evaluated as a control group. To determine risk factors for AVN, clinical, laboratory and therapeutic variables were analyzed by logistic regression. Results Symptomatic AVN was present in 17 patients (4 males, 13 females, mean age of 27,4±6,7 years). Among the 17 patients, 28 joints presented AVN. 12 occurred in hips (2 bilateral), 6-in ankles, 4-in knees, 3-in shoulders and 1- in lumbar spine. In 9 patients AVN involved 2 or more joints. 14 patients were on steroids at the time of presentation of AVN. 2 patients were not on CS and 1 patient did not has documentation of steroid use. Meta-analysis demonstrates a significant increased risk of AVN in patients with high disease activity and class IV LN (p<0,005). LN patients with AVN showed an earlier onset age (p<0,05) and received significantly higher total cumulative corticosteroid dose. AVN was not significantly associated with use of immunosuppressive agents. Serositis, coagulation disorders, vasculitis, cigarette smoking were higher incidence in male with LN and AVN. Raynaud‘s phenomenon, autoimmune thyroiditis, arthritis, Sjögren’s syndrome, IgM anticardiolipin antibodies, antiphospholipid syndrome, Cushingoid body habitus were higher incidence in female with LN and AVN. Conclusion Many risk factors have been involved in the development of AVN in LN patients. AVN is prevalent in class IV LN and in younger patients. Since asymptomatic osteonecrosis may remain undetected, its true prevalence could be much higher than we reported. Multifocal lesions involving more than three anatomical sites are unusual. Corticosteroids are the principal risk factor, although some cases of AVN occur in relatively steroid naïve patients. Early detection of AVN is important because the prognosis depends of the stage and location of the lesion. An individual risk assessment for AVN development should be made prior to and during treatment for LN, especially in patients high dose corticosteroids.

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P0278CARDIOVASCULAR RISK FACTORS AND THE LONG TERM OUTCOME OF LUPUS NEPHRITIS

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0278 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Marwa Omrane ◽

Raja Aoudia ◽

Mondher Ounissi ◽

Soumaya Chargui ◽

Mouna Jerbi ◽

...

Keyword(s):

Risk Factors ◽

Cardiovascular Disease ◽

Renal Failure ◽

Cardiovascular Risk ◽

Lupus Nephritis ◽

Cardiovascular Risk Factors ◽

Increased Risk ◽

Traditional Risk Factors

Abstract Background and Aims Systemic Lupus Erythematosus (SLE) is associated with an increased risk of cardiovascular morbidity and cardiovascular mortality. The risk of cardiovascular events is 1.3–2.7 times higher in SLE patients than in the general population, and even higher in patients with lupus nephritis (LN). Traditional risk factors as well as SLE-specific and treatment-related factors all contribute to the increased risk of cardiovascular disease. The primary aim of the present study was to evaluate cardiovascular risk factors, morbidity and mortality in patients with LN. Method This is a retrospective study of patients over the age of 16, with LN proved by kidney biopsy and followed up in our department over a period of 17 years. The diagnosis of lupus was made according to criteria of The American College of Rheumatology revised in 1997. Demographic, clinical and para-clinical data were collected from medical observations. Results We collected 155 women and 19 men with a sex ratio F / H of 8.2. The mean age at the time of the discovery of LN was 32.6 years [15-45 years]. Overall median follow-up time was 81.2 months. Renal symptomatology was dominated by proteinuria noted in all patients with an average proteinuria at 3.3 g / 24h, associated to a nephrotic syndrome in 68% of patients, hematuria was present in 69% of patients and renal failure was present in half of cases with an average serum creatinine of 110 µmol / l. At the time of diagnosis of LN, hypertension was noted in 48.9% of cases, diabetes in 2.8% of cases and obesity in 57.4% of cases with an index average body mass of 28.5 Kg / m2. Smoking was reported in 17.2% of the cases. The average cholesterol level was 5,5±2,1 mmol/l, the average triglycerid level was 2,5±1,1 mmol/. Antiphospholipid syndrome was found in 14.9% of cases. We performed 243 renal biopsies with 174 initial and 69 iterative biopsies. The histological lesions were polymorphic dominated by LN class IV (54.3%), arteriolosclerosis was observed in 47.7% and lesions of thrombotic microangiopathy in 29.8%. Corticosteroid therapy was prescribed in all patients combined with immunosuppressive therapy in 54.6% of cases. The overall survival of the patients at 10 years was 85%. During follow-up, cardiovascular complications found in our series were mainly strokes (6.3%) and coronary insufficiency (5.2%) and transient ischemic attack (6.9%). After a univariate analysis, the additional cardiovascular risk factors identified in our study were antiphospholipid syndrome (p = 0.01), renal failure (p = 0.01), long-term corticosteroid therapy (p = 0.009), the chronicity of the disease (evolution of lupus> 10 years) (p = 0.014), proliferative forms (p=0.001), arteriolosclerosis (p=0.0002) and lesions of thrombotic microangiopathy (p=0.018). Survival in patients without cardiovascular risk factors was better (96% vs 88%). Conclusion In conclusion, in addition to traditional risk factors SLE patients have several disease related risk factors that explain increase cardiovascular disease. A careful control for this risk factors is essential to continuously improve survival in SLE.

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Initial renal histology and early response predict outcomes of Brazilian lupus nephritis patients

Lupus ◽

10.1177/0961203319890681 ◽

2019 ◽

Vol 29 (1) ◽

pp. 83-91

Author(s):

G Vajgel ◽

C B L Oliveira ◽

D M N Costa ◽

M A G M Cavalcante ◽

L M Valente ◽

...

Keyword(s):

Kidney Disease ◽

Lupus Nephritis ◽

Confidence Interval ◽

Odds Ratio ◽

Estimated Glomerular Filtration Rate ◽

Interstitial Fibrosis ◽

End Stage ◽

Class Iv

Objective We analyzed baseline and follow-up characteristics related to poorer renal outcomes in a Brazilian cohort of admixture race patients with lupus nephritis. Methods Overall, 280 outpatients with a diagnosis of systemic lupus erythematosus and previous kidney biopsy of lupus nephritis were recruited from August 2015 to December 2018 and had baseline laboratory and histologic data retrospectively analyzed; patients were then followed-up and data were recorded. The main outcome measure was the estimated glomerular filtration rate at last follow-up. Secondary analyses assessed the impact of initial kidney histology and treatment in long-term kidney survival. Results Median duration of lupus nephritis was 60 months (interquartile range: 27–120); 40 (14.3%) patients presented progressive chronic kidney disease (estimated glomerular filtration rate <30 and ≥10 ml/min/1.73 m2) or end-stage kidney disease at last visit. Adjusted logistic regression analysis showed that class IV lupus nephritis (odds ratio 14.91; 95% confidence interval 1.77–125.99; p = 0.01) and interstitial fibrosis ≥25% at initial biopsy (odds ratio 5.87; 95% confidence interval 1.32–26.16; p = 0.02), lack of complete or partial response at 12 months (odds ratio 16.3; 95% confidence interval 3.74–71.43; p < 0.001), and a second renal flare (odds ratio 4.49; 95% confidence interval 1.10–18.44; p = 0.04) were predictors of progressive chronic kidney disease. In a Kaplan-Meier survival curve we found that class IV lupus nephritis and interstitial fibrosis ≥25% were significantly associated with end-stage kidney disease throughout follow-up (hazard ratio 2.96; 95% confidence interval 1.3–7.0; p = 0.036 and hazard ratio 4.96; 95% confidence interval 1.9–12.9; p < 0.0001, respectively). Conclusion In this large cohort of admixture race patients, class IV lupus nephritis and chronic interstitial damage at initial renal biopsy together with non-response after 1 year of therapy and relapse were associated with worse long-term renal outcomes.

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Stroke in hemodialysis patients randomized to different intravenous iron strategies: a prespecified analysis from the PIVOTAL trial

Kidney360 ◽

10.34067/kid.0004272021 ◽

2021 ◽

pp. 10.34067/KID.0004272021

Author(s):

Patrick B. Mark ◽

Pardeep S. Jhund ◽

Matthew R. Walters ◽

Mark C. Petrie ◽

Albert Power ◽

...

Keyword(s):

Risk Factors ◽

Stroke Risk ◽

Controlled Trial ◽

Intravenous Iron ◽

Hemodialysis Patients ◽

High Dose ◽

Pivotal Trial ◽

Increased Risk ◽

Iv Iron

Background: People with kidney failure treated with hemodialysis (HD) are at increased risk of stroke compared to similarly aged people with normal kidney function. One concern is that treatment of renal anemia might increase stroke risk. We studied risk factors for stroke in a prespecified secondary analysis of a randomized controlled trial of intravenous iron treatment strategies in HD. Methods: We analyzed data from the Proactive IV IrOn Therapy in HaemodiALysis Patients (PIVOTAL) trial focusing on variables associated with risk of stroke. The trial randomized 2,141 adults, who had started hemodialysis <12 months earlier and who were receiving an erythropoiesis-stimulating agent (ESA), to high-dose IV iron administered proactively or low-dose IV iron administered reactively in a 1:1 ratio. Possible stroke events were independently adjudicated. We performed analyses to identify variables associated with stroke during follow-up and assessed survival following stroke. Results: During a median 2.1 years follow-up, 69 (3.2%) patients experienced a first post randomization stroke. 57 (82.6%) were ischemic strokes and 12 (17.4%) hemorrhagic strokes. There were 34 post randomization strokes in the proactive arm and 35 in the reactive arm (hazard ratio (95% confidence interval): 0.90 (0.56, 1.44), p=0.66). In multivariable models, female gender, diabetes, history of prior stroke at baseline, higher baseline systolic blood pressure, lower serum albumin and higher C-reactive protein were independently associated with stroke events during follow up. Hemoglobin, total iron or ESA dose were not associated with risk of stroke. 58% of patients with a stroke event died during follow-up, compared to 23% without a stroke. Conclusions: In hemodialysis patients, stroke risk is broadly associated with risk factors previously described to increase cardiovascular risk in this population. Proactive intravenous iron does not increase stroke risk.

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P0418LUPUS NEPHRITIS: A MONOCENTRIC STUDY

Nephrology Dialysis Transplantation ◽

10.1093/ndt/gfaa142.p0418 ◽

2020 ◽

Vol 35 (Supplement_3) ◽

Author(s):

Marwa Omrane ◽

Raja Aoudia ◽

Mondher Ounissi ◽

Soumaya Chargui ◽

Mouna Jerbi ◽

...

Keyword(s):

Systemic Lupus Erythematosus ◽

Renal Failure ◽

Lupus Nephritis ◽

Renal Biopsy ◽

Predictive Factors ◽

Lupus Erythematosus ◽

Renal Outcome ◽

Sustained Remission ◽

Systemic Lupus ◽

Therapeutic Protocols

Abstract Background and Aims Systemic lupus erythematosus is a multi-visceral autoimmune disease. Renal involvement is one of the most common and serious manifestations of this disease. The histological lesions are highly polymorphic and the renal biopsy remains crucial for the therapeutic management of lupus nephritis (LN). The aim of our investigation was to study the epidemiological, clinical, biological and histological characteristics, outcomes and to evaluate the therapeutic protocols used for lupus nephritis’ treatment and to identify predictive factors of renal prognosis in patients with lupus nephritis. Method It was a retrospective study including patients over 16 years old with lupus nephritis proved by kidney biopsy and followed up over a period of 17 years in our department. Results We collected 155 women and 19 men with a sex ratio F / H of 8.2. The mean age at the time of the discovery of LN was 32.6 years with a maximum between 15 years and 45 years. The most frequent extra-renal manifestations were articular and dermatological manifestations (79%). Renal symptomatology was dominated by proteinuria noted in all patients, associated to a nephrotic syndrome in 68% of patients. At the time of diagnosis of LN, hematuria was present in 69% of patients and renal failure was present in half of cases. Immunologically, antinuclear antibody were positive in 89.1% of cases, anti DNA positive in 73.4% of cases, anti Sm positive in 79.8% of cases and Antiphospholipids were positive in 50% of cases, associated with an antiphospholipid syndrome in 14.9% of cases. We performed 243 renal biopsies with 174 initial and 69 iterative biopsies. The histological lesions were polymorphic dominated by LN class IV (36.6%) isolated or associated with LN class V (17.7%). All patients received a corticosteroid for induction or maintenance treatment. It was associated with immunosuppressive treatment according to different treatment regimens. The median duration of follow-up was 81.2 months. Renal outcome was marked by complete and sustained remission in 36.7% of cases, incomplete remission with chronic kidney disease in 34.5% of cases, chronic renal failure in 28.7% of cases. At univariate analysis, we identified the young age below 35 years at the time of the discovery of LN, the male sex, increased serum creatinine at the time of biopsy, proliferative forms, the presence of histological signs of chronicity and lesions of thrombotic microangiopathy as predictive factors of poor renal outcomes. Conclusion Lupus nephritis is one of the most common and serious manifestations of Systemic lupus erythematosus. The generalization of renal biopsy, the use of early codified therapeutic protocols and regular monitoring and evaluation of disease activity according to the appropriate scores can improve management and survival of patients with renal impairment.

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Preeclampsia, HELLP Syndrome, and Postpartum Renal Failure with Thin Basement Membrane Nephropathy: Case Report and a Brief Review of Postpartum Renal Failure

Case Reports in Obstetrics and Gynecology ◽

10.1155/2020/3198728 ◽

2020 ◽

Vol 2020 ◽

pp. 1-7

Author(s):

K. C. Janga ◽

Pavani Chitamanni ◽

Shraddha Raghavan ◽

Kamlesh Kumar ◽

Sheldon Greenberg ◽

...

Keyword(s):

Renal Failure ◽

Basement Membrane ◽

Renal Biopsy ◽

Hellp Syndrome ◽

Tubulointerstitial Nephritis ◽

Laboratory Evaluation ◽

High Dose ◽

Acute Tubulointerstitial Nephritis ◽

Thin Basement Membrane Nephropathy ◽

First Case

A 36-year-old primigravida female from a birthing center was referred for elevated blood pressure to the hospital two days after normal spontaneous vaginal delivery with nausea, vomiting, and diarrhea. During this two-day period, she was experiencing persistent vaginal bleeding and lower abdominal pains for which she took six doses of 600 mg ibuprofen. Further laboratory evaluation reflected leukocytosis, anemia, thrombocytopenia, elevation of liver enzymes, and renal failure with hyperkalemia requiring emergent hemodialysis once in the Medical Intensive Care Unit (MICU). She was diagnosed with HELLP syndrome with underlying preeclampsia. A week later, due to hypertension controlled with medications and nonoliguric renal failure with no active urine sediments, a renal biopsy was indicated to direct management. The renal biopsy supported the diagnosis of diffuse severe acute tubulointerstitial nephritis with hypereosinophilia and thin basement membrane nephropathy (see figures). She was subsequently treated with high-dose steroids which resulted in the normalization of blood pressures and renal function returning to baseline. We report the first case of acute tubulointerstitial nephritis in an individual with thin basement membrane nephropathy secondary to postpartum complications.

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Crescentic Glomerulonephritis with Anti-GBM and p-ANCA Antibodies

Case Reports in Nephrology ◽

10.1155/2012/132085 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Tariq Javed ◽

Parag Vohra

Keyword(s):

Renal Failure ◽

Renal Biopsy ◽

Recurrence Rate ◽

Crescentic Glomerulonephritis ◽

Diagnosis And Treatment ◽

Atypical Presentation ◽

Long Term Follow Up ◽

Pulmonary Renal Syndrome

We are presenting a case of renal failure with anti-GBM and p-ANCA antibodies positive. Patients with dual antibodies are considered to be a vasculitis-variant of anti-GBM antibody nephritis. These patients may have atypical presentation and it may delay diagnosis and treatment. Recurrence rate is higher in these patients. We reviewed the literature of cases and studies on cresenteric glomerulonephritis with anti-GBM and p-ANCA positive patients. We recommend that patients suspected with pulmonary-renal syndrome should be checked for anti-GBM and p-ANCA antibodies, should undergo renal biopsy and should should have close long term follow up to watch for recurrence.

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Analysis of clinical risk factors in relapsed patients with class IV lupus nephritis

Experimental and Therapeutic Medicine ◽

10.3892/etm.2018.6130 ◽

2018 ◽

Cited By ~ 1

Author(s):

Jing Wang ◽

Yuan‑Yuan Qi ◽

Xue‑Ping Chen ◽

Li Ma ◽

Li‑Li Zhang ◽

...

Keyword(s):

Risk Factors ◽

Lupus Nephritis ◽

Clinical Risk Factors ◽

Clinical Risk ◽

Class Iv

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A Novel Reduced Intensity Conditioning Can Induce a High Incidence of Sustained Donor Engraftment After Double Unit Cord Blood Transplantation (CBT) without Anti-Thymocyte Globulin

Blood ◽

10.1182/blood.v116.21.2351.2351 ◽

2010 ◽

Vol 116 (21) ◽

pp. 2351-2351

Author(s):

Doris M Ponce ◽

Craig Sauter ◽

Marissa Lubin ◽

Anne Marie Gonzales ◽

Glenn Heller ◽

...

Keyword(s):

Risk Factors ◽

Acute Gvhd ◽

Progression Free Survival ◽

High Dose ◽

Free Survival ◽

Prior Therapy ◽

Cd34 Cells ◽

High Incidence ◽

Related Mortality

Abstract Abstract 2351 CBT can be curative for patients with high-risk hematologic malignancies. However, patients of older age, those with extensive prior therapy, or significant co-morbidities may not tolerate high-dose myeloablative conditioning. Reduced intensity (RI) or non-myeloablative (NMA) conditioning has been successfully used in CBT, especially in patients with lymphomas. However, patients with myeloid malignancies without extensive prior therapy have an increased risk of graft rejection following NMA CBT. Further, the addition of anti-thymocyte globulin (ATG) to enhance engraftment increases the risk of serious infections and Epstein-Barr virus post-transplant lymphoproliferative disease, and could increase the risk of relapse. Therefore, we investigated the efficacy and safety of a novel ATG-free RI conditioning prior to double unit CBT in patients with acute leukemias and myelodysplasia with the hypothesis that this regimen can induce a high incidence of sustained donor engraftment. Conditioning consisted of cyclophosphamide 50 mg/kg (day -6), fludarabine 30 mg/m2/day × 5 (days -6 to -2), thiotepa 5 mg/kg/day × 2 (days -5 and -4), and total body irradiation 200 cGy × 2 (days -2 and -1). All patients received cyclosporine-A and mycophenolate mofetil for graft-versus-host disease (GVHD) prophylaxis. Between 10/01/07-04/30/10, 20 patients were transplanted. The median age was 56 years (range 18–69). Thirteen (65%) had AML (9 CR1, 4 CR2), 4 (20%) had ALL (3 CR1, 1 CR3), and 3 (15%) had MDS (with one patient also having follicular lymphoma). The majority had high-risk disease. Indications for RI conditioning were the risk factors for transplant-related mortality (TRM) with high-dose conditioning of age ≥50 years, and/or extensive prior therapy, and/or significant co-morbidities. Thirteen patients had only 1 of these risk factors, whereas 7 had ≥2 risk factors. Units were predominantly 4–5/6 HLA-matched to the recipient (one 6/6, twenty-four 5/6, fifteen 4/6). The median infused cell doses of the larger units were 2.7 × 107 total nucleated cells/kg (range 1.46–5.56) and 0.95 × 105 CD34+ cells/kg (range 0.35–3.32), and 1.89 × 107/kg total nucleated cells/kg (range 1.42–2.47) and 0.59 × 105/kg CD34+ cells/kg (range 0.18–1.52) for the smaller units, respectively. The cumulative incidence of sustained donor engraftment at day 45 was 95% (95%CI: 81–100). The single patient with graft failure was 100% donor in the day 21 bone marrow, but died early post-transplant of multi-organ failure without count recovery. The median time to neutrophil recovery ≥0.5 × 109/l was 25 days (range 13–43). The median total donor chimerism in the day 21 bone marrow was 94% (both units combined, range 71–100), and sustained engraftment was accounted for by one unit in 18/19 engrafting patients. The incidence of grade II-IV acute GVHD at day 100 was 55% (95%CI: 32–78), and 46% (95%CI: 21–71) of patients have had late acute GVHD requiring ongoing therapy or chronic GVHD to date. The incidence of day 100 transplant-related mortality (TRM) was 20% (95%CI: 2–38). Notably, none of the 13 patients with only one risk factor died of transplant-related causes. By contrast, 5/7 (71%) patients with ≥2 risk factors died of TRM by day 100 (p=0.03, Table 1). Two additional patients died of relapse. With a median follow-up of 13 months (range 3–31), 1 year progression-free survival is 74% (95%CI: 55–94) (Figure 1). We demonstrate that this ATG-free RI conditioning is associated with a high incidence of sustained donor engraftment, and acceptable toxicities in older patients without other risk factors. While longer follow-up is needed, progression-free survival is encouraging provided multiple risk factors are not present. This conditioning combined with double unit grafts warrants further investigation, and may also be a promising alternative to high-dose conditioning in younger patients. Table 1. Day 100 TRM according to number of risk factors (age ≥50 years, extensive prior therapy, significant co-morbidities). Risk Factors Day 100 TRM P Value 1 (N = 13) 0/13 (0%) 0.03 ≥2 (N = 7) 5/7 (71%) Figure 1. Progression-Free Survival At 1 Year Figure 1. Progression-Free Survival At 1 Year Disclosures: Giralt: Celgene: Honoraria, Speakers Bureau; Millenium: Honoraria, Speakers Bureau.

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Elimination of Established Risk-Factors in Primary Central Nervous System Lymphoma - Impact of High-Dose Chemotherapy Followed by Autologous Stem-Cell Transplantaion - a Multicenter Retrospective Analysis

Blood ◽

10.1182/blood.v118.21.3089.3089 ◽

2011 ◽

Vol 118 (21) ◽

pp. 3089-3089

Author(s):

Elisabeth Schorb ◽

Benjamin Kasenda ◽

Johannes Atta ◽

Nikolas von Bubnoff ◽

Thomas Elter ◽

...

Keyword(s):

Risk Factors ◽

Stem Cell ◽

Serum Level ◽

Performance Status ◽

Brain Structures ◽

High Dose Chemotherapy ◽

High Dose ◽

Prognostic Impact ◽

Deep Brain

Abstract Abstract 3089 Introduction: High-dose chemotherapy (HDT) and autologous stem-cell transplantation (ASCT) demonstrated high efficacy in the treatment of newly-diagnosed primary CNS lymphoma (PCNSL) in eligible patients (pts). Prognosis of PCNSL is associated with several clinical and histopathological risk factors (RF). Early complete response (CR) during chemotherapy (CHT) was recently reported to be an additional independent prognostic factor in pts undergoing polychemotherapy without HDT+ASCT. In this analysis, we examined the extent to which known RF determined survival in pts who were treated with HDT-ASCT. We additionally investigated the impact of HDT-ASCT specific factors (e.g. conditioning regimen) on survival. Pts. and Methods: Retrospective multicenter (N=10) analysis of 100 pts with untreated PCNSL who underwent HCT-ASCT with or without whole brain radiotherapy (WBRT). We used univariate and multivariate Cox regression analysis to investigate the prognostic impact of the following factors on overall survival (OS): early CR, age, performance status, involvement of deep brain structures, and LDH serum level and thiotepa dose. Until now data of 82 pts have been analyzed. Results: Median age at diagnosis was 53 years (range 23–69), the majority was male (67%). After a median follow-up of 58 months median OS was reached after 121 mo (range 3–149 mo). Before HDT+ASCT, 48/82 pts (58%) did not achieve CR (35 PR, 3 SD, 10 PD). After HDT+ASCT, 54/82 pts (66%) were in CR and altogether 39% of the pts were irradiated after HDT+ASCT. Of note, of those pts with PD before HDT+ASCT, 5/10 achieved CR after HDT+ASCT without WBRT. Overall, at the time of last follow-up, 24 pts have died. Of the surviving pts, 50 were in CR (86%), 2 in PR (3%), 2 in SD, and 2 developed progression /relapse. None of the established RF (age, performance status, involvement of deep brain structures, and LDH serum level) significantly distinguished outcome with regard to OS in uni and multivariate analysis. Additionally, neither CR before HDT-ASCT, nor sex nor the dose of thiotepa (10mg versu 20mg/m2) was associated with decreased OS. Conclusion: We conclude that HCT+ASCT is able to neutralize established RF in PCNSL. HDT+ASCT is a high-efficient treatment option independently of remission rate before entering HDT. New prognostic factors for pts eligible for HCT-ASCT have to be identified to guide therapy and care for PCNSL pts and to define stratification criteria for future trials. Most recent follow up will be presented. Disclosures: No relevant conflicts of interest to declare.

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