scholarly journals Report of 5 Cases of Extramedullary Myeloma with Central Nervous System Involvement Treated with a Combination of Carfilzomib/Thalidomide/Dexamethasone As a First Line Treatment at a Single Institution in Mexico

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5704-5704 ◽  
Author(s):  
Ramiro Espinoza ◽  
Diana Berenice Nolasco ◽  
Sosa Alejandro ◽  
Zapata Nidia ◽  
Cervera Eduardo ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Multiple Myeloma ◽  
Nervous System ◽  
Treatment Guidelines ◽  
Conflicts Of Interest ◽  
Central Nervous System Involvement ◽  
Infectious Complications ◽  
High Dose ◽  
Single Institution ◽  
Number Of Patients

Abstract Extramedullary myeloma (EMM) is defined by the presence of plasma cells outside the bone marrow in a patient with multiple myeloma (MM) (Touzeau & Moreau, 2016). EMM is by itself a rare entity with cases involving central nervous system (CNS) being rarer, it has an estimated incidence of 1-1.8% of all MM cases (Majd et al, 2015). Its presentation in comparison to classical MM has an adverse prognosis including a shorter progression free survival (PFS) (Gozzetti et al, 2015) and overall survival (OS) with a mean life expectancy of 1.5-6 months (Majd et al, 2015). Currently there are not international treatment guidelines for this disease. The aim of this report is to illustrate our experience at a single institution in Mexico, presenting five cases of CNS-EMM treated with proteasome inhibitor (carfilzomib) in combination with thalidomide, dexamethasone plus radiotherapy. The patient's characteristics and the treatment regimen are shown in table 1 and 2. Within three months, following the criteria to the international multiple myeloma working group (IMMWG), all five patients accomplished a positive response (one partial response [PR] four very good partial responses [VGPR]), at sixth months two patients improved their response from previously PR to a VGPR and from VGPR to complete response (CR), one sustained its VGPR and after this period of time two patients had progressive disease (PD) and died due to infectious complications. After the tenth month a third patient also died of infectious complications. By the end of this report two patients still alive, one is being evaluated for autologous stem cell transplantation (ASCT) and the other one wasn't eligible according to European Group for Blood and Marrow Transplantation (EBMT) risk score and will continue treatment without ASCT. Previously reported cases in the literature with old chemotherapy regimens shown to have median survival of 1.5 months (Petersen et al 1999), nowadays the recommended therapy is based on triplet induction therapy followed by high-dose melphalan/ASCT, a triplet consolidation therapy and maintenance treatment with lenalidomide (Touzeau & Moreau, 2016); so far there is no evidence of proteasome inhibitors penetrating the blood brain barrier (Nooka et al, 2013) and this might be the reason why the literature strongly recommends the use of lenalidomide as this one does penetrate into the CSF after oral administration (Muscal et al, 2012), we did not use lenalidomide due to the high monetary cost of the treatment but instead we use thalidomide in combination with carfilzomib and dexamethasone with improved OS in three of our five patients (VGPR + CR). Despite the small number of patients presented in this report and the lack of cytogenetics or FISH information, we think this might be a good therapeutic approached in patients with CNS-EMM, especially in those scenarios with not accessible resources or absence of clinical trials. Further studies evaluating the addition of monoclonal antibodies (daratumumab, elotuzumab) need to be done in order to improve the OS and PFS in this group of patients. Disclosures No relevant conflicts of interest to declare.

scholarly journals Treatment of secondary central nervous system involvement in systemic aggressive B cell lymphoma using R-MIADD chemotherapy: a single-center study

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yuchen Wu ◽  
Xuefei Sun ◽  
Xueyan Bai ◽  
Jun Qian ◽  
Hong Zhu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
Central Nervous System Lymphoma ◽  
Progression Free Survival ◽  
B Cell Lymphoma ◽  
High Dose ◽  
Cns Involvement ◽  
The Central Nervous System ◽  
Secondary Central Nervous System

Abstract Background Secondary central nervous system lymphoma (SCNSL) is defined as lymphoma involvement within the central nervous system (CNS) that originated elsewhere, or a CNS relapse of systemic lymphoma. Prognosis of SCNSL is poor and the most appropriate treatment is still undetermined. Methods We conducted a retrospective study to assess the feasibility of an R-MIADD (rituximab, high-dose methotrexate, ifosfamide, cytarabine, liposomal formulation of doxorubicin, and dexamethasone) regimen for SCNSL patients. Results Nineteen patients with newly diagnosed CNS lesions were selected, with a median age of 58 (range 20 to 72) years. Out of 19 patients, 11 (57.9%) achieved complete remission (CR) and 2 (10.5%) achieved partial remission (PR); the overall response rate was 68.4%. The median progression-free survival after CNS involvement was 28.0 months (95% confidence interval 11.0–44.9), and the median overall survival after CNS involvement was 34.5 months. Treatment-related death occurred in one patient (5.3%). Conclusions These single-centered data underscore the feasibility of an R-MIADD regimen as the induction therapy of SCNSL, further investigation is warranted.

Treatment of Burkitt's Like Lymphoma: A Single Institution Case Series

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4962-4962
Author(s):  
Vishal Ranpura ◽  
Shilpan S. Shah
Keyword(s):  
Central Nervous System ◽  
Bone Marrow ◽  
Nervous System ◽  
Peripheral Blood ◽  
Chemotherapy Regimen ◽  
Conflicts Of Interest ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Response To Treatment ◽  
Single Institution

Abstract Abstract 4962 Background: Burkits's like (BL) lymphoma is a new pathologic entity with features partially resembling to Burkitt's lymphoma and diffuse large B cell lymphoma. The incidence of BL lymphoma is infrequent and may present with lymph node enlargement, involvement of bone marrow and peripheral blood. The natural history of BL lymphoma and its treatment remains unclear. Here we present a single institution case series of five patients diagnosed with BL lymphoma and their treatment. Methods: We searched the pathology reports of all patients diagnosed with lymphoma over the last one year. No inclusion or exclusion criteria were used. Results: We identified a total of five patients with diagnosis of BL lymphoma. All patients presented with lymphadenopathy and with no involvement of bone marrow, central nervous system or peripheral blood. Table 1 summarizes characteristics of all patients, their chemotherapy regimen and subsequent response to treatment. Conclusion: BL lymphoma is a new pathologic entity with low incidence. Treatment with DA-EPOCH and R-HyperCVAD has very good response rate. The data is limited by single institution case series and limited follow up time. Further studies are recommended to evaluate optimal chemotherapy regimen. Table 1: Characteristics, presentation, treatment and response in patients with BL lymphoma No Age Sex LDH, Presentation Treatment Response Duration of response 1 68 F 386 Parotid and abdominal lymphadenopathy DA-R-EPOCH CR 16months 2^ 44 M 536 Neck, Axillary, media, abdomen pelvis and sacral mets R-HyperCVAD PR 3months 3* 66 F 310 Inguinal and pelvic lymphadenopathy R-HyperCVAD f/b Autologus BMT, CR 14months 4** 71 M 237 Parotid mass 3 cycles of DA-EPOCH f/b 3 cycles of R-CHOP CR 9months 5^ 52 M 186 Neck Lymphadenopathy R-HyperCVAD CR 3months DOX, DOXOL, and anthracenediones in soluble fractions of human myocardial strips after sequential DOX loading/clearance and anthracenedione treatment Notes: DA-REPOCH: Dose Adjusted Rituximab-Etoposide, Vincristine, Cyclophosphamide, Doxorubicine, Prednisone, R-HyperCVAD: Rituximab, hyperfractionated Cyclophosphamide, Vincristine, Doxorubicin, Dexamethasone, CR: complete remission, BMT: bone marrow transplantation, R-CHOP: rituximab, doxorubicin, cyclophosphamide, vincristine and prednisone. CNS: central nervous system * patient underwent bone marrow transplant because of relapse at current presentation. ** patient was changed from DA EPOCH to R-CHOP as patient was not able to tolerate EPOCH ^ Epatients are halfway through their treatment cycles and are actively getting treatment Disclosures: No relevant conflicts of interest to declare.

Intrathecal Rituximab for EBV-Associated Post-Transplant Lymphoproliferative Disorder with Central Nervous System Involvement Unresponsive to Intravenous Rituximab-Based Treatments: A Prospective Study

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 4352-4352
Author(s):  
Qifa Liu ◽  
Meiqing Wu ◽  
Jing Sun ◽  
Yu Zhang ◽  
Fen Huang ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Prospective Study ◽  
Lymphoproliferative Disorder ◽  
Conflicts Of Interest ◽  
Central Nervous System Involvement ◽  
Post Transplant ◽  
System Involvement ◽  
Dismal Prognosis ◽  
Significant Difference

Abstract Backgroud: Although the introduction of rituximab as a first-line treatment has improved outcome of post-transplant lymphoproliferative disorder (PTLD), PTLD with central nervous system involvement (CNS-PTLD) still has a dismal prognosis because of low penetrance across the blood-brain barrier. In this prospective study, we reported intrathecal rituximab was efficacy and safety in the patients with CNS-PTLD who had failed to respond to the intravenous rituximab-based treatments. Methods: From June 2009 to November 2013, 32 cases of EBV-associated PTLD were recorded in the Southern Medical University Institute of Hematology. Fourteen patients diagnosed with CNS-PTLD were enrolled in this prospective study. For the patients who failed to response to the initial intravenous rituximab-based treatments, sequential dose-escalation schedule of intrathecal rituximab (initial dose of 20mg, increased by 10mg each week and maximum dose of 50 mg) was administrated weekly. Results: Three patients were responsive and 11 were unresponsive to the initial treatments within one week after the treatments. For the 11 patients who failed to respond to the initial treatments, 9 patients received intrathecal rituximab within 7-11 days and 2 patients refused the treatment. After two cycles of rituximab-based treatments, 10 patients achieved complete response (CR), 2 patients were partial response and 2 patients were non-response. With a median follow up of 664 (range 18 to 1545) days after the diagnosis of CNS-PTLD, 7 patients survived and 7 died. The causes of death included PTLD progressing (n=3), PTLD relapse (n=1), GVHD (n=1), CMV pneumonia (n=1) and pseudomonas aeruginosa sepsis (n=1). The 3-year probability of OS was 45.7% ±14.7% in CNS-PTLD, which was no significant difference as compared to PTLD without CNS involvement(55.6% ±11.7%, P=0.706). Conclusion: Intrathecal rituximab might be an effective and safe method for CNS-PTLD in the allo-HSCT recipients who were unresponsive to the intravenous rituximab-based treatments. Disclosures No relevant conflicts of interest to declare.

scholarly journals Ocular manifestations and treatment of central nervous system lymphomas

2006 ◽  
Vol 21 (5) ◽  
pp. 1-7 ◽  
Author(s):  
Kaan Gündüz ◽  
Jose S. Pulido ◽  
Colin A. McCannel ◽  
Brian Patrick O'Neill
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Systemic Chemotherapy ◽  
Central Nervous System Involvement ◽  
Central Nervous System Lymphoma ◽  
Tumor Formation ◽  
Lymphoid Cells ◽  
Current Status ◽  
Intraocular Lymphoma ◽  
High Dose

✓ Intraocular primary central nervous system lymphoma (PCNSL), also called primary intraocular lymphoma (PIOL), is a subset of PCNSL in which lymphoma cells invade the subretinal pigment epithelial space and vitreous cavity with or without central nervous system involvement at the time of ocular diagnosis. The frequency of this rare condition has increased over the past years in immunosuppressed as well as immunocompetent patients. The authors review the current status of PIOL and elaborate on their group's experience with its diagnosis and treatment. The incidence of PIOL is increasing. There is evidence that chronic antigenic stimulation may result in the development of PIOL. Recent advancements in the diagnosis of PIOL include better handling of vitreous specimens for cytological studies, immunocytological investigation for lymphoid cells, flow cytometry, cytokine evaluation, and molecular analysis. Because PIOL has a nonspecific presentation, the differential diagnosis should include infectious and noninfectious causes presenting with vitreitis and/or subepithelial infiltration as well as paraneoplastic syndromes including CRMP-5 optic neuropathies. Given that therapy is long-term and has significant systemic and ocular complications, tissue diagnosis is important. Treatment of PIOL may include systemic chemotherapy in which high-dose methotrexate-based regimens are used as well as intraocular injections of methotrexate and rituximab (anti-CD20 antibody). Cranial and ocular external-beam radiotherapy is being used less often today. Further studies are needed to prevent the tumor formation in terms of eliminating antigenic load and inhibiting B-cell chemokines as well as to determine the optimal local and systemic chemotherapy and immunotherapy options in the management of PIOL.

Multiple myeloma with central nervous system involvement in a cat

2008 ◽  
Vol 233 (5) ◽  
pp. 743-747 ◽  
Author(s):  
Sherry L. Appel ◽  
Noël M. M. Moens ◽  
Anthony C. G. Abrams-Ogg ◽  
J. Paul Woods ◽  
Stephanie Nykamp ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Multiple Myeloma ◽  
Nervous System ◽  
Central Nervous System Involvement ◽  
System Involvement

scholarly journals Assessing Steroid Toxicity in the Elderly with Multiple Myeloma

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5557-5557
Author(s):  
Vidhya Nair ◽  
Hadi Mohammed ◽  
Sharmeen Mahmood ◽  
Pushpinderdeep Singh Kahlon ◽  
Philip Kuriakose
Keyword(s):  
Central Nervous System ◽  
Multiple Myeloma ◽  
Nervous System ◽  
Adverse Effects ◽  
Elderly Patients ◽  
Dose Reduction ◽  
The Elderly ◽  
High Dose ◽  
Full Dose ◽  
Starting Dose

Background The mainstay of treatment for Multiple Myeloma (MM) includes various combinations of chemotherapy, which generally includes high dose steroids. The median age at diagnosis for MM is above 60 years. Patients above the age of 70 may not be considered for an auto peripheral blood transplant, resulting in being treated with chemotherapy alone. This often leads to a relatively long period of steroid exposure. Increasing age is a risk factor for decreased tolerance to steroids, and increased drug toxicity. As such, the steroid dose (usually Dexamethasone) is often considered for reduction in patients above a certain age. However, there are no clear guidelines regarding a standard dose to use in the elderly, nor is there uniformity among clinicians in the way doses are chosen. Purpose To assess a) the starting dose of Dexamethasone (dex) in the elderly, b) frequency of dose reduction of dexamethasone, c) adverse effects of dex treatment in the elderly with MM, and d) average time after dose reduction. Methods We performed a 10 year retrospective chart review on patients, age 70 or greater treated at Henry Ford Health System with a diagnosis of MM from 2000-2015. Patients were grouped by age 70-75 years, 76-80 years, and greater than 80 years based on when treatment was initiated. We investigated the starting treatment dose of dex, ranging from 1-20 mg weekly and 21-40 mg weekly. Secondly, we assessed for the occurrence of dose reduction; and, if present, the length of time to reducing the dose. Lastly, the types of adverse effects to dex leading to dose reduction were grouped by system, such as, central nervous system, musculoskeletal, endocrine, gastrointestinal and psychiatric. Data collected was categorical, thus, no statistical tests were performed as this was a descriptive study. Results A total of 150 patients were reviewed and 91 patients met the inclusion criteria. Of these patients, 8 (8.8%) were started at doses between 1-20 mg and majority (62.5%) were ages 70-75, thus, there was no relation between lower starting dose and age. Of the 91 patients, 24 (26.4%) had a dose reduction and 11 (12.1%) had both chemotherapy and dex discontinued prior to therapy completion. Majority (87.5%) of patients that had a dose reduction were initially started at 40 mg. The reasons for dose reduction included adverse effects grouped by musculoskeletal (29.17%), psychiatric (16.67%), endocrine (12.3%), central nervous system (4.17%), and gastrointestinal (4.17%). Of note, 8 patients (33.3%) had dose reductions as result of their clinical trial requirement. The average length of dex therapy before dose reduction was 17.2 months. Conclusion The majority of elderly patients (age 70 or above) with MM tolerated full doses of dex without adverse effects. Secondly, there was no relation between lower starting dose for dex and advanced age. However, since there were limited patients (n=8) who started at a low dose, other than those on clinical trials, we were not able to do a comparison of starting doses. But we were able to show that the majority of patients tolerated full dose, despite their age. The most frequent cause of steroid toxicity was musculoskeletal, such as leg swelling. On average, elderly patients were able to tolerate full dose of dex for over 1 year prior to requiring a dose reduction. Summary Our data demonstrates no correlation between advanced age in MM and lack of tolerability of high dose steroids. In conclusion, current findings do not justify reduced doses solely based on age alone. Future studies could include investigating statistical analysis on steroid exposure and survivorship. Disclosures Kuriakose: Alexion: Consultancy, Honoraria, Speakers Bureau; Bayer: Consultancy.

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