scholarly journals Primary Testicular Lymphoma:a Single Center Experience and Literature Review

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5415-5415
Author(s):  
Ling Li ◽  
Fangwen Zhang ◽  
Ken H. Young ◽  
Wenjing Duan ◽  
Zhaoming Li ◽  
...  

Abstract Background: Primary testicular lymphoma (PTL), a rare and aggressive lymphoma mainly affecting the elderly, makes up 1-2% of Non-Hodgkin's lymphomas (NHL). Diffuse large B cell lymphoma (DLBCL) is the most common histological type of PTL. At present, comprehensive management is used for the treatment of PTL, such as operation, chemotherapy and radiotherapy. Although there are multiple treatment methods for PTL, its prognosis is very poor. Materials and Methods: We retrospectively studied clinical data of 28 patients who were 10 to 82 years old and were diagnosed with PTL between March 2006 and November 2014.The clinical data included clinical characteristic, staging, treatment and outcomes. All of the patients received therapy [rituximab, CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone), intrathecal prophylaxis, or irradiation for the contralateral testis] after orchiectomy. The therapeutic effects were evaluated as complete response (CR), partial response (PR), progressive disease (PD) and stable disease (SD). The prognostic evaluation was based on age, lactic dehydrogenase (LDH), Eastern Cooperative Oncology Group Performance Status (ECOG PS) and staging. Results: The main clinical features of the 28 patients were shown in Table 1. All patients had testicular mass. Nine patients achieved complete response (CR), 8 patients had partial response (PR), and progressive disease (PD) occurred in 3 patients after 13, 12 and 20 months after treatment, respectively(Table 2). Eight patients died. The median overall survival (OS) was 19.6 months and the median progression-free survival (PFS) was 19.4months(Figure 1,2). Conclusion: PTL is a highly aggressive disease. Although there are multiple treatment methods for PTL, it readily recurs and its prognosis is very poor. From this study, we can see that age, LDH, ECOG PS and staging are the main factors to affect PFS and OS of the patients with PTL(Table 3). Disclosures No relevant conflicts of interest to declare.

Esculapio ◽  
2021 ◽  
Vol 16 (4 (oct 2020 - dec 2020)) ◽  
Author(s):  
Faiza Rehman Lodhi ◽  
Amjad Zafar ◽  
Muhammad Abbas Khokhar ◽  
Ali Waheed Goraya ◽  
Sobia Yaqub

Objective: Diffuse large B cell lymphoma (DLBCL) is a lymphoid B cells neoplasm with a diffuse pattern and high proliferation rate. Cyclophosphamide, doxorubicin, vincristine and prednisolone (CHOP) was considered effective as other complicated regimens with more toxicity profile. Rituximab is a monoclonal antibody directed against CD20 positive B cell. It has good activity therapeutically in patients of DLBCL. It increases response rates and survivals when added to CHOP chemotherapy. Although R-CHOP is more effective but due to high cost of Rituximab it is usually not incorporated with chemotherapy in most of our patients and CHOP is still used extensively. Due to heterogeneity of disease and difference in ethnicity, there may be difference in outcomes of two regimens. This study will help us in tailoring our management plan that will result in better outcome of patients. Methods: 70 patients aged between 20-65 years having DLBCL were taken in this study. We rando-mized patients by lottery method into two groups. Group I received CHOP with dose of Cyclophosphamide 750mg/m2, Doxorubicin 50mg/m2, Vincristine 1.4 mg/m2 and prednisolone 40mg/m2.Chemotherapy was given on Day-1 while prednisolone was given for 5 days from Day-1 of chemotherapy. Group II received R- CHOP which includes same chemotherapy with same dosage. Rituximab was included in Group II with dose of Rituximab 375 mg /m2. Each cycle was given at three weeks interval. Response in terms of CR (Complete Response), PR (Partial Response), SD (Stable Disease) or PD (Progressive Disease) was evaluated as per leukemia network after 4 cycles of chemotherapy. The quantitative variables were calculated by taking mean and standard deviation. The response was assessed in percentage and frequencies and compared by applying chi square test. Results: Group I had 37.1% while Group II had 68.6% complete response with p value of 0.019. Partial response was 48.6% in Group I while 20.0% in Group II. 14.3% in Group I and 8.6% in Group II either had stable disease or progressive disease. Conclusions: R-CHOP has superior response rates as compared to CHOP, therefore, whenever possible Rituximab should be added as target therapy in chemotherapy. Key Words: Diffuse large B cell lymphoma, CHOP, R- CHOP How to Cite: Lodhi F.R, Zafar A, Khokhar M.A, Goraya AW, Ashraf S, Yaqub S. Comparison of R-CHOP with CHOP in patients of diffuse large B cell lymphoma. Esculapio.2020;16(04):79-82.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4952-4952
Author(s):  
Ashis Mukhopadhyay ◽  
Melissa Adde ◽  
Ian Magrath ◽  
Soma Mukhopadhyay

Abstract Background: The Non-Hodgkin’s Lymphomas (NHL) in childhood are usually high grade and diffuse histology. They require intensified short duration chemotherapy in contrast to adult NHL. The aim of our study was to observe the result of aggressive short duration chemotherapy in paediatric NHL. Materials & Methods: We included consecutive 160 paediatric NHL patients in paediatric haemato-oncology department of Netaji Subhash Chandra Bose Cancer Research Institute during period from June 1996 to December 2007. The inclusion criteria were patients less than 25 yrs of age with a diagnosis of NHL and Patients are clinically staged according to the St. Jude’s (Murphy’s) classification. Patients with > 25% blasts in the bone marrow were treated as leukemia and excluded from the study. Each patient received 3 cycles A and 3 cycles B of MCP842 protocol of INCTR. Response was assessed at the completion of 2cycles of chemotherapy (1 each of A and B). Result: A total of 160 previously untreated patients were entered in this study. The age range was 1 to 25 yrs (median 13.5). Fourty Eight (48) patients had Lymphoblastic Lymphoma (LL), 64 patients (40%) had Burkitt Lymphoma, 40 (25%) diffuse Large B Cell Lymphoma (DLCL) and 8 (5%) had Anaplastic Large Cell. The abdomen was the most common site (56%) of involvement followed by the mediastinum (15.62%). One hundred and Thirty four (134)(83.75%) patients achieved complete response after 2 cycles of therapy, 14 patients (8.75%) achieved partial response and 6 (3.75%) had no response, 6 (3.75%) were not evaluable. With median follow up of 4 & 1/2 yaers (range 7 months – 10 years) a total of 38 (23.75%)patients (19 LL, 13 Burkitt Lymphoma, 5 DLCL and 1 ALCL) have died. The causes of death were progressive disease in 26 (16.25%), infection in 6 (3.75%), tumour lysis in 4 (2.5%), hepatitis in 1 (0.63%), and unknown 1 (0.63%). One hundred and twenty two (122) patients (76.25%) are alive and in complete remission. The patients tolerated chemotherapy well. Grade IV febrile Neutropenia was seen in 34 patients (21.25%). Conclusion: Result of MCP 842 was promising and we will continue the protocol in future.


2021 ◽  
Vol 9 (4) ◽  
pp. e001752
Author(s):  
Rivka R Colen ◽  
Christian Rolfo ◽  
Murat Ak ◽  
Mira Ayoub ◽  
Sara Ahmed ◽  
...  

BackgroundWe present a radiomics-based model for predicting response to pembrolizumab in patients with advanced rare cancers.MethodsThe study included 57 patients with advanced rare cancers who were enrolled in our phase II clinical trial of pembrolizumab. Tumor response was evaluated using Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 and immune-related RECIST (irRECIST). Patients were categorized as 20 “controlled disease” (stable disease, partial response, or complete response) or 37 progressive disease). We used 3D-slicer to segment target lesions on standard-of-care, pretreatment contrast enhanced CT scans. We extracted 610 features (10 histogram-based features and 600 second-order texture features) from each volume of interest. Least absolute shrinkage and selection operator logistic regression was used to detect the most discriminatory features. Selected features were used to create a classification model, using XGBoost, for the prediction of tumor response to pembrolizumab. Leave-one-out cross-validation was performed to assess model performance.FindingsThe 10 most relevant radiomics features were selected; XGBoost-based classification successfully differentiated between controlled disease (complete response, partial response, stable disease) and progressive disease with high accuracy, sensitivity, and specificity in patients assessed by RECIST (94.7%, 97.3%, and 90%, respectively; p<0.001) and in patients assessed by irRECIST (94.7%, 93.9%, and 95.8%, respectively; p<0.001). Additionally, the common features of the RECIST and irRECIST groups also highly predicted pembrolizumab response with accuracy, sensitivity, specificity, and p value of 94.7%, 97%, 90%, p<0.001% and 96%, 96%, 95%, p<0.001, respectively.ConclusionOur radiomics-based signature identified imaging differences that predicted pembrolizumab response in patients with advanced rare cancer.InterpretationOur radiomics-based signature identified imaging differences that predicted pembrolizumab response in patients with advanced rare cancer.


2019 ◽  
Vol 3 (12) ◽  
pp. 1815-1825 ◽  
Author(s):  
M. O’Dwyer ◽  
R. Henderson ◽  
S. D. Naicker ◽  
M. R. Cahill ◽  
P. Murphy ◽  
...  

Abstract Daratumumab (DARA) has shown impressive activity in combination with other agents for the treatment of multiple myeloma (MM). We conducted a phase 1b study to assess the safety and preliminary efficacy, as well as potential mechanisms of action, of DARA (16 mg/kg) in combination with a weekly schedule of subcutaneous bortezomib (1.3-1.5 mg/m2), cyclophosphamide (150-300 mg/m2), and dexamethasone (40 mg) (CyBorD DARA) as initial induction before autologous stem cell transplantation (ASCT). Eligible patients were ≤70 years of age with untreated MM requiring treatment and who lacked significant comorbidities. A total of 18 patients were enrolled. Their median age was 56 years (range, 32-66 years), and all patients had Eastern Cooperative Oncology Group performance status ≤1. The International Staging System stages were I, II, and III in 78%, 17%, and 6% of patients, respectively; 28% of patients had high-risk genetic features. There was no dose-limiting toxicity, and the incidence of grade 3 or 4 infection or neutropenia was &lt;10%. On an intention-to-treat basis, 94% achieved ≥very good partial response with ≥complete response in 44% of patients. Among 14 of 15 patients who underwent ASCT and were evaluable for response, all 14 achieved at least very good partial response, with 8 (57%) of 14 achieving complete response. After ASCT, 10 (83%) of 12 patients in whom minimal residual disease analysis was possible were negative at a sensitivity of 10−5 (56% on intention-to-treat/whole study population) according to next-generation sequencing. Flow cytometry analysis of patient samples indicated CyBorD DARA induced activation of macrophage-mediated antibody-dependent cellular phagocytosis. This trial was registered at www.clinicaltrials.gov as #NCT02955810.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 3964-3964 ◽  
Author(s):  
Mark T Drayson ◽  
Oscar Berlanga ◽  
Tim Plant ◽  
Nicola J Newnham ◽  
Philip Young ◽  
...  

Abstract Abstract 3964 Introduction: Monitoring multiple myeloma (MM) patients is required to help guide therapy and assess response. Currently the most commonly employed tests to monitor MM patients include serum protein electrophoresis (SPEP), immunofixation (IFE), 24hr urine and serum free light chain (FLC) analysis. Whilst electrophoretic tests are adequate for IgG MM, they may be inadequate for IgA MM where the monoclonal immunoglobulin co-migrates with other serum proteins (∼60% of cases). Recently the inclusion of FLC testing to identify patients in stringent complete response (sCR) has been recommended. Novel nephelometric reagents have become available that can quantify IgA kappa and IgA lambda immunoglobulins (HLC) in serum. Here we assess the use of these tests at maximum response to detect residual disease and comment on the prognostic value of sCR. Patients, materials and methods:196 IgA MM patient samples recruited from the MRC IX trial were assessed at maximum response. Briefly, patients were randomised on to intensive (I: induction therapy with CVAD or CTD followed by high-dose melphalan and ASCT) and non-intensive (NI: clodronate or zoledronic with MP or CTDa) arms. Median age was 59 years (range: 37–71) and 74 years (range: 65–89) for patients in the I and NI arms, respectively. 31% patients in the I arm and 39% in the NI arm presented with stage III disease. Samples were taken 3 months after autologous stem cell transplant or maximum response and analysed nephelometrically using serum FLC and HLC immunoassays. Ratios for both FLC and HLC were compared to normal ranges (FLC normal ratio= 0.256–1.65, IgAkappa/IgAlambda normal ratio=0.8–2.04). Additionally, isotype-matched immunoparesis was described as <0.48 g/L IgAkappa and <0.36g/L IgAlambda. Results were compared to SPEP, IFE and total immunoglobulin assays (where immunoparesis was described as <6g/L IgG and <0.4g/L IgM). 2-year progression free survival (PFS) was determined using Kaplan Meier analysis (SPSS v 19.0). Results: 3 months post-therapy, patient responses were: I arm: progressive disease (PD): n=1(<1%); minimal response (MR): n=1(<1%); partial response (PR): n=10(8%); very good partial response (VGPR): n=35(29%); complete response (CR): n=13(11%); sCR: n=58(48%); for two patients no data was available; NI arm: progressive disease (PD): n=3(4%); stable disease (SD): n=5(7%); MR: n=10(13%); PR: n=31(41%); VGPR: n=16(21%); CR: n=11(15%). Within the 2-year follow up, 41% patients in the I arm and 46% in the NI arm relapsed. Median PFS was not significantly different between the two arms (not reached v 7 months, p=0.65). Response (3VGPR) was not associated with PFS in either arm (I, p=0.717; NI, p=0.236). By contrast, achievement of a sCR (p=0.013) was significantly associated with longer PFS in the I arm and tended towards significance in the NI arm (p=0.063). An abnormal HLC ratio was associated with shorter PFS in both arms (I, p=0.002; NI, p=0.032). In all patients achieving a 3VGPR, an abnormal HLC ratio was associated with shorter PFS (p>0.0001). Similarly, in patients achieving a 3CR an abnormal HLC ratio was also associated with shorter PFS (p=0.04). Furthermore, patients achieving a CR where both FLC and HLC ratios were normal had a significantly longer PFS than those with an abnormal FLC or HLC ratio (median PFS not reached v 18 months, p=0.007). Isotype-matched immunoparesis was associated with shorter PFS in all patients achieving a CR (p=0.061). By contrast, systemic immunoparesis of either IgG or IgM immunoglobulins were not associated with PFS (p=0.525 and p=0.964, respectively). Discussion: Novel therapies have dramatically improved MM patient outcomes, however improvements in the assessment of those outcomes has not followed a similar trajectory. Here we present data suggesting immunoglobulin HLC ratios may be better markers of residual disease than electrophoretic methods. In addition a response category based on normalisation of both FLC and HLC ratios may be more valuable than sCR. Finally, the identification of isotype matched immune reconstitution as a marker of outcome suggests a preferential suppression of immunoglobulin production not previously reported. Conclusion: Normalisation of the FLC and HLC ratio at maximum response is a better assessment of disease than IFE. Further work is required to validate these results and to assess FLC and HLC ratios against multi-parametric flow cytometry. Disclosures: Young: Binding Site: Employment.


Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4893-4893
Author(s):  
Carlos Daniel Bermudez ◽  
Martha Leticia Suarez ◽  
Sergio Andres Cancelado ◽  
Carlos Alberto Ramirez

Abstract Abstract 4893 Background: The follicular lymphoma (FL) it is a subset of non-Hodgkin lymphomas, accounting for between 15% and 30% of new diagnoses of lymphoma. It has raised many combinations of treatments, but the long-term results remain unchanged. At present there is no consensus on the first line therapy for the management of follicular lymphoma. Since the advent of rituximab use, this change, achieving better response rates and survival, the paradigm is the use of R-CHEMO, but there are many active combinations, including the use CVP, CHOP, FCM, and others, which have shown benefit, but with consequences given for severe toxicity, and treatment-related deaths. In Colombia, the situation it is not different, the use of chemotherapy is left for consideration by the treating physician. This study aimed to assess treatment preferences for patients with new diagnosis of follicular lymphoma in Colombia, evaluate the responses of the combination R-CHEMOTHERAPY in our population, considering the specific ethnic characteristics and limitations of a developing country. Patients and Methods: The study included patients diagnosed with follicular lymphoma confirmed by hematopathologist with experience by performing at least 10 immunohistochemical tumor markers. This study is a descriptive study. During the study took into account the principles of autonomy, beneficence and justice written in the Belmont report. Informed consent was obtained from patients for participation in this study. It also welcomes the resolution expressed law Colombian Ministry of Health No. 008 430 1993 (4 October 1993) Article 11 investigation classified as safe. We analyzed cases diagnosed from January 2007 to July 2011 in two private institutions in the city of Bogotá, Colombia, we obtained 20 cases meeting the inclusion criteria. All the patients were scheduled to undergo primary therapy with 6 cycles of full-dose R-CVP or R-CHOP. Results: in this group, there were 5 men and 15 women (75% of all patients), and the median age at diagnosis was 56 years, the initial stage was 10% for stage II, 40% for stage III and the remaining 50% for stage IV, the bone marrow compromise reached by 45% (9 cases). The initial functional status classified by the ECOG scale was 0: 45%, 1: 50% and 3: 5%. the B symptoms were present in 95% of the patients analyzed. In accordance with the International Prognostic Index (FLIPI), we find the following: low risk: 25% (5 cases), intermediate risk: 35% (7 cases) and high risk: 40% (8 patients). the pathological grading was grade 1: 55% (11 patients), grade 2 for 35% (4 patients) and 3 for 10% (2 patients). When we reviewed found that the preferred treatment, for 80% of the population uses the R-CVP and the remaining 20% use of R-CHOP scheme, one patient in the R-CHOP group was complicated with high-risk febrile neutropenia requiring hospitalization. There were no treatment-related deaths. We found that 80% of patients achieved a complete hematologic remission and 20% partial response for an overall response of 100%. If we analyze separately patients treated with R-CVP scheme was 75% complete response and 25% partial response, with an overall response of 100%. Conclusion: This study shows the preference of treatment in 2 institutions in Bogotá, Colombia, which are in accordance with international guidelines, the results show a population with similar general characteristics with the others studies, in which a 100% overall hematologic response was achieved, 75% complete response for the R-CVP and 100% for the R-CHOP, however the latter with 25% of severe infectious complications. We consider a good treatment strategy for the implementation in our population is the R-CVP scheme, which is well tolerated, showing benefit in our patients and remission rates even higher than the studies previously published. Disclosures: No relevant conflicts of interest to declare.


Sarcoma ◽  
2013 ◽  
Vol 2013 ◽  
pp. 1-9 ◽  
Author(s):  
Julian F. Guest ◽  
Erikas Sladkevicius ◽  
Nicholas Gough ◽  
Mark Linch ◽  
Robert Grimer

Soft tissue sarcomas are a rare type of cancer generally treated with palliative chemotherapy when in the advanced stage. There is a lack of published health utility data for locally advanced “inoperable”/metastatic disease (ASTS), essential for calculating the cost-effectiveness of current and future treatments. This study estimated time trade-off (TTO) and standard gamble (SG) preference values associated with four ASTS health states (progressive disease, stable disease, partial response, complete response) among members of the general public in the UK (n=207). The four health states were associated with decreases in preference values from full health. Complete response was the most preferred health state (mean utility of 0.60 using TTO). The second most preferred health state was partial response followed by stable disease (mean utilities were 0.51 and 0.43, respectively, using TTO). The least preferred health state was progressive disease (mean utility of 0.30 using TTO). The utility value for each state was significantly different from one another (P<0.001). This study demonstrated and quantified the impact that different treatment responses may have on the health-related quality of life of patients with ASTS.


Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5390-5390
Author(s):  
Alma Aslan ◽  
Alev Turker ◽  
Ayse Kars ◽  
Ibrahim Barista ◽  
Evren Ozdemir

Abstract Diffuse large B-cell lymphoma (DLBCL) is a treatable and potentially curable malignancy that is increasing in prevalence in the elderly. Because of geriatric considerations including functional status and comorbidities and increased toxicity concerns, older patients are sometimes given reduced dose therapy. Mitoxantrone has a broad anti-tumour activity including lymphoma with potentially less cardiotoxicity than doxorubicin, which may be of particular importance in the treatment of elderly aggressive lymphoma patients. This study's objective was to evaluate the efficacy and safety of R-CNOP (rituximab plus cyclophosphamide/mitoxantrone/vincristine/prednisone) combination regimen at standart doses in elderly patients with newly diagnosed diffuse large B-cell lymphoma. Mitoxantrone was given at the dose of 12 mg/m2. Twenty-four patients were enrolled. All patients had an ECOG performance status ≤ 2. Twenty-two patients (91.7%) had several comorbidities, the most common were cardiac diseases and hypertension (56%). Excluding one patient and with a borderline heart function (ejection fraction of 50%), all patients had an ejection fraction (EF) of ≥60%. The median age was 71 years (50-78) and 20 (83%) were female. Nineteen (79.2%) patients were stage III-IV and 15 (62.5%) patients had high-intermediate or high aa-IPI score. Thirteen patients (52%) had Ki-67 proliferation index > 80%. Three patients was myc positive. Twelve were non-germinal center phenotype. Median number of R-CNOP cycles administered to the patients was 6 (3-6). Treatment response was evaluated with PET-CT before and after the treatment. Of 24 patients, 19 (79.2%) had complete response, 3 (12.5%) had partial response, 2 (8.3%) had refractory/progressive disease. Two patients who had refractory/progressive disease received salvage chemotherapy and achieved complete response. The median follow-up was 18.78 (5.6-82.8) months. The median time to progression for responding patients (n=3) was was 14.3 (4.5-79.1) months. Median DFS and OS were not reached. Main toxicity was hematological. Grade ≥3 hematologic toxicity occurred in 13 patients: neutropenia (37.5%), thrombocytopenia (8.4%), and anemia (8.3%). Four (16.7%) patients developed febrile neutropenia and two patient developed pulmonary thromboembolism during therapy. Treatment cycle postponed in 11 (45.8%) patients without dose reduction because of hematological toxicity. Nineteen (79.2%) patients received G-CSF primary prophylaxis. After the treatment EF decreased (≤50%) only in two patients, who had coronary artery disease before the treatment. The patient who had borderline EF had stable EF after the treatment. R-CNOP was shown to be an effective and safe regimen in elderly patients with diffuse large B-cell lymphoma. The majority of the patients had long term disease control. Hematological toxicity was common. Disclosures No relevant conflicts of interest to declare.


Blood ◽  
1985 ◽  
Vol 65 (1) ◽  
pp. 115-119 ◽  
Author(s):  
F Calvo ◽  
S Castaigne ◽  
F Sigaux ◽  
M Marty ◽  
L Degos ◽  
...  

Abstract Seven patients with hairy cell leukemia were treated by intensive chemotherapy because they were considered to have a progressive disease and a poor short-term prognosis. The mean age was 47 years (range, 36 to 58). Six of seven patients had prior splenectomies with minor or transient hematologic responses. One patient had no spleen enlargement. The seven patients had never received any cytotoxic drugs and had prolonged granulocytopenia (less than 300/microL) with recurrent, severe infectious episodes. Chemotherapy included Rubidazone (zorubicine hydrochloride) 450 mg/m2 on day 1, arabinosyl cytosine 200 mg/m2/d from day 1 to day 5, and cyclophosphamide, 2,000 mg/m2 on day 5. Responses were assessed through examination of repeat bone marrow biopsy specimens and blood counts. A complete response was defined as normal blood counts associated with the disappearance of hairy cell infiltration and fibrosis on the bone marrow biopsy specimens. A partial response was defined as normal blood counts with persistence of leukemic cells in the bone marrow. Three patients achieved a complete response, and one patient had a partial response. Three patients died of infectious complications during induction chemotherapy. For the responding patients, the mean duration of aplasia was 37 +/- 5 days. Follow-up for the responding patients has been 44+, 24, 32+, and 23+ months. One patient with a complete response died while on maintenance therapy. We conclude that complete and prolonged histologic remission of hairy cell leukemia can be obtained with intensive chemotherapy. The toxicity of chemotherapy is such, however, that progressive disease after splenectomy needs to be more clearly defined.


Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 5010-5010
Author(s):  
Brady Beltran ◽  
Antonio Paredes ◽  
Celia Moises ◽  
Gadwin Sanchez ◽  
Luis Riva ◽  
...  

Abstract Background: Selective ligands of retinoid X receptor (i.e. bexarotene) are used in the treatment of early and advanced stages of cutaneous T-cell lymphoma (CTCL). Relapsed or refractory cases of CTCL have poor prognosis and are in need of safe and more effective therapies. Synergy between bexarotene and interferon or phototherapy has been previously described. Objective: Report a Peruvian single-institution experience on eight cases of relapsed or refractory CTCL who were treated with low-dose bexarotene in combination with phototherapy or interferon alfa-2a. Methods: From 2002 to 2007, eight patients with relapsed or refractory CTCL were treated with bexarotene 300 mg by mouth daily in combination with interferon alfa-2a 9 million units subcutaneously three times a week (five patients) or phototherapy (two patients received PUVA therapy and one patient received narrow-wave UVB therapy). Bexarotene, interferon and phototherapy were continuously administered until progression of disease or unacceptable toxicity. Five patients were diagnosed with mycosis fungoides (MF), two patients had aggressive epidermotropic CD8+ cytotoxic lymphoma (AECL) and one patient had Sezary Syndrome (SS). Results: Mean age was 58 years (range 36 to 70). Patients were exposed to three prior lines of therapy in average (range 2 to 5). One patient had complete response, four patients had partial response and three patients experienced progressive disease. The overall response for the entire group was 62.5% with a mean duration of response of 20 months (range 7 to 67 months). Forty percent (2/5) of patients had partial response to the bexarotene and interferon combination; no complete responses were observed. The two responders, one patient with SS and one patient with MF, are alive 67 and 17 months, respectively. On the other hand, 100% (3/3) of patients responded to the bexarotene and phototherapy combination. A patient with stage I MF experienced a complete response and two patients had a partial response, one patient with MF and one with AECL. Of note, the patient who achieved a complete response was treated with bexarotene and narrow-wave UVB. Most commonly side effects observed were mild, five patients experienced grade 1–2 hypercholesterolemia, hypertriglyceridemia grade 1–2 was seen in four patients and grade 3 in one case. Two patients developed grade 1–2 hypothyroidism. Our patient with SS developed cardiomyopathy related to interferon after 17 months of therapy and continues on single-agent bexarotene. The use of bexarotene in the treatment of AECL has seldom been reported. We are also reporting one of the longest lasting responses described in a patient with SS treated with bexarotene. Conclusions: Low-dose bexarotene in combination with phototherapy or interferon alfa-2a in the treatment of relapsed and refractory CTCL seems safe and effective. Further research is necessary to develop improved, more effective therapies. Table 1. Characteristics of reported patients Age Sex Histology Stage Number previous treatments Treatment Response Duration response (months) Bexarotene/interferon Bexarotene/phototherapy F: female; M: male; SS: Sezary syndrome; AECL: aggressive epidermotropic CD8+ cytotoxic lymphoma; MF: mycosis fungoides; PR: partial response; CR: complete response; PD: progressive disease 69 F SS - 2 X PR 67 61 M MF III 2 X PR 17 36 F AECL IB 4 X PD - 70 F MF IIB 2 X PD - 57 M MF IIB 5 X PD - 44 M MF IB 2 X CR 17 59 M MF IIB 2 X PR 7 67 M AECL IIB 3 X PR 12


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