Non-Gastric Extranodal Marginal Zone MALT B-Cell Lymphoma In a Peruvian Cancer Referral Center (Instituto Nacional de Enfermedades Neoplasicas)

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 5099-5099
Author(s):  
Jose Carlos Revilla Lopez ◽  
Jorge A Benavides vasquez ◽  
Lina P Huerta Saenz
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Cancer Center ◽  
Anatomical Location ◽  
First Line ◽  
Common Location ◽  
Ann Arbor

Abstract Abstract 5099 Non-Gastric Marginal Zone B-Cell Lymphoma of Mucosa-Associated Lymphoid Tissue in a Peruvian Referral Cancer Center (Instituto Nacional de Enfermedades Neoplasicas) Jose C. Revilla, MD1, Jorge A. Benavides, MD, Lina Huerta-Saenz, MD*,2. 1Oncological Medicine Deparment, Instituto Nacional de Enfermedades Neoplasicas, Lima, Peru,2Albert Einstein Medical Center, Philadelphia, PA, USA Historically, non-gastric marginal zone MALT lymphomas have been difficult to characterize because of their wide anatomical distribution. Previous studies have shown broad distribution patterns with different clinical courses and responses to therapy. There are still questions regarding which could be the best clinical management and this remains without a conclusive answer. A retrospective 10-year study from 1997 to 2007 of patients with diagnosis of non-gastric marginal zone B-cell lymphoma of mucosa-associated lymphoma tissue (MALT) was performed in a Peruvian referral cancer center. To be eligible for the study, all patients had to have pathological confirmation of diagnosis with a clinically dominant non-gastric site of localization. Disease stage was defined according to the Ann Arbor staging criteria, Zubrod performance status scale and location of extranodal disease. Serum lactate dehydrogenase (LDH) and β2-microglobulin levels were used for laboratory monitoring. Bone marrow compromise was also evaluated. All data regarding medical treatment, response to treatment, failure, death and disease status at last follow-up were obtained. The results showed 23 patients with confirmed diagnosis, 74% of whom were female, and the mean age at time of diagnosis was 52 years old. The clinical predominant disease stages at the time of diagnosis were Ann Arbor stage I or II (76%). B-symptoms were present in 35% of the patients and only 13% of them had increased LDH levels. Bone marrow compromise was not present in the majority of patients (96%) and 75% of them had a low international prognostic index (IPI) score. The predominant anatomical location was the conjunctiva (39.1%) followed by the lung (13.1%). These findings were different from previous studies in European and North American populations where the salivary glands were the most common location. Ethnicity may be proposed as a factor related to these differences in anatomical location patterns. Regarding medical management, chemotherapy was the first-line of treatment and anthracyclines were the agents predominantly used (47.8% of patients). Radiotherapy alone was given to 18.2% of patients during the study and combinations of chemotherapy and radiotherapy were used in 4.6% of patients. Follow-up was completed for an average of 25.1 months (range=14.3-35.9 mo), with a complete response to the treatment in 56.6% of patients. Hashimoto's thyroiditis and hepatitis C were the only concomitant medical conditions reported for one patient each, respectively. The survival curve did not show significant differences by gender (p<0.05). In this study, non-gastric B-cell lymphoma of mucosa-associated lymphoma tissue (MALT) showed an indolent clinical course with female predominance and early stages at time of diagnosis. Ocular conjunctiva was the most common location and most of the patients showed good clinical response to the first-line chemotherapy treatment; additionally, there was a low associated mortality rate. Disclosures: No relevant conflicts of interest to declare.

High Rate of Initial Treatment Failure in Patients with Primary Mediastinal B-Cell Lymphoma Treated with R-CHOP

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 1601-1601 ◽  
Author(s):  
Jeremy S. Abramson ◽  
Matthew D Hellmann ◽  
Yang Feng ◽  
Jeffrey A. Barnes ◽  
Tak Takvorian ◽  
...  
Keyword(s):  
Risk Factors ◽  
B Cell ◽  
Cell Lymphoma ◽  
Univariate Analysis ◽  
B Cell Lymphoma ◽  
Refractory Disease ◽  
Large B Cell Lymphoma ◽  
Ann Arbor ◽  
Large B Cell

Abstract Abstract 1601 Introduction: Primary Mediastinal B-cell Lymphoma (PMBCL) is an uncommon variant of Diffuse Large B-cell Lymphoma (DLBCL). Given the rarity of this disease, data guiding management is extrapolated from DLBCL trials, or from small retrospective analyses limited to PMBCL. Prospective evaluation of R-CHOP in the MiNT trial showed excellent results in PMBCL, but this trial was limited to young low risk patients. We present the largest retrospective series to date of R-CHOP for PMBCL in all risk groups. Methods: We identified cases of PMBCL at our institution using a comprehensive clinicopathologic database derived from tumor registry data. Natural language processing software was used to search pathology reports for terms of “mediastinal lymphoma,” “mediastinal large cell lymphoma,” “mediastinal large B-cell lymphoma,” as well as “lymphoma” in mediastinal biopsy specimens. Cases were included if they met clinicopathologic criteria for PMBCL, defined as a large B-cell lymphoma with typical features for PMBCL presenting with a dominant anterior mediastinal mass. All patients had to have been treated with R-CHOP. Progression-free survival (PFS) and overall survival (OS) are calculated by the Kaplan-Meier method and univariate analysis is performed to assess predictors of outcome. Results: Fifty-eight cases from 2000–2011 met inclusion criteria and are included in the analysis. The median age was 38 years (range 20–82) and 60% were male. Forty-four patients (76%) presented at limited Ann Arbor stage and 12 patients (21%) at advanced stage; presenting stage could not be discerned in 2 patients. Fifty-five percent of patients presented with mediastinal bulk ≥10cm in size; median size was 11cm (range 5–17cm). LDH was elevated at diagnosis in 60% of patients, normal in 21%, and unknown in 19%. By revised IPI score, 19% were low-risk (0 risk factors), 60% were intermediate risk (1–2 risk factors) and 12% were high-risk (≥3 risk factors). R-CHOP was given for a median of 6 cycles (range 1–8); 51 of 58 patients received 6 or 8 cycles. Among patients who achieved initial remission, 78% underwent consolidative radiotherapy and the remainder were observed after chemotherapy alone. The overall response rate was 81% (90%CI [71%–89%]) with 72% complete responses and 9% partial responses. Ten patients (17%) had primary refractory disease defined as progression on treatment or within 3 months of completion of therapy. Among 46 patients who achieved a response, 5 (11%) subsequently relapsed. Two patients, both elderly, died during treatment. Among the 10 patients with primary refractory disease, 6 have died from progressive lymphoma, 2 patients are alive with active disease undergoing salvage therapy, 1 is alive and free of disease greater than 8 years from diagnosis, and 1 was lost to follow-up. Among 5 patients with relapsed disease, 2 are alive without disease at last follow-up, while 3 have died of progressive lymphoma. Median follow-up for the entire series is 58 months. Five-year PFS is 68% (95% CI, 55% to 80%) and 5-year OS is 76% (95% CI, 65% to 88%). On univariate analysis, advanced Ann Arbor stage and high R-IPI score were associated with inferior PFS and OS. (p=0.006 and p<0.001, respectively for PFS, p=0.005 and p<0.001 for OS, log-rank test). Conclusion: PMBCL treated with R-CHOP carries an overall favorable prognosis, though primary refractory disease occurs in a significant number of patients, and is rarely curable with second line therapy. Advanced stage disease and high R-IPI scores are associated with inferior outcome. Novel treatment approaches warrant evaluation in high-risk patients. Disclosures: No relevant conflicts of interest to declare.

Outcome of Nodular Lymphocyte Predominant Hodgkin Lymphoma Patients Treated with or without Rituximab

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 4878-4878
Author(s):  
Heidi Mocikova ◽  
Jozef Michalka ◽  
Jan Koren ◽  
Pavla Stepankova ◽  
Alexander Wild ◽  
...  
Keyword(s):  
B Cell ◽  
Conventional Treatment ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Line Treatment ◽  
First Line ◽  
Large B Cell Lymphoma ◽  
Large B Cell ◽  
First Line Treatment

Abstract Abstract 4878 Background. Strong CD20 expression in nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) suggests the feasibility of rituximab in the treatment of this disease. Methods. We analysed the outcome of 102 patients with NLPHL treated with or without rituximab in combination with conventional treatment: chemotherapy and/or radiotherapy. Histologies were reviewed for the purpose of this study. Rituximab was administered in 26 of 102 NLPHL patients (13 in the first line treatment and in 13 of 20 relapsed patients). Additionally, rituximab with chemotherapy was administered in 11 patients with histologic transformation to diffuse large-B cell lymphoma. Median follow-up was 7.1 years. Median patient age was 34.2 years. Results. The 10-year overall survival (OS) rate and progression - free survival (PFS) of the whole group was 88% and 65%, respectively. There was no difference in OS and PFS in patients with clinical stage IA without risk factors treated without or with rituximab (30 vs 3 patients) and conventional treatment, however the follow-up in the rituximab group was short. The addition of rituximab to conventional treatment did not affect the OS in the group of patients with more advanced disease: 58 patients without vs 10 with rituximab (94% [95% CI: 88 – 100%] vs 100% [-], P=0.566). PFS in both groups did not differ significantly in the first line treatment (69% [95% CI: 57 – 82%] vs 100% [-], P=0.165), however when all lines of treatment were analysed, PFS was significantly better in patients treated without rituximab (92% [95% CI: 84 – 100%] vs 38% [95% CI: 22 – 65%], P< 0.001). Histologic transformation to diffuse large B - cell lymphoma was diagnosed in 11 rituximab naive patients, but this was not statistically significant when compared to 0 patients after rituximab treatment (14,5% vs 0%, P=0.061). Histologic transformation was the only poor prognostic factor that influenced OS (HR 7.936, P=0.004). Conclusions. Rituximab does not prevent relapses in NLPHL. This study confirms favorable OS of NLPHL patients regardless whether rituximab was used or not. The absence of histologic transformation in NLPHL patients treated with rituximab deserves further investigation. Disclosures: No relevant conflicts of interest to declare.

Poor Outcomes Among Patients Failing Dose-Adjusted EPOCH in Aggressive Lymphoma: A Collaborative, Retrospective Study

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1518-1518 ◽  
Author(s):  
Jackie Vandermeer ◽  
Allison M Winter ◽  
Ajay K. Gopal ◽  
Ryan D. Cassaday ◽  
Brian T. Hill ◽  
...  
Keyword(s):  
B Cell ◽  
Board Of Directors ◽  
Salvage Therapy ◽  
Research Funding ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
First Line ◽  
Advisory Committees ◽  
First Line Therapy

Abstract Introduction Among patients with aggressive B-NHL who fail RCHOP, about half respond to standard salvage regimens and may proceed to curative-intent, transplant-based therapy. However, whether pts failing more intensive regimens such as dose-adjusted, infusional EPOCH benefit from standard salvage regimens is unclear. We hypothesized that such patients comprise a higher-risk cohort, facing inferior response rates and outcomes using standard salvage regimens. We undertook a collaborative study to assess response rates and survival among pts failing EPOCH for aggressive B-NHL, to inform patient management and design of clinical trials in this setting. Methods Pharmacy records and institutional databases were queried, identifying pts receiving EPOCH over the last 10 years at the University of Washington/SCCA and the Cleveland Clinic Foundation, for combined analysis. Under IRB approval, patient characteristics, histology, outcome with EPOCH, time to EPOCH failure, response to salvage, and overall survival were analyzed. Diffuse large B cell lymphoma (DLBCL), primary mediastinal B-cell lymphoma, B-cell-lymphoma unclassifiable, HIV-associated B cell lymphoma, and transformed B cell non-Hodgkin lymphoma were included. Pts receiving <2 cycles EPOCH, or who had inadequate follow-up (<3 months), were excluded. Failure of EPOCH was defined as failure to respond or progression during therapy, need for initiation of salvage therapy, or death during therapy of any cause. Adverse events or treatment change due to toxicity were not included in the definition of failure. JMP 11 was used to generate kaplan-meier survival estimates. Results 124 pts with aggressive B-NHL receiving EPOCH were identified. 54 had not relapsed, and among 70 remaining da-EPOCH failures, 37 met the above inclusion criteria. Median age was 55. 27% were female, and 23 received EPOCH as first-line therapy. All but 3 received rituximab with EPOCH. Histologies were primarily DLBCL in 22/37 (60%) and BCL-U in 12/37 (32%) carrying a MYC translocation; most of these harbored additional translocations in BCL2 and/or BCL6 (10/12). However, data regarding MYC rearrangement was not available for all pts. 2 had HIV-associated B-NHL and 3 had PMBCL. With 18 months follow up, the median time to EPOCH failure was 5 months. Only 3 EPOCH failures occurred late (>12 months). Median OS from the date of EPOCH failure was 10 months (Figure 1). Those receiving EPOCH as first-line therapy (23) had a median OS of 14 months from EPOCH failure, as opposed to 4 months for those receiving EPOCH as salvage therapy (log-rank p=.01). Salvage chemotherapy regimens after EPOCH were diverse, and generally ineffective; 6/28 (21%) regimens produced a response (Table 1). Among patients failing EPOCH within a year, platinum-containing salvage (RICE/RDHAP) was effective in only 2/13 patients (15%). 9 patients did not receive any salvage, most of whom died or proceeded to palliative measures and/or hospice care. Conclusions A relatively low overall response rate (21%) was observed in this retrospective analysis of patients failing EPOCH. Analogous to early RCHOP failure in the CORAL study, those failing EPOCH within a year may face inferior outcomes with platinum-based salvage therapy. While combined from two institutions, our data represent a modest sample size and require confirmation. If verified, examination of mechanisms of resistance to EPOCH, and selecting EPOCH failures for clinical trials of novel targeted therapies and transplant-based approaches, may prove critical. Table 1. Salvage Therapy for REPOCH failures Regimen: response/total number treated Notes Response to any salvage: 6/28 (21%) Some patients received more than 1 chemo salvage; responses were tabulated per regimen. RICE: 4/12 2/3 alive post transplant(1 auto 1 allo; 1 declined transplant and survived; 1 died) RDHAP: 1/6 Gemcitabine-based: 0/5 HyperCVAD (Part A and/or B): 1/5 Survivor had CNS only relapse, received regimen B and transplant 9- received no systemic treatmen; most died or proceeded to palliative measures and/or hospice Figure 1. Figure 1. Disclosures Gopal: Gilead: Consultancy, Research Funding; Pfizer: Consultancy, Research Funding; Spectrum: Consultancy, Research Funding; Emergent/Abbott: Research Funding; Sanofi-Aventis: Honoraria; Seattle Genetics: Consultancy, Honoraria; BioMarin: Research Funding; Piramal: Research Funding; Janssen: Consultancy; Millenium: Honoraria, Research Funding; BMS: Research Funding; Merck: Research Funding. Hill:Seattle Genetics: Honoraria, Membership on an entity's Board of Directors or advisory committees; Celgene: Honoraria, Membership on an entity's Board of Directors or advisory committees; Pfizer: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees; Janssen: Honoraria, Membership on an entity's Board of Directors or advisory committees. Till:Roche/Genentech: Research Funding; Pfizer: Research Funding.

Primary mediastinal large B-cell lymphoma: Treatment outcomes in 43 patients

2006 ◽  
Vol 24 (18_suppl) ◽  
pp. 17556-17556
Author(s):  
C. L. Mello ◽  
O. Feher ◽  
V. C. Lima ◽  
C. Valadares ◽  
F. A. Soares ◽  
...  
Keyword(s):  
B Cell ◽  
First Generation ◽  
Response Rate ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Third Generation ◽  
First Line ◽  
Large B Cell Lymphoma ◽  
Large B Cell

17556 Objectives: Primary mediastinal B-cell Lymphoma (PMBL) is recognized as a separate entity in the WHO classification. Treatment for PMBL is based on a combination of conventional dose chemotherapy, high dose chemotherapy and radiation therapy. The best strategy is still undefined. We conducted a retrospective analysis of patients with PBML to identify clinical prognostic factors. Methods: A retrospective analysis of 43 patients treated at Hospital do Cancer AC Camargo, Sao Paulo, Brazil, between 1989 and 2004. All patients had previous diagnosis of diffuse large B-cell lymphoma, with positive CD20 on neoplastic cells. A predominant anterior mediastinal lesion should be present. Induction chemotherapy regimens were grouped in first generation (CHOP/CHOP-like), third generation (PromaceCytabon/MACOP) and other (pediatric regimens, COP). Results: Age ranged from 16 to 82 years-old, 30 females and 13 male. Age < 35 yo was associated with a better prognosis (5 years OS - 56% × 34%, p = 0.048). Among clinical variables, female gender, stage IA-IIB, IPI 0–1, normal LDH, absence of mediastinal bulky disease were associated with better prognosis, although not statistically significant. Response rate to first generation regimens was: 37% CR (11/29), 24% PR (7/29) and 24% PD (7/29). Four patients were treated with Third generation regimens with 2 CR and 2 PR. 20 out of 25 patients with PR or CR to first line chemotherapy received mediastinal radiation therapy. More than 65% of patients had a follow up of 5 years or more. With a median follow up of 22.3 months, projected 5 year OS was 47% and for the responders the median PFS was 8,4 months. No difference in OS and PFS was observed among the three chemotherapy groups. Conclusion: Our analysis showed that response rate to first line regimens was around 60% and 25% of patients were primarily refractory to CHOP regimen. Age younger than 35 years old was associated with a better prognosis. 5 years overall survival was 45% and is in accordance with the literature. Although recent studies have demonstrated biological similarities between PMBL and Hodgkin’s Lymphoma, the prognosis of PMBL is less favorable than HL. Better understanding of the disease will help in developing more appropriate therapeutic strategies for PMBL. No significant financial relationships to disclose.

Chlamydia Psittaci-Eradicating Antibiotic Therapy as a Potential Therapeutic Strategy Against Marginal Zone B-Cell Lymphoma of the Ocular Adnexa.

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 3274-3274 ◽  
Author(s):  
Andres J. Ferreri ◽  
Maurilio Ponzoni ◽  
Massimo Guidoboni ◽  
Antonia A. Lettini ◽  
Laura Caggiari ◽  
...  
Keyword(s):  
Antibiotic Therapy ◽  
B Cell ◽  
Marginal Zone ◽  
Mononuclear Cells ◽  
Cell Lymphoma ◽  
Objective Response ◽  
B Cell Lymphoma ◽  
Chlamydia Psittaci ◽  
Ocular Adnexa

Abstract Background: Chlamydia psittaci (Ch.ps.) DNA has been detected in tumor tissue of 80% of patients with ocular adnexal lymphoma (OAL) and in peripheral blood mononuclear cells (PBMCs) of 40% of them [Ferreri AJM, et al. J Natl Cancer Inst 96:586, 2004]. Chronic antigenic stimulation provided by Ch.ps. persistent infection may favor the development and sustaining of OAL. Removal of this stimulation with antibiotic therapy could result in lymphoma regression. Aim: To assess the rate of Ch.ps. eradication and anti-lymphoma activity of antibiotic therapy in OAL patients. Methods: Twelve patients with Ch.ps.-positive marginal zone B-cell lymphoma of the ocular adnexa, at diagnosis (n=5) or relapse, were treated with doxycycline 100 mg, bid orally, for three weeks. The presence of Ch.ps. DNA in lymphoma samples was assessed by multiplex touchdown PCR. Specificity of the amplified PCR fragments was confirmed by direct sequencing of both sense and anti-sense strands. The presence of Ch.ps. DNA in PBMCs collected before and one and 12 months after antibiotic therapy was evaluated in 11 cases. Six of these patients had Ch.ps.-positive PBMCs and were assessable for bacterial eradication rate. Nine patients had measurable disease at the time of therapy and were evaluable for objective response; a lymphomatous lesion of ocular adnexa was detected in all 9 evaluable patients: unilateral in three, bilateral in three, associated with regional lymphadenopathies in two, and with multiple subcutaneous nodules in one. Objective response was assessed one, three and six months after therapy conclusion and every six months during follow-up. Observation period after doxycycline ranged from 1 to 29 months (median 25). Results: All patients but one completed antibiotic therapy, with excellent tolerability. At one month from doxycycline assumption, Ch.ps. DNA was no longer detectable in PBMC of the six positive patients; these results were confirmed at one year of fw-up in all the three assessable cases. Objective response was complete in two patients (26+ and 9+ months), partial in two (29+ and 6+ m.) and lower than 50% in two (3+ and 5+ m.), whereas one patient had stable disease at one month of fw-up. Two patients experienced progression and received salvage treatment. Importantly, two patients showed lymphoma regression in previously irradiated orbit, and the two patients with regional lymphadenopathies achieved complete response; doxycycline was the 4th-line treatment in one of them. Time to the best response ranged from 3 to 24 months. All patients are alive and well (median fw-up: 54 m.). Conclusions: Ch.ps.-eradicating antibiotic therapy is followed by tumor regression in OAL patients, even after multiple relapses. Estimation of response rate requires a longer follow-up. A large phase II trial is warranted to confirm whether this fast, cheap and well-tolerated therapy may be a valid alternative to conventional, more aggressive strategies against OAL.

Histologic Transformation in Marginal Zone Lymphomas

Blood ◽  
2012 ◽  
Vol 120 (21) ◽  
pp. 1571-1571
Author(s):  
Silvia Franceschetti ◽  
Annarita Conconi ◽  
Kathrin Aprile Von Hohenstaufen ◽  
Gloria Margiotta Casaluci ◽  
Anastasios Stathis ◽  
...  
Keyword(s):  
B Cell ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
Performance Status ◽  
B Cell Lymphoma ◽  
Lymphocytic Leukemia ◽  
High Dose ◽  
Off Label ◽  
Clinical Variables

Abstract Abstract 1571 Background. Information concerning histologic transformation (HT) of marginal zone lymphomas (MZL) into aggressive entities is scant. We retrospectively analyzed the clinical variables at diagnosis and outcome, with special reference to HT, in a population of consecutive patients (pts) with confirmed diagnosis of MZL, including extranodal MZL (MALT lymphoma), splenic MZL (SMZL) and nodal MZL (NMZL). Patients and methods. The database of the Oncology Institute of Southern Switzerland (IOSI, Bellinzona) and of the Hematology Division of the Amedeo Avogadro University of Eastern Piedmont (Novara) includes 373 cases of MZL diagnosed and treated since 1979 to 2012: 186 MALT lymphomas (50%), 88 SMZL (23%), 36 NMZL (10%). Sixty-three patients (17%) could not be properly classified (uMZL): they presented with bone marrow infiltration with or without detectable involvement of peripheral blood but without splenomegaly and with apparently no other extranodal or nodal involved site. Results. Incidence was not significantly different according to sex (male: 47; female: 53%), median age at diagnosis was 68 years (20–94 years); 244 pts (65%) had stage III-IV disease. LDH was elevated in 45/212 (21%) tested pts, beta2-microglobulin in 108/205 (53%) tested pts. B symptoms were reported in 27/368 pts (7%). Five percent of pts had an ECOG performance status higher than 1. Serologic evidence of hepatitis C virus (HCV) infection was reported in 45/243 (19%) pts for whom the data was available. Among the 186 MALT lymphomas, 91 pts (49%) had a gastric localization, and 54 (29%) had multiple extranodal sites of disease involvement. Median overall survival (OS) and progression-free survival of the whole population were 15 years and 8 years, respectively. After a median follow-up of 5 years, HT was observed in 14 cases (4%, 95%CI:2%-6%). A diagnosis of diffuse large B cell lymphoma was documented in 12 pts (85.7% of patients undergoing HT), while in two cases the diagnosis was of classical Hodgkin lymphoma and mantle cell lymphoma, respectively. HT occurred after a median interval of 3 years (range: 1–12 years) after diagnosis. With respect to MZL type, HT occurred in 6% SMZL, 4% MALT lymphomas, 3% NMZL, and 2% uMZL (P=0.635). Risk of HT was 4% (95%CI, 2–8%) at 5 years, 6% (95%CI:3%-11%) at 10 years and 9% (95%CI, 5–16%) at 15 years; the rate of transformation tended to plateau from that point onward. At the time of HT, most pts had high LDH serum levels (8/11, 73%) and presence of B symptoms (6/10, 60%). After transformation, nine pts received anthracycline-containing regimens, and four pts were treated with high dose cytarabine regimens; in a single patient only supportive measures were adopted. In four pts, autologous stem cell transplantation was performed after induction. At a median follow-up of 12 months after HT, five of 14 pts died, all for lymphoma-related causes, with a 2-year post-transformation survival rate of 38% (95%CI:5%-74%). There was no significant association between the risk of HT and any of the clinical variables at diagnosis or frontline treatment strategies. Conclusions. This large retrospective series documents that the risk of HT is low across all MZL types. The incidence of HT in MZL is apparently lower than that of other indolent B cell malignancies, namely follicular lymphoma (FL) and chronic lymphocytic leukemia (CLL). As also observed in FL and CLL, HT in MZL occurs relatively early during the clinical course, pointing to putative biological differences at diagnosis in MZL patients destined to transform. Disclosures: Off Label Use: Trial partially supported by a research grant by Celgene. Lenalidomide was provided free by Celgene. The use of Lenalidomide is off-label in untreated DLBCL. Bertoni:OncoEthix SA: Research Funding.

Lymphoma Patients Secondary to Congenital and Acquired Immunodeficiency Syndroms in a Turkish Pediatric Oncology Center

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 5445-5445
Author(s):  
Nurdan Tacyildiz ◽  
Gulsah Tanyildiz ◽  
Gulsan Yavuz ◽  
Emel Unal ◽  
Handan Dincaslan ◽  
...  
Keyword(s):  
T Cell ◽  
B Cell ◽  
Progressive Disease ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
First Line ◽  
First Line Therapy ◽  
Line Therapy ◽  
Lymphoproliferative Syndrome

Abstract PURPOSE An increased incidence of lymphoma is seen in various types of immune deficiency syndromes,including congenital immune deficiency diseases, organ transplantation with iatrogenic immunosuppression and autoimmune disorders. Prognosis of the lymphomas secondary to immunodeficiencies is stil poor. We aimed to analyse clinical features and treatment results of our patients that diagnosed as lymphoma and have immundeficiency syndrom. PATIENTS Between 2002-2014, we have seen 12 (7male, 5 female) childhood lymphoma that related immunodeficiencies. Ages of patients were between 4-15 years (median 8 years).The follow up period is 1-140 months (median: 38.5 months) and survival rate is %58. Five of patients died because of the progressive disease. The characteristics of patients are summarized in the table. TABLE- Clinical characteristics of patients Patient Age (year) Gender Diagnosis Follow up (months) Survival 1. G.C 10 Male T-NHL + AT 6 Alive (lost to follow up) 2. İ.D 4 Male T-NHL + ALPS 9 Eksitus 3. M.K 12 Female T cell rich B cell lymphoma+ CVID 2 Eksitus 4. S.K 9 Female B cell lymphoblastic lymphoma+AT 54 Alive 5. B.C 5 Male BL + Renal transplantation 1 Eksitus 6. S.K 7 Female BL + AT 6 Eksitus 7. C.G 12 Male BL + WAS 48 Eksitus 8. K.B 11 Female BL+EBV associated lymphoproliferative syndrome 29 Alive 9. M.Y 15 Female HL + CVID 140 Alive 10. B.Ç 4 Male HL + selective IgA deficiency 132 Alive 11. S.S 7 Male HL + AT 70 Alive 12. B.K 5 Male HL + AT 48 Alive TOTAL n = 12 4-15 years Median : 8 4 female 8 male 8 NHL (survival % 37.5) 4 HL (survival% 100) 1-140 months Median : 38.5 Survival %58 RESULTS Two of 5 Ataxia Telangiectasia (AT) patients diagnosed as Hodgkin's lymphoma (HL) while other three diagnosed as non-Hodgkin's lymphoma (NHL) (1 Burkitt's lymphoma-BL,1 B cell lymphoblastic lymphoma-BCLL,1 T-cell NHL). One of 2 common variable immunodeficiency (CVID) patient diagnosed as HL and the other one diagnosed T-cell rich B-cell lymphoma (TCRBCL). Wiscott-Aldrich syndome (WAS), autoimmune lymphoproliferative syndrome (ALPS ) and selective immunoglobulin A deficiency patients diagnosed as large B-cell lymphoma (LBCL), T-cell NHL and HL, respectively. In one patient, EBV associated BL developed secondary to renal transplantation. Another EBV associated BL patient has been diagnosed recently who has DNA instability defect. Follow-up period of patients were between 1-140 months (median 38.5 months). Almost half of the patients ( 42%) were diagnosed as BL,BCLL or TCRBCL. Although, survival of our patients for median 38.5 months is 58% (5 patients died with progressive disease) ,four of the 5 BL&TCRBCL patients have been died. Two patients who are living after BL and BCLL diagnosis in that group are treated with Rituximab as first line therapy. CONCLUSİON BL is most common lymphoma type in immundeficient lymphoma patients which may be subject for future research . Although special attention has been given to these patients, especially survival of BL lymphoma secondary to immunodeficiencies are poor. Special treatment modalities, like targeted terapies may be necessary as first line therapy to improve survival of these patients. Disclosures No relevant conflicts of interest to declare.

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