Splenic Marginal Zone Lymphoma in an East Mediterranean/Middle Eastern Population Cohort: Possible Association with Hepatitis B Instead of Hepatitis C Virus As an Etiologic and Prognostic Factor (ThREG-NHL01 Study)

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 5332-5332
Author(s):  
Tuncay Aslan ◽  
Evren Ozdemir ◽  
Aysegul Uner ◽  
Elif Gungor ◽  
Nevin Alayvaz Aslan ◽  
...  
Keyword(s):  
Overall Survival ◽  
Marginal Zone ◽  
Multivariate Analyses ◽  
Middle Eastern ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
East Mediterranean ◽  
Hbsag Positivity ◽  
Baseline Characteristics

Abstract Splenic marginal zone lymphoma (SMZL) is a rare disease. It constitutes less than 2% of lymphoid neoplasms. Chronic antigenic stimulation is frequently blamed in the pathogenesis of extranodal marginal zone lymphomas including SMZL. Chronic hepatitis C is frequently observed in SMZL patients. However these reports are largely from North America and Europe. Data from various countries with different hepatitis prevalence are lacking. In this multicenter cohort study we aimed to find out clinical characteristics of SMZL patients in Turkey including viral hepatitis status and treatment details. Data were gathered from voluntary centers under auspices of Hematolojik Onkoloji Dernegi using a SPSS database. Categorical and continuous data were expressed as ratio (%) and median (range) and they were compared by the Chi-square and Mann Whitney U tests, respectively. Survival analyses were computed by the Kaplan-Meier method. Overall survival (OS) was calculated from diagnosis to the date of mortality of any reason. Patients who did not die at last follow-up were censored at this time for OS computations. Parameters related to survival were investigated by Cox regression univariate and multivariate analyses. 66 patients were reported from 8 tertiary care hematology/oncology centers. The diagnosis of SMZL was established by local hematopathologists. Data on baseline clinical characteristics are presented in table 1. Median follow-up duration was 20.4 months (0.3-208) (23 months for surviving patients). 13 patients died during follow-up. Median OS was not reached. Estimated 6 year survival was 59.5% (Figure 1). Older age, no splenectomy during follow-up, <90 x 103 platelet count, lower albumin, higher lactate dehydrogenase, higher b2-microglobulin and HBsAg positivity were associated with increased risk of death. Age, albumin and HBsAg positivity remained significant in multivariate analysis (table 2). HCV positivity was not observed in any patient in this East Mediterranean/Middle Eastern cohort. However 12.1% HBsAg and 32% HBsAg and/or anti-HBcAg positivities are higher than expected for our population. This finding should be confirmed in a larger cohort. Risk factors associated with worse prognosis were generally similar to those reported in European/North American populations. However, HBsAg positivity has not been reported as a risk factor previously. Figure 1 Overall survival Figure 1. Overall survival Table 1 Baseline characteristics Table 1. Baseline characteristics Table 2 Univariate and multivariate analyses for survival Table 2. Univariate and multivariate analyses for survival Disclosures No relevant conflicts of interest to declare.

Rituximab, Either As Single Agent or in Combination, Is Superior to Other Treatments in Terms of Response Rate and Progression-Free Survival (PFS) in Patients with Splenic Marginal Zone Lymphoma (SMZL)

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 4986-4986 ◽  
Author(s):  
Ana Marin-Niebla ◽  
Monica Else ◽  
Fatima De la Cruz ◽  
Paul Batty ◽  
Eduardo Rios-Herranz ◽  
...  
Keyword(s):  
Overall Survival ◽  
Marginal Zone ◽  
Single Agent ◽  
Progression Free Survival ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Free Survival ◽  
Prior Therapy ◽  
Grade 3

Abstract Abstract 4986 Background. Splenic marginal zone lymphoma (SMZL) is an uncommon indolent B-cell non-Hodgkin's lymphoma usually presenting with marked splenomegaly and bone marrow (BM) and/or peripheral blood (PB) involvement. Although splenectomy has traditionally been the treatment of choice in symptomatic patients, systemic treatment is required in cases of widespread disease, high risk from surgery, or relapse after splenectomy. There is no standard first line treatment in SMZL, but the anti-CD20 monoclonal antibody Rituximab has shown encouraging results in SMZL, with sustained responses. Aims. To assess, retrospectively, response to treatment, toxicity profile, progression-free survival (PFS) and overall survival (OS) after treatment with rituximab in SMZL. Methods. Twenty-nine patients from two different centers, diagnosed with SMZL between 1982 and 2011, received one or more treatments with rituximab. Eighteen patients (62%) received rituximab alone and 20 (69%) combined with chemotherapy; thus 9 patients received each of these treatments sequentially, to improve response, with or without interim relapse. The median age at diagnosis was 62 years (range 37–89 years); the male:female ratio was 2:3; B symptoms were present in 12 patients; ECOG performance status was 0–2 in 28/29 patients. All presented with splenomegaly, with involvement of the BM in 27 patients, PB in 24, lymph nodes in 11 and extranodal involvement in 7 patients. Diagnosis was made according to the WHO 2008 classification by spleen histology (n=12), BM histology (n=19), PB morphology (n=12) and immunophenotype (n=13). Rituximab monotherapy was administered at 375 mg/m2/weekly x4 weeks. In combination with fludarabine-based regimens (n=14), or other regimens including CHOP (n= 6), rituximab was administered on day 1 of each cycle. Responses were assessed according to the Response Criteria Guidelines for SMZL (Matutes, Leukemia 2008). Toxicity was graded according to the CTCAE v3.0. The Fisher exact test was used to compare best responses between groups. Survival was estimated using the Kaplan-Meier method. Results. All patients responded to rituximab monotherapy and/or to a combination treatment. At least one complete response (CR) was achieved in 20/29 patients (69%). This compares with a CR in only 4/17 (24%) of these same patients after any prior therapy (p=0.005): 11 patients had received splenectomy, with or without chemotherapy and 6 had received chemotherapy only. There was no difference in the CR rate between rituximab monotherapy (71%) versus rituximab combinations (68%). The most common adverse event was grade 3–4 neutropenia (n=7, 24%). Two patients had grade 3–4 infections. Anemia (n=3) and thrombocytopenia (n=2) were grade 1–2 only. Four cases presented with histological transformation prior to rituximab, all achieving CR with rituximab combination therapy. After a median follow-up of 24 months from rituximab treatment (range 4–102 months), progression-free survival (PFS) has been evaluated in 28/29 patients (1 patient not assessed yet at the time of presenting these data). PFS was longer after rituximab (n=32 evaluable treatments), either alone or in combination, than it had been in these same patients after prior therapy (n=17 prior treatments in total) (p=0.002) (Fig 1). When we compared PFS between rituximab monotherapy (n=17) and rituximab combined with chemotherapy (n=15 combined treatments), single agent rituximab was superior to rituximab in combination (p=0.02) (Fig 2). The median overall survival was not yet reached. Survival at 2 years after rituximab was 100%. Only one death, due to lung cancer, occurred at 48 months follow-up. Conclusions. In patients with SMZL, the CR rate after rituximab, either alone or in combination with chemotherapy, was significantly better than the CR rate after other prior treatments in the same patients, with manageable toxicity. Although we observed no difference in the CR rate between rituximab monotherapy or combination therapy, PFS was longer in patients receiving rituximab as single agent. Therefore, the addition of chemotherapy did not appear to add any advantage over rituximab alone in our cohort. Rituximab, with or without splenectomy, should be considered in the therapeutic scenario of SMZL. Disclosures: No relevant conflicts of interest to declare.

Outcomes In Splenic Marginal Zone Lymphoma: Analysis Of 108 Patients Treated In British Columbia (BC)

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 4369-4369
Author(s):  
Katharine He Xing ◽  
Amrit Kahlon ◽  
Joseph M. Connors ◽  
Brian Skinnider ◽  
Randy D. Gascoyne ◽  
...  
Keyword(s):  
British Columbia ◽  
Hepatitis C ◽  
Research Funding ◽  
Marginal Zone ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Transformation Rate ◽  
Splenic Marginal Zone Lymphoma ◽  
First Line

Abstract Introduction Splenic marginal zone lymphoma (SMZL) is uncommon and accounts for less than 1% of all non-Hodgkin lymphomas. The optimal treatment for SMZL is unknown. We describe the outcome of 108 patients with SMZL treated in British Columbia. Methods All patients with SMZL diagnosed between 1985 and June 2012 were identified in the BC Cancer Agency Centre for Lymphoid Cancer and Lymphoma Pathology Databases. Overall survival (OS) was measured from time of diagnosis to death or last follow-up. Progression-free survival (PFS) was measured from the date of diagnosis to the date of lymphoma recurrence or transformation, or death. Time to transformation (TTT) was calculated from date of diagnosis to date of transformation to aggressive lymphoma. Results 108 patients were identified with a diagnosis of SMZL. Baseline patient characteristics: median age 67 years (range 30-88), male 41%, stage IV 98%, B symptoms 17%, performance status ≥2 22%, splenomegaly 93%, bone marrow involvement 93%, peripheral blood involvement 87%. Hepatitis C serology was positive in 5 of 60 patients with available data. As initial treatment, 53 underwent splenectomy (10 with chemotherapy), 38 chemotherapy alone (21 with a rituximab-containing regimen), 2 received antiviral therapy for hepatitis C, and 15 were observed. Of the 43 patients who had splenectomy alone, 9 subsequently received chemotherapy upon progression, 1 had excision for a soft tissue mass, and 4 transformed to diffuse large B cell lymphoma (DLBCL). Of the 38 who received chemotherapy first line, 6 subsequently received combined chemotherapy and splenectomy, 1 splenectomy alone, 4 chemotherapy alone, and 7 transformed to DLBCL. Neither of the 2 patients who received antivirals had further progression. With a median follow-up of 7 years (range 3 months to 18 years) for living patients, the 5 and 10 year OS were 65% and 48%, respectively. The 5 and 10 year PFS were 38% and 18%, respectively. The 5 year OS for patients who had a splenectomy as their first-line therapy compared to other treatments was 76% vs 53% (p=0.01); and the 5 year OS for patients who received chemotherapy alone as first-line compared to other treatments was 52% vs 72% (p=0.04). There was no difference in outcomes between those treated with rituximab containing chemotherapy as first line compared to other treatments (p=0.65). The 5 and 10 year PFS after first-line splenectomy were 52% and 18%, respectively. A total of 14 patients transformed to DLBCL with a median TTT of 3.2 years (range 6 months to 11.9 years). The 5, 10, 15 year rates of transformation were 9%, 21% and 35%, respectively. Conclusions Splenectomy remains a reasonable treatment option for patients with SMZL. Patients selected for splenectomy as initial management of symptomatic disease experience improved outcomes. The transformation rate in SMZL is similar to that of other indolent lymphomas. Disclosures: Connors: F Hoffmann-La Roche: Research Funding; Roche Canada: Research Funding. Skinnider:Roche Canada: Research Funding. Gascoyne:Roche Canada: Research Funding. Sehn:Roche Canada: Research Funding. Savage:Roche Canada: Research Funding. Slack:Roche Canada: Research Funding. Shenkier:Roche Canada: Research Funding. Klasa:Roche Canada: Research Funding. Gerrie:Roche Canada: Research Funding. Villa:Roche Canada: Research Funding.

Splenectomy Is Still a Valid Option For Splenic Marginal Zone Lymphoma

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5129-5129
Author(s):  
Alessandro Pulsoni ◽  
Pasqualina D'Urso ◽  
Gianna Maria D'Elia ◽  
Giorgia Annechini ◽  
Caterina Stefanizzi ◽  
...  
Keyword(s):  
Marginal Zone ◽  
Conflicts Of Interest ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Spleen Size ◽  
Second Line ◽  
Second Line Therapy ◽  
Line Therapy

Abstract Introduction Splenic marginal zone lymphoma (SMZL) is an indolent B-cell lymphoma characterized by splenomegaly, frequent moderate lymphocytosis with or without villous morphology and possible involvement of various organs, especially the bone marrow (BM). Diagnosis is classically based on the spleen histology, but it can be made on the BM biopsy, based on the typical intrasinusoidal cell infiltration pattern and immunohistochemistry. Different therapeutic options are available, but to date there are no conclusive comparative data. Patients and methods We retrospectively analyzed 83 consecutive patients with a diagnosis of SMZL observed at our Institution between 1999 and 2013. The diagnosis was based on the BM biopsy in 79 patients; the BM was negative in 4 patients. Diagnosis was histologically confirmed on the spleen in 27 patients who underwent splenectomy. Patients presented a median age of 72.5 years (range 38-84); 43 were males. The median spleen size at diagnosis was 145 mm, ranging from 100 to 300 mm. The majority of patients were stage IV at diagnosis for BM infiltration (95%); B symptoms were present in 4 of them (4.8%). Forty-two patients (50.6%) had a lymphocytosis at diagnosis and 13 (15.6%) presented an IPI score higher than 3. Thirty-five of them (42%) had a MZL BM infiltration superior to 30% of the total bone cellularity. Forty-two patients (50.6%) underwent a watch and wait policy (WW), while 41 (49.4%) were treated within 6 months from diagnosis, mainly because of symptomatic splenomegaly; in these patients, treatment consisted of splenectomy, chemotherapy or chemotherapy plus immunotherapy with Rituximab. The features of patients submitted to WW with respect to patients treated at diagnosis were comparable for the various parameters mentioned above, except for spleen size (higher in patients treated at presentation) and lymphocyte count (higher in patients who were observed). After a median follow-up of 64 months, the overall median survival was 96%. Among the 42 WW patients, 18 (42.8%) are still untreated after a median follow-up of 57.5 months, while 24 (57.2%) have required therapy; the median treatment-free interval in these patients was 25.5 months. Concerning the 41 patients who underwent treatment at diagnosis, after a median follow-up of 50 months, 13 (31.7%) have required a subsequent second-line treatment. The interval between first-line approach and re-treatment in patients treated at diagnosis was 30 months. Overall, 27 patients were treated with splenectomy only (either at diagnosis or after a WW period): only 6 of them (22%) had a subsequent progression after a median latency of 42 months; 26 were treated with chemotherapy alone (alkylating agents in the majority of them, combination therapy in a minority): 15 of them (60%) had a subsequent relapse or progression after a median of 9 months; 12 patients received a Rituximab-containing regimen: of these, only 2 (16%) have so far required a second-line therapy after 10 and 26 months respectively. Conclusions The WW policy is a valid option for asymptomatic patients: in these patients, after 4.5 years from diagnosis more than 40% is still untreated. In patients requiring treatment, splenectomy alone is followed in the majority of patients by a long period of good disease control: only 22% required a second-line therapy after 3.5 years. The addiction of Rituximab to chemotherapy seems to reduce the probability of relapse and to prolong the response duration. However, these preliminary data need to be confirmed by larger studies. Disclosures: No relevant conflicts of interest to declare.

Long-term follow-up analysis of 100 patients with splenic marginal zone lymphoma treated with splenectomy as first-line treatment

2014 ◽  
Vol 55 (8) ◽  
pp. 1854-1860 ◽  
Author(s):  
Julien Lenglet ◽  
Catherine Traullé ◽  
Nicolas Mounier ◽  
Claire Benet ◽  
Nicolas Munoz-Bongrand ◽  
...  
Keyword(s):  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Line Treatment ◽  
First Line ◽  
Long Term Follow Up ◽  
First Line Treatment

scholarly journals Splenic Marginal Zone Lymphoma: French Registries Population-Based Treatment and Survival Analyses (2002-2014)

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 1-1
Author(s):  
Marc Maynadie ◽  
Sophie Gauthier ◽  
Morgane Mounier ◽  
Côme Bommier ◽  
Sebastien Orazio ◽  
...  
Keyword(s):  
Stable Disease ◽  
Marginal Zone ◽  
Cell Lymphoma ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Second Line ◽  
First Line ◽  
Massive Splenomegaly

Background. Splenic Marginal Zone Lymphoma (SMZL) is a rare indolent B-cell lymphoma characterized by a massive splenomegaly and a moderate lymphocytosis. There is no standard of care for SMZL so far. The treatment is indicated if constitutional symptoms, massive splenomegaly and/or cytopenia, after a watch and wait period that is highly variable. Rituximab with or without chemotherapy, chemotherapy, and splenectomy are valid treatment approaches. Our objectives were to describe the characteristics of the patients with SMZL retrospectively collected from a French population database, to analyze the treatments received in first line (Tt1) and in second line (Tt2) in term of efficacy, and to describe their outcome. Methods. We extracted the patients with a diagnostic of SMZL from the 3 French specialized registries databases (Basse-Normandie, Gironde and Côte d'Or; 3,6 M inhabitants) according to ICD-O-3 classification, cases coded as 9689/3 included between 2002 and 2014, with a follow-up until January 1rst, 2018. Demographic and clinical variables including clinical presentation, morphology and treatments at the first line and the second line were collected and reviewed by hematological experts in lymphoma (CT,XT,MM). World population standardized (WSP) incidence rate, observed survival (OS), net survival (NS: disease specific survival) were estimated using STATA v15. Results. 284 patients met inclusion criteria. The WSP incidence rate was 0.30/100,000 p-y. Clinical characteristics were: median age of 72-y-old (31 - 93), male (n=148, 52%), Ann-Arbor Stage I-II (n=40, 15%), Stage IV (n= 230, 81 %), first malignancy (n=47, 17%), Hb &lt; 8g/dL (n=15, 5%), Plaq &lt; 80 G/L (n=38, 13%). A clonal B-cells blood involvement was present in 96% of cases with a RHM score of 0-2 in 84%. With a median follow-up at 7 years, the 5-year OS and NS were respectively 65% (59-70) and 74% (67-82). Among 283 pts with Tt1 information, 232 were actively treated (82%). In Tt1, a splenectomy alone was performed in 93 cases (40%); rituximab was used alone in 12 cases (5); 39 (17%) received chemotherapy alone and 65 (28%) a combination R-chemotherapy. 61% of patients were treated within 3 months from diagnosis, others had a 17 months median delay. A CR after Tt1 was obtained in 71 cases (31%), a partial response or stable disease were found in 86 cases (37%). Relapse or progression were observed in 32 cases (20%). The median TTNT was 29 months (m); being quite equivalent in patients treated with other treatment than in patients with splenectomy alone (17.3 m vs 16.4 m, p = 0.1). Among 221 cases with Tt2 information, 57 were actively treated (25%). A splenectomy alone was performed in 3 cases (5%); rituximab was used alone in 5 cases (9%); 7 (12%) received chemotherapy alone and 40 (70%) had R-Chemotherapy. A CR was obtained in 25 cases (44%), a PR or stable disease was found in 17 cases (30%). Relapse or progression were observed in 7 cases (17%). The median TTNT was 13 m. Transformation in diffuse large B-cell lymphoma was observed in 21 cases (1 in untreated patient, 16 after Tt1 and 4 afterTt2). The 5-y NS was significantly better in patients treated by splenectomy alone than in other patients (91% vs 72%, p=0.002). Conclusion: This study reports the real world data of SMZL from cancer registries over 12 years with a long follow-up. New drugs and strategies are in need to improve the results of conservative approaches in SMZL. Disclosures Thieblemont: Cellectis: Speakers Bureau; Roche, Amgen, Kyte Gilead, Celgene, Abbvie, Novartis, Cellectis: Honoraria, Membership on an entity's Board of Directors or advisory committees, Other: Travel support; Roche, Hospita: Research Funding. OffLabel Disclosure: Rituximab used in treated patients

Liver Infiltration In Splenic Marginal Zone Lymphoma: Prevalence and Prognostic Implication

Blood ◽  
2010 ◽  
Vol 116 (21) ◽  
pp. 4137-4137 ◽  
Author(s):  
Eva Domingo-Domenech ◽  
Vicente Romagosa ◽  
Eva González-Barca ◽  
Marta Rodriguez-Aliberas ◽  
Ana Oliveira ◽  
...  
Keyword(s):  
Overall Survival ◽  
Liver Biopsy ◽  
Marginal Zone ◽  
Conflicts Of Interest ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Prognostic Implication ◽  
Number Of Patients ◽  
Almost All ◽  
Baseline Demographic

Abstract Abstract 4137 Introduction: There are no studies in the literature about the frequency of liver infiltration by splenic marginal zone lymphoma (SMZL). In the last review published by the International Group of SMZL (Matutes E et al, Leukemia 2008;22:487) clinical liver infiltration seems to be infrequent and it is considered, as other organ involvement, to appear during the course of the disease and confers a bad prognosis. Objective: To investigate in a selected sample of patients with SMZL the prevalence of liver infiltration, as well as its possible prognostic implication in survival or transformation. Material and methods: In the majority of our SMZL patients who underwent a splenectomy between 1995 and 2001, a liver biopsy was realised during the surgical procedure in order to investigate hepatic infiltration by lymphoma. Results: Forty-eight patients with SMZL were diagnosed in our center during this period; of them, 19 were splenectomized being liver biopsy performed in 16 (only in those cases that gave informed consent). Splenectomy was performed at diagnosis in 11 patients (time from diagnosis to splenectomy < 3 months). Liver biopsy was positive for SMZL infiltration in 81.3% (13/16) of the cases. Baseline demographic and clinical characteristics of both groups of patients are shown in the following table; there were no significant differences between the groups. We neither find statistical significant differences in overall survival between both groups: mean overall survival for positive and negative liver biopsy patients was 116 ± SD 13 months and 74 ± 29 months, respectively (p=0.69). Conclusion: Histological hepatic infiltration is present in almost all SMZL patients when splenectomized. In our experience, this finding has no prognostic implication. Due to the small number of patients of this study, its significance is limited but to the best of our knowledge, it is the first report that investigates the prevalence of liver infiltration in SMZL and its impact in survival. Larger series are necessary to confirm our findings. Disclosures: No relevant conflicts of interest to declare.

Acquired Angioedema due to C1 Inhibitor Deficiency Preceding Splenic Marginal Zone Lymphoma: Further Insights from Clinical Practice

2020 ◽  
Vol 181 (12) ◽  
pp. 941-946
Author(s):  
Mariana Paes Leme Ferriani ◽  
Orlando Trevisan-Neto ◽  
Julia S. Costa ◽  
Janaina M.L. Melo ◽  
Adriana S. Moreno ◽  
...  
Keyword(s):  
Clinical Practice ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
C1 Inhibitor ◽  
C1 Inhibitor Deficiency ◽  
Acquired Angioedema ◽  
Risk Of Death ◽  
Increased Risk

<b><i>Background:</i></b> Acquired angioedema due to C1 inhibitor deficiency (AAE-C1-INH) is a very rare disease. In clinical practice, it may be difficult to differentiate AAE-C1-INH from hereditary angioedema due to C1-INH deficiency (HAE-C1-INH). In both conditions, patients are at an increased risk of death from asphyxiation due to upper airway obstruction. The association of AAE-C1-INH with lymphoproliferative and autoimmune diseases, and with presence of anti-C1-INH antibodies has been well documented, and treatment of the underlying condition may result in complete remission of angioedema. <b><i>Objectives:</i></b> To discuss the clinical evaluation, diagnosis, and treatment outcomes of AAE-C1-INH in the context of the care of 2 patients with recurrent isolated angioedema. <b><i>Methods:</i></b> Two patients were followed up prospectively at our clinic. Measurements of C3, C4, C1-INH, and C1q levels were carried out by nephelometry, and the functional activity of C1-INH was determined by a chromogenic assay. Hematological investigation included morphological and immunophenotyping analysis of peripheral blood, bone marrow, and spleen histopathology. Sequencing of the 8 exons and adjacent intronic regions of the <i>SERPING1</i> gene was performed using the Sanger method. <b><i>Results:</i></b> Two patients were diagnosed with AAE-C1-INH associated with splenic marginal zone lymphoma during follow-up. <b><i>Conclusions:</i></b> Close follow-up, including detailed clinical history, physical examination, and laboratory tests, of our patients with AAE-C1-INH was essential for the early diagnosis and successful treatment of the lymphoproliferative disease, leading to the resolution of the angioedema attacks.

scholarly journals Clinical features and possible prognostic factors in patients with Marginal Zone Lymphoma: Retrospective analysis from two centers

2021 ◽  
Author(s):  
Pınar Akyol ◽  
Abdulkerim Yıldız ◽  
Murat Albayrak ◽  
Murat Yıldırım ◽  
Mesut Tığlıoğlu ◽  
...  
Keyword(s):  
Overall Survival ◽  
Platelet Count ◽  
Marginal Zone ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Need For Treatment ◽  
Significant Difference ◽  
Hematological And Biochemical Parameters ◽  
Nodal Marginal Zone Lymphoma ◽  
The Impact

Abstract Objectives Marginal zone lymphoma accounts 5%-17% of all non-Hodgkin lymphomas and has an indolent clinical course. The parameters that predict prognosis and the need for treatment are still unclear. The aim of the current study was to examine the impact of parameters on the course of disease and the need for treatment in marginal zone lymphoma. Methods A retrospective study was conducted with marginal zone lymphoma patients in the two centres between 2010 and 2018. The demographic and disease characteristics, and also hematological and biochemical parameters at the time of diagnosis were examined. The effect of the parameters on overall survival and need for treatment were analyzed. Results During the follow-up, 25 patients required treatment and 15 patiens were followed up without treatment. Overall survival was significantly higher in patients with nodal marginal zone lymphoma than in extranodal and splenic marginal zone lymphoma patients. overall survival of patients who required treatment was 92.9 months while untreated patients was 58.4 months and there was no significant difference among the groups. The platelet count of untreated patients at the time of diagnosis were significantly higher than patients who received treatment. No significant relationship was found between any parameter and overall survival. Conclusions We demonstrated platelet count at the time of diagnosis as a predictive factor for future treatment need. It is an objective and simple blood test that may be helpful to predict the course of the disease although further studies are warranted.

scholarly journals Splenic Marginal Zone Lymphoma: Risk of Transformation, Clinico-Biological Features at Transformation and Prognosis

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 4015-4015
Author(s):  
Gabriela Bastidas ◽  
Silvia Bea ◽  
Alba Navarro ◽  
Eva Gine ◽  
Julio Delgado ◽  
...  
Keyword(s):  
Multivariate Analysis ◽  
Research Funding ◽  
Marginal Zone ◽  
B Cell Lymphoma ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Complex Karyotype ◽  
Biological Features ◽  
Histological Transformation

BACKGROUND: The clinical course of splenic marginal zone lymphoma (SMZL) is usually indolent, but development of transformation to aggressive lymphoma (SMZL-T) is seen in 10 to 15% of the cases. Risk factors to predict SMZL-T are poorly defined. AIM: The aims of our study were: 1.- to assess the risk of transformation in a large series of SMZL patients; 2.- to analyze the prognostic factors for this event and 3.- to analyze clinical and biological features of SMZL-T. PATIENT AND METHODS: We identified 84 SMZL diagnosed at the HCB between 1994 and 2017 and 15 of them (18%) had developed histological transformation (SMZL-T). In addition, we reviewed 21 SMZL with transformation referred to HCB from other centers. Median age at diagnosis of low-grade SMZL in the 84 patients was 63 years (range, 32-91), and male/female ratio was 34/50. Complex karyotype (CK) defined as ≥3 chromosomal aberrations by conventional cytogenetics was observed in 22% and del(7q) in 59% of cases. The risk distribution according to the Splenic Marginal Zone Lymphoma Study Group simplified score HPLL/ABC was: low: 34, intermediate: 51 and high: 15. After a median follow-up of 80 months (range, 1-265), 37 of 84 (44%) patients have died, with a median overall survival (OS) of 146 months. To assess the risk of transformation and to identify predictive factors for such event, we exclusively evaluated the cohort of 84 patients from HCB. RESULTS: Fifteen of the 84 patients in the HCB cohort (18%) developed histological transformation. The cumulative incidence of transformation at 60 months from diagnosis was 15% (95% CI: 7 - 23) (Figure). In univariate analysis, variables predicting transformation were anemia, lymphopenia, thrombocytopenia, hypoalbuminemia, CK and high-risk scores of Intergruppo Italiano Linfomi (IIL) and HPLL (p<0.05 for all variables). In addition, lack of response to front-line treatment, relapse, and an early relapse at 12 and 24 months from treatment (p<0.05 for all variables) predicted for a higher risk of transformation. In multivariate analysis, only CK maintained significance to predict transformation (HR 4.85 (CI 95%: 1.15-20.56) p=0.03). Main clinico-biological features of all 36 SMZL-T patients (including HCB patients and those referred from other centers) are described in the Table. Thirty-five patients transformed to diffuse large B-cell lymphoma (DLCBL) and 1 to Hodgkin lymphoma. Treatment at transformation is detailed in Table. Response was assessable in 34 cases: 21 (62%) achieved a complete response (CR), and 13 patients did not respond. Among responders, 11 continue in CR, 9 relapsed during the follow-up and 1 patient died from therapy related acute myeloid leukemia (AML) when still in CR. Twenty SMZL-T patients (56%) eventually died, 19 related to lymphoma and 1 from AML. The median survival of the 36 SMZL-T patients from the time of transformation (SFT) was 59 months; with 60-months SFT of 46% (95% CI: 28-64) (Figure). Variables at the time of transformation predicting survival were age >60 years, thrombocytopenia and renal impairment. In addition, patients not achieving a CR, those not receiving an ASCT, and those who relapsed had a poorer SFT (p<0.05 for all variables). In multivariate analysis the independent variables predicting a worse SFT were: age > 60 years (HR 6.28 (95% CI: 1.55-25.39), p=0.01), thrombocytopenia (HR 32.77 (95% CI: 1.97-546.06), p=0.01), and response < CR (HR 8.75 (95% CI: 2.76-27.72), p<0.0001). Finally, SMZL-T was analyzed as a time-dependent variable for OS, showing that patients who eventually developed transformation had a significant higher risk of death (HR 3.894 (95% IC: 1.816-8.350), p<0.001). CONCLUSION: The risk of SMZL-T in our series of HCB was 15% at 60 months. The only factor that significatively predicted risk of SMZL-T in a multivariant analysis was the presence of complex karyotype. SMZL-T patients had a significative shorter survival compared to not transformed patients. Disclosures Gine: Roche: Other: Travel expenses, Research Funding; Gilead: Other: Travel expenses, Research Funding; Janssen: Other: Travel expenses, Research Funding. Lopez-Guillermo:Celgene: Consultancy, Research Funding; Gilead: Consultancy, Research Funding; Roche: Consultancy, Research Funding; Janssen: Research Funding.

Assessment of the Frequency of Additional Malignancies in Patients with Splenic Marginal Zone Lymphoma (SMZL).

Blood ◽  
2004 ◽  
Vol 104 (11) ◽  
pp. 4536-4536 ◽  
Author(s):  
Emilio Iannitto ◽  
Stefano De Cantis ◽  
Viviana Minardi ◽  
Vincenzo Callea ◽  
Giuseppina Calvaruso ◽  
...  
Keyword(s):  
General Population ◽  
Marginal Zone ◽  
The Elderly ◽  
Incidence Rates ◽  
Late Complications ◽  
Marginal Zone Lymphoma ◽  
Splenic Marginal Zone Lymphoma ◽  
Second Malignancies ◽  
Epithelial Tumor

Abstract Splenic marginal zone lymphoma (SMZL) is an indolent neoplasm, mostly occurring in the elderly, with a median survival time exceeding 10 years. The incidence of secondary cancer and late complications related to therapy have not been investigated. To assess the frequency of additional neoplasms in a series of consecutive SMZL patients and to estimate the corresponding risk of second cancers compared with that of the general population. we investigated the incidence of additional cancers in 129 patients diagnosed with SMZL in three italian hematological centers. With a median follow-up time of 33.2 months, twelve additional invasive cancers were recorded (9.3%). The 3 and 5-year cumulative incidence rates of second malignancy were 5.7% and 19.4%, respectively. Five more patients (3.8%) had a diagnosis of malignant epithelial tumor prior to that of SMZL. The incidence of second malignancies was slightly, but significantly, higher than the expected incidence rate. The Observed-to-Expected ratio (O/E) was 12/5.92=2.03, 95% confidence interval (CI) 1.05 to 3.56; p <0.05. Of the twelve second malignancies observed, four were urologic neoplasms ( O/E=4/1,08=3.70 (95% CI:1.01 to 9.48; p <0.05), four were lung cancers (O/E=4/0.44365=9.16; 95% CI: 1.41 to 13.25; p <0.05) and the other four diagnosis were of hepatic carcinoma (1), endometrial cancer (1), breast cancer (1), and colo-rectal cancer (1). Our findings evidence an increased frequency of additional neoplasms in patients with SMZL and suggest that this figure is significantly different than that expected in the general population. The incidence of cases with urinary tract and lung malignancies in our series is higher than expected. Although confirmatory data are needed, in our opinion SMZL patients are at risk of secondary maligancies and should be carefully investigated at diagnosis and monitored during the follow-up.

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