Still a Role of Splenectomy as First-Line Therapy of Splenic Marginal Zone Lymphoma?

Relevance. Follicular lymphoma (FL) and marginal zone lymphoma (MZL) are the most common variants of indolent non-Hodgkin lymphoma (iNHL). Despite the adequate choice of the first-line therapy, 10 15% of affected patients with iNHL develop refractory disease, and in most of the cases the relapse of the disease among the others patients is detected, and each subsequent remission is shorter than the previous. According to the data of the National LymphoCare Study Group, the relapses that can occur during the first 2 years (POD24) after the beginning of the first-line therapy with R-CHOP in patients with FL, as well as with MZL, have a negative impact on the prognosis: 5-year overall survival in these patients decreases from 90 to 50%. The arsenal of therapy options is actively expanding along with a deep understanding of the biological basis of the disease development. At present, the most topical problem remains the choice of the best approach for each patient the personalization of the therapy. The review includes the data from clinical trials concerning FL and MZL treatment presented at the Congress of the American Society of Hematology in 2020. There are a large number of studies concerning new anticancer drugs in the world, such as monoclonal antibodies to different targets on the surface of B-cells, macrophages, bispecific antibodies, antibody conjugates, immunomodulators, BCR signaling pathway inhibitors (Bruton tyrosine kinase, PI3K inhibitors), immune checkpoint inhibitors and many others. Conclusion. The development of weighting approach for the choice of therapy will give a chance to patients with FL and MZL to stay alive up to the next era of new effective anticancer drugs. Future strategies, according to the current studies, show the trend away from the cytotoxic chemotherapy in iNHL therapy.

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A case report of nodal marginal zone lymphoma: Diagnosis and management

Asian Journal of Oncology ◽

10.4103/asjo.asjo_80_16 ◽

2017 ◽

Vol 03 (01) ◽

pp. 078-080

Author(s):

Dilip Nikam

Keyword(s):

Marginal Zone ◽

Cell Lymphoma ◽

B Cell Lymphoma ◽

Marginal Zone Lymphoma ◽

First Line ◽

Non Hodgkin Lymphoma ◽

First Line Therapy ◽

Line Therapy ◽

Asymptomatic Patients ◽

Nodal Marginal Zone Lymphoma

AbstractNodal marginal zone B-cell lymphoma (NMZL) is a rare subtype of non-Hodgkin lymphoma, usually presented at advanced stage. The treatment for NMZL is currently consistent with follicular lymphoma which includes observation for asymptomatic patients to cyclophosphamide, doxorubicin, vincristine, prednisone (CHOP) or CHOP plus rituximab regimen, fludarabine or bendamustine and rituximab (B-R) for advanced, symptomatic patients. The B-R regimen is effective and could be evaluated further as the first-line therapy.

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The prognostic role of baseline CEA and CA 19-9 values and their time-dependent variations in advanced colorectal cancer patients submitted to first-line therapy

Tumor Biology ◽

10.1007/s13277-014-2693-3 ◽

2014 ◽

Vol 36 (3) ◽

pp. 1519-1527 ◽

Cited By ~ 12

Author(s):

M. Tampellini ◽

A. Ottone ◽

I. Alabiso ◽

C. Baratelli ◽

L. Forti ◽

...

Keyword(s):

Colorectal Cancer ◽

Cancer Patients ◽

Advanced Colorectal Cancer ◽

Time Dependent ◽

Prognostic Role ◽

First Line ◽

First Line Therapy ◽

Line Therapy ◽

Colorectal Cancer Patients

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Practical Aspects of Interface Application in CPAP Treatment

Canadian Respiratory Journal ◽

10.1155/2019/7215258 ◽

2019 ◽

Vol 2019 ◽

pp. 1-8 ◽

Cited By ~ 2

Author(s):

Adel Bachour ◽

Heidi Avellan-Hietanen ◽

Tuula Palotie ◽

Paula Virkkula

Keyword(s):

Airway Pressure ◽

Positive Airway Pressure ◽

Medical Team ◽

Cpap Therapy ◽

Air Leak ◽

First Line ◽

First Line Therapy ◽

Line Therapy ◽

Nasal Stuffiness

While continuous positive airway pressure (CPAP) is an effective first-line therapy for sleep apnea, CPAP fails in one third of patients mainly due to poor adherence to the CPAP device and masks. The role of the medical team is to guide the patient in choosing the best mask, thus insuring good CPAP therapy adherence. Once a suitable mask is found, the brand of the mask does not affect patient satisfaction or CPAP adherence. For the majority of patients, nasal masks are by far more suitable than oronasal masks. Orosanal masks are indicated in case of nasal stuffiness or when an air leak manifests through the mouth. Re-evaluation of the efficacy of CPAP therapy is recommended when switching to oronasal masks.

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

Blood ◽

10.1182/blood.v122.21.4369.4369 ◽

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.

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The Role Of 18F-FDG PET/CT For Staging and Response Assessment To Chemotherapy In Marginal Zone Lymphoma.

Blood ◽

10.1182/blood.v122.21.4328.4328 ◽

2013 ◽

Vol 122 (21) ◽

pp. 4328-4328

Author(s):

Marta Ruiz Mercado ◽

Estrella Carrillo Cruz ◽

Fátima de la Cruz Vicente ◽

Víctor A Marín-Oyaga ◽

María Solé Rodríguez ◽

...

Keyword(s):

Marginal Zone ◽

Response Assessment ◽

Marginal Zone Lymphoma ◽

First Line ◽

Pet Ct ◽

18F Fdg ◽

Fdg Pet Ct ◽

Residual Lesions ◽

Fdg Avidity

Abstract Introduction Marginal Zone Lymphoma (MZL) is an indolent lymphoma in which the use of PET/CT is poorly explored and also controversial due to the heterogeneous 18F- FDG avidity described in these types of lymphoma. Our aim in the present study is to evaluate the role of 18F-FDG-PET/ CT (PET/CT) in comparison to CT with intravenous contrast enhancement at initial staging and response assessment to chemotherapy in these patients. Methods and Materials A retrospective single-center study that included 34 patients, diagnosed of MZL between 1998 and 2012, with at least one PET/CT available. A total of 55 PET/ CT were performed: 25 at initial diagnosis, 19 for first- line response assessment, 6 in relapse and 5 after relapse- treatment. Locations of involved areas were registered comparing staging CT and PET/CT and were classified as discrepancy or not. Results Patients´ baseline characteristics are shown in table 1. At diagnosis, all patients presented with at least one abnormal focal FDG uptake except for one, which reflected a sensitivity of 96%. Median SUVmax was higher in nodal marginal zone lymphoma (NMZL) and extranodal marginal zone lymphoma (ENMZL): 6,1 (4- 8,4) and 6,9 (2- 13,8) respectively, in comparison to splenic marginal zone lymphoma (SMZL) 3,4 (3,2- 3,6) p=0,3. SUVmax was much higher in a patient with histological transformation to a DLBCL (SUVmax 37). Among 17 patients with both radiological imaging at the time of diagnosis, there were 8 patients (47%) with more involved areas demonstrated by PET/ CT than by CT alone, 75% of them were extranodal lesions. PET/CT upstaged 5 patients but in only 2 of them entailed a change in therapeutic management. Four patients did not show FDG avidity by PET/CT in some areas suspected to be involved by CT, what generated a CT sensitivity of 76%. Overall, CR was attained in 24 patients (66%) with 5-y OS 78%. Among 19 patients with a PET/CT available for first-line response assessment, responses were: 12 CR, 2 PR, 1PD and isolated residual lesions in 4 patients. Progression was documented in 2 of the 4 patients with residual lesions which were considered positive, and in 2 patients who maintained remission, the image was interpreted as a false positive (FP). The response assessment was performed by both radiological imaging in 13 patients. Discrepancies were found in 4 cases: CT showed CR in 3 patients while PET/CT detected localized residual disease and in another patient, CT showed stable disease whereas PET/CT demonstrated CR. Overall, none of the patients in CR by PET/CT relapsed. Five-year OS was 100% in contrast to 64% for those patients with a positive PET/CT after completing treatment (p=0,2), with a mean follow-up of 50 months (10-152). The NPV was 100% and PPV was 71% (5/7). Relapse was detected in 9 patients (37.5%). Six patients had PET/CT for re-staging and 5 for response assessment. All re-staging PET/CT had FDG-avidity with a median SUVmax of 9.9 (4.6-17.2). PET/CT for response after salvage treatment demonstrated 3 CR and 2 localized residual lesions. The NPV and PPV was 100% and 50%, respectively. Conclusions MZL shows higher 18F- FDG avidity in NMZL y ENMZL subtypes. PET/CT is more sensitive than CT at initial staging, chiefly in identifying extranodal involvement. Response assessment PET/CT had a NPV of 100% and PPV of 71 and 50% after first and second-line treatment, respectively. Disclosures: No relevant conflicts of interest to declare.

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Splenectomy Is Still a Valid Option For Splenic Marginal Zone Lymphoma

Blood ◽

10.1182/blood.v122.21.5129.5129 ◽

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.

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