EHA Library - The official digital education library of European Hematology Association (EHA)

EPIDEMIOLOGY, CHARACTERIZATION AND THERAPEUTIC MANAGEMENT OF MARGINAL ZONE LYMPHOMA: A SINGLE-CENTER EXPERIENCE
Author(s):
Carlos De Miguel
,
Carlos De Miguel
Affiliations:
Hematology,Hospital Universitario Puerta de Hierro Majadahonda,Majadahonda,Spain
Belén Navarro
,
Belén Navarro
Affiliations:
Hematology,Hospital Universitario Puerta de Hierro Majadahonda,Majadahonda,Spain
Javier Martín
,
Javier Martín
Affiliations:
Pathology,Hospital Universitario Puerta de Hierro Majadahonda,Majadahonda,Spain
Paloma Martín
,
Paloma Martín
Affiliations:
Pathology,Hospital Universitario Puerta de Hierro Majadahonda,Majadahonda,Spain
Rafael Forés
,
Rafael Forés
Affiliations:
Hematology,Hospital Universitario Puerta de Hierro Majadahonda,Majadahonda,Spain
Carmen Bellas
,
Carmen Bellas
Affiliations:
Pathology,Hospital Universitario Puerta de Hierro Majadahonda,Majadahonda,Spain
José Rafael Cabrera
José Rafael Cabrera
Affiliations:
Hematology,Hospital Universitario Puerta de Hierro Majadahonda,Majadahonda,Spain
(Abstract release date: 05/18/17) EHA Library. de Miguel Jiménez C. 05/18/17; 182599; PB1885
Dr. Carlos de Miguel Jiménez
Dr. Carlos de Miguel Jiménez
Contributions
PDF Abstract
Abstract

Abstract: PB1885

Type: Publication Only

Background
Marginal zone lymphomas are a group of relatively uncommon lymphomas whose cells are derived from B lymphocytes of the 'marginal zone' of the secondary lymphoid follicles.

Aims
The objective of this study is to review our series evaluating the epidemiology, clinical presentation, morphological, immunohistochemical and molecular characterization and therapeutic management in a tertiary hospital.

Methods
We evaluated a total of 56 patients diagnosed between May 2008 and February 2017. We collected the epidemiological and clinical data, including location, clinical stage, FLIPI and associated risk, antigenic stimulus, symptomatology at diagnosis (ECOG, constitutional syndrome, B symptoms) and response to treatment. We reviewed the levels of LDH, beta2microglobulin, ESR, peripheral blood (PB) immunophenotype and studied the morphological, immunohistochemical and molecular characteristics (MALT1 translocation and immunoglobulin heavy chain rearrangement (CDR2 / CDR3 of IgH)) in PB, bone marrow and affected organs. All diagnoses were classified according to WHO (2016 revision). In addition, we performed an autoimmunity test in most patients.

Results
Among the 56 patients, 26 were men (46.4%) and 30 women. The median age at diagnosis was 64 years (37-92). The most frequent subtype was the splenic marginal zone lymphoma (17 patients, 30.4%), followed by MALT: 10 pulmonary (17.9%), 10 gastric (17.9%), 5 cutaneous (8.9%), 5 ORL (8.9%), 2 (3.6%), 1 hepatic, 1 thyroid and 1 lacrimal gland (1.8%) and nodal marginal zone lymphoma (3 patients, 5.4%). Five of them presented with multifocal disease (8.9%). Fifty percent (28) had a clinical stage III / IV and 32 patients (57.1%) had a low risk at diagnosis (FLIPI 0-1).

We found an antigenic stimulus in 11 patients (Helicobacter pylori, Sjögren's syndrome, Hashimoto's thyroiditis). The molecular study of MALT1 was performed in 25 patients and 3 presented the translocation (12%). Six of seventeen cases (35.3%) showed IgH rearrangements. Antinuclear antibodies were positive in 15 of 32 patients (46.9%).
A total of 46 patients (82.1%) required a treatment, of whom 35 achieved a complete remission (CR) (76.1%) and 10 partial remission (PR) (21.7%) after the first line of treatment. Among these, 17 received immunochemotherapy (37%), 10 immunotherapy (21.7%), 8 surgery (17.4%), 7 antibiotics (15.2%) and 4 radiotherapy (8.7%). We observed 7 relapses (16.7%) and 3 progressions (7.1%), achieving a CR in 7 (70%) and PR in 3 (30%) after rescue treatment. There was just one case of high grade transformation (1.8%), who was the only patient decesed in the series (1.8%), with a median follow-up of 70 months.

Conclusion
Marginal zone lymphoma is an indolent lymphoma, with a good prognosis and very good response to current therapy. It is sometimes associated with autoimmune phenomena and infectious agents. It is essential a correct staging and characterization to optimize its therapeutic management and outcome.

Session topic: 19. Indolent Non-Hodgkin lymphoma - Clinical

Keyword(s): Marginal zone, MALT lymphoma

Abstract: PB1885

Type: Publication Only

Background
Marginal zone lymphomas are a group of relatively uncommon lymphomas whose cells are derived from B lymphocytes of the 'marginal zone' of the secondary lymphoid follicles.

Aims
The objective of this study is to review our series evaluating the epidemiology, clinical presentation, morphological, immunohistochemical and molecular characterization and therapeutic management in a tertiary hospital.

Methods
We evaluated a total of 56 patients diagnosed between May 2008 and February 2017. We collected the epidemiological and clinical data, including location, clinical stage, FLIPI and associated risk, antigenic stimulus, symptomatology at diagnosis (ECOG, constitutional syndrome, B symptoms) and response to treatment. We reviewed the levels of LDH, beta2microglobulin, ESR, peripheral blood (PB) immunophenotype and studied the morphological, immunohistochemical and molecular characteristics (MALT1 translocation and immunoglobulin heavy chain rearrangement (CDR2 / CDR3 of IgH)) in PB, bone marrow and affected organs. All diagnoses were classified according to WHO (2016 revision). In addition, we performed an autoimmunity test in most patients.

Results
Among the 56 patients, 26 were men (46.4%) and 30 women. The median age at diagnosis was 64 years (37-92). The most frequent subtype was the splenic marginal zone lymphoma (17 patients, 30.4%), followed by MALT: 10 pulmonary (17.9%), 10 gastric (17.9%), 5 cutaneous (8.9%), 5 ORL (8.9%), 2 (3.6%), 1 hepatic, 1 thyroid and 1 lacrimal gland (1.8%) and nodal marginal zone lymphoma (3 patients, 5.4%). Five of them presented with multifocal disease (8.9%). Fifty percent (28) had a clinical stage III / IV and 32 patients (57.1%) had a low risk at diagnosis (FLIPI 0-1).

We found an antigenic stimulus in 11 patients (Helicobacter pylori, Sjögren's syndrome, Hashimoto's thyroiditis). The molecular study of MALT1 was performed in 25 patients and 3 presented the translocation (12%). Six of seventeen cases (35.3%) showed IgH rearrangements. Antinuclear antibodies were positive in 15 of 32 patients (46.9%).
A total of 46 patients (82.1%) required a treatment, of whom 35 achieved a complete remission (CR) (76.1%) and 10 partial remission (PR) (21.7%) after the first line of treatment. Among these, 17 received immunochemotherapy (37%), 10 immunotherapy (21.7%), 8 surgery (17.4%), 7 antibiotics (15.2%) and 4 radiotherapy (8.7%). We observed 7 relapses (16.7%) and 3 progressions (7.1%), achieving a CR in 7 (70%) and PR in 3 (30%) after rescue treatment. There was just one case of high grade transformation (1.8%), who was the only patient decesed in the series (1.8%), with a median follow-up of 70 months.

Conclusion
Marginal zone lymphoma is an indolent lymphoma, with a good prognosis and very good response to current therapy. It is sometimes associated with autoimmune phenomena and infectious agents. It is essential a correct staging and characterization to optimize its therapeutic management and outcome.

Session topic: 19. Indolent Non-Hodgkin lymphoma - Clinical

Keyword(s): Marginal zone, MALT lymphoma

Premium Sponsors

What's new at EHA

Browse the open-source material from EHA past Congresses and meetings.

References

EHA Terms & Conditions
2025 ©
European Hematology Association
USER TERMS AND CONDITIONS / PRIVACY POLICY
(Amended according to GDPR)

By clicking “Accept Terms & all Cookies” or by continuing to browse, you agree to the storing of third-party cookies on your device to enhance your user experience and agree to the user terms and conditions of this learning management system (LMS).

Cookie Settings
Accept Terms & all Cookies