Splenic and Nodal Marginal Zone Lymphomas Are Indolent Disorders at High Hepatitis C Virus Seroprevalence with Distinct Presenting Features but Similar Morphologic and Phenotypic Profiles

Luca Arcaini, Marco Paulli, Emanuela Boveri, Daniele Vallisa, Patrizia Bernuzzi, Ester Orlandi, Paolo Incardona, Ercole Brusamolino, Francesco Passamonti, Sara Burcheri, Claudio Schena, Cristiana Pascutto, Luigi Cavanna, Umberto Magrini, Mario Lazzarino

Research output: Contribution to journalArticle

Abstract

BACKGROUND. Splenic and nodal marginal zone lymphomas (MZL) are subtypes of marginal zone-derived neoplasms. Due to their rarity, little is known concerning their relation, pattern of dissemination, and treatment outcome. METHODS. The authors analyzed the clinicopathologic features and outcome of 43 patients (34 patients with splenic MZL and 9 patients with nodal MZL). All lesional tissues obtained at diagnosis were reviewed histologically. RESULTS. Among the patients with splenic MZL, 30 patients had Stage IV disease (based on the Ann Arbor staging system). Twenty-six patients presented with splenomegaly with or without limited involvement of abdominal lymph nodes, whereas 7 patients showed disease extension to superficial lymph nodes. Hepatitis C virus (HCV) serology was positive in 35% of patients. Seventeen patients underwent splenectomy, 8 patients received chemotherapy, and 7 patients were followed without initial treatment. Interferon produced a lymphoma response in three of four HCV positive patients. Of 27 treated patients, 13 patients achieved a complete response, and 12 patients achieved a partial response. The median event-free survival (EFS) was 3.3 years (5.1 years for patients with disease confined to the abdomen and 2.1 years for patients with disease extension to superficial lymph nodes). Among nine patients with nodal MZL, four patients had Stage IV disease. HCV serology was positive in two patients. Five patients responded to chemotherapy. The median EFS was 2.8 years. The median overall survival was not reached for patients with both types of MZL. CONCLUSIONS. The results of the current study demonstrated that splenic and nodal MZL are indolent lymphomas with different presenting features but common morphologic and biologic characteristics, including high HCV seroprevalence. Studies will be required to identify specific biologic markers and to define the best treatment.

Original languageEnglish
Pages (from-to)107-115
Number of pages9
JournalCancer
Volume100
Issue number1
DOIs
Publication statusPublished - Jan 1 2004

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Seroepidemiologic Studies
Hepacivirus
Lymphoma
Lymph Nodes
Serology
Disease-Free Survival
Drug Therapy

Keywords

  • Low-grade non-Hodgkin lymphoma
  • Marginal zone
  • Nodal marginal zone lymphoma
  • Splenic marginal zone lymphoma

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

Splenic and Nodal Marginal Zone Lymphomas Are Indolent Disorders at High Hepatitis C Virus Seroprevalence with Distinct Presenting Features but Similar Morphologic and Phenotypic Profiles. / Arcaini, Luca; Paulli, Marco; Boveri, Emanuela; Vallisa, Daniele; Bernuzzi, Patrizia; Orlandi, Ester; Incardona, Paolo; Brusamolino, Ercole; Passamonti, Francesco; Burcheri, Sara; Schena, Claudio; Pascutto, Cristiana; Cavanna, Luigi; Magrini, Umberto; Lazzarino, Mario.

In: Cancer, Vol. 100, No. 1, 01.01.2004, p. 107-115.

Research output: Contribution to journalArticle

Arcaini, L, Paulli, M, Boveri, E, Vallisa, D, Bernuzzi, P, Orlandi, E, Incardona, P, Brusamolino, E, Passamonti, F, Burcheri, S, Schena, C, Pascutto, C, Cavanna, L, Magrini, U & Lazzarino, M 2004, 'Splenic and Nodal Marginal Zone Lymphomas Are Indolent Disorders at High Hepatitis C Virus Seroprevalence with Distinct Presenting Features but Similar Morphologic and Phenotypic Profiles', Cancer, vol. 100, no. 1, pp. 107-115. https://doi.org/10.1002/cncr.11893
Arcaini, Luca ; Paulli, Marco ; Boveri, Emanuela ; Vallisa, Daniele ; Bernuzzi, Patrizia ; Orlandi, Ester ; Incardona, Paolo ; Brusamolino, Ercole ; Passamonti, Francesco ; Burcheri, Sara ; Schena, Claudio ; Pascutto, Cristiana ; Cavanna, Luigi ; Magrini, Umberto ; Lazzarino, Mario. / Splenic and Nodal Marginal Zone Lymphomas Are Indolent Disorders at High Hepatitis C Virus Seroprevalence with Distinct Presenting Features but Similar Morphologic and Phenotypic Profiles. In: Cancer. 2004 ; Vol. 100, No. 1. pp. 107-115.
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abstract = "BACKGROUND. Splenic and nodal marginal zone lymphomas (MZL) are subtypes of marginal zone-derived neoplasms. Due to their rarity, little is known concerning their relation, pattern of dissemination, and treatment outcome. METHODS. The authors analyzed the clinicopathologic features and outcome of 43 patients (34 patients with splenic MZL and 9 patients with nodal MZL). All lesional tissues obtained at diagnosis were reviewed histologically. RESULTS. Among the patients with splenic MZL, 30 patients had Stage IV disease (based on the Ann Arbor staging system). Twenty-six patients presented with splenomegaly with or without limited involvement of abdominal lymph nodes, whereas 7 patients showed disease extension to superficial lymph nodes. Hepatitis C virus (HCV) serology was positive in 35{\%} of patients. Seventeen patients underwent splenectomy, 8 patients received chemotherapy, and 7 patients were followed without initial treatment. Interferon produced a lymphoma response in three of four HCV positive patients. Of 27 treated patients, 13 patients achieved a complete response, and 12 patients achieved a partial response. The median event-free survival (EFS) was 3.3 years (5.1 years for patients with disease confined to the abdomen and 2.1 years for patients with disease extension to superficial lymph nodes). Among nine patients with nodal MZL, four patients had Stage IV disease. HCV serology was positive in two patients. Five patients responded to chemotherapy. The median EFS was 2.8 years. The median overall survival was not reached for patients with both types of MZL. CONCLUSIONS. The results of the current study demonstrated that splenic and nodal MZL are indolent lymphomas with different presenting features but common morphologic and biologic characteristics, including high HCV seroprevalence. Studies will be required to identify specific biologic markers and to define the best treatment.",
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T1 - Splenic and Nodal Marginal Zone Lymphomas Are Indolent Disorders at High Hepatitis C Virus Seroprevalence with Distinct Presenting Features but Similar Morphologic and Phenotypic Profiles

AU - Arcaini, Luca

AU - Paulli, Marco

AU - Boveri, Emanuela

AU - Vallisa, Daniele

AU - Bernuzzi, Patrizia

AU - Orlandi, Ester

AU - Incardona, Paolo

AU - Brusamolino, Ercole

AU - Passamonti, Francesco

AU - Burcheri, Sara

AU - Schena, Claudio

AU - Pascutto, Cristiana

AU - Cavanna, Luigi

AU - Magrini, Umberto

AU - Lazzarino, Mario

PY - 2004/1/1

Y1 - 2004/1/1

N2 - BACKGROUND. Splenic and nodal marginal zone lymphomas (MZL) are subtypes of marginal zone-derived neoplasms. Due to their rarity, little is known concerning their relation, pattern of dissemination, and treatment outcome. METHODS. The authors analyzed the clinicopathologic features and outcome of 43 patients (34 patients with splenic MZL and 9 patients with nodal MZL). All lesional tissues obtained at diagnosis were reviewed histologically. RESULTS. Among the patients with splenic MZL, 30 patients had Stage IV disease (based on the Ann Arbor staging system). Twenty-six patients presented with splenomegaly with or without limited involvement of abdominal lymph nodes, whereas 7 patients showed disease extension to superficial lymph nodes. Hepatitis C virus (HCV) serology was positive in 35% of patients. Seventeen patients underwent splenectomy, 8 patients received chemotherapy, and 7 patients were followed without initial treatment. Interferon produced a lymphoma response in three of four HCV positive patients. Of 27 treated patients, 13 patients achieved a complete response, and 12 patients achieved a partial response. The median event-free survival (EFS) was 3.3 years (5.1 years for patients with disease confined to the abdomen and 2.1 years for patients with disease extension to superficial lymph nodes). Among nine patients with nodal MZL, four patients had Stage IV disease. HCV serology was positive in two patients. Five patients responded to chemotherapy. The median EFS was 2.8 years. The median overall survival was not reached for patients with both types of MZL. CONCLUSIONS. The results of the current study demonstrated that splenic and nodal MZL are indolent lymphomas with different presenting features but common morphologic and biologic characteristics, including high HCV seroprevalence. Studies will be required to identify specific biologic markers and to define the best treatment.

AB - BACKGROUND. Splenic and nodal marginal zone lymphomas (MZL) are subtypes of marginal zone-derived neoplasms. Due to their rarity, little is known concerning their relation, pattern of dissemination, and treatment outcome. METHODS. The authors analyzed the clinicopathologic features and outcome of 43 patients (34 patients with splenic MZL and 9 patients with nodal MZL). All lesional tissues obtained at diagnosis were reviewed histologically. RESULTS. Among the patients with splenic MZL, 30 patients had Stage IV disease (based on the Ann Arbor staging system). Twenty-six patients presented with splenomegaly with or without limited involvement of abdominal lymph nodes, whereas 7 patients showed disease extension to superficial lymph nodes. Hepatitis C virus (HCV) serology was positive in 35% of patients. Seventeen patients underwent splenectomy, 8 patients received chemotherapy, and 7 patients were followed without initial treatment. Interferon produced a lymphoma response in three of four HCV positive patients. Of 27 treated patients, 13 patients achieved a complete response, and 12 patients achieved a partial response. The median event-free survival (EFS) was 3.3 years (5.1 years for patients with disease confined to the abdomen and 2.1 years for patients with disease extension to superficial lymph nodes). Among nine patients with nodal MZL, four patients had Stage IV disease. HCV serology was positive in two patients. Five patients responded to chemotherapy. The median EFS was 2.8 years. The median overall survival was not reached for patients with both types of MZL. CONCLUSIONS. The results of the current study demonstrated that splenic and nodal MZL are indolent lymphomas with different presenting features but common morphologic and biologic characteristics, including high HCV seroprevalence. Studies will be required to identify specific biologic markers and to define the best treatment.

KW - Low-grade non-Hodgkin lymphoma

KW - Marginal zone

KW - Nodal marginal zone lymphoma

KW - Splenic marginal zone lymphoma

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