RESUMO
The objective of this guideline is to provide healthcare professionals with clear guidance on the diagnosis and management of patients with marginal zone lymphoma (MZL).
Assuntos
Humanos , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Biópsia , Linfoma de Zona Marginal Tipo Células B/classificação , Linfoma Extranodal de Células T-NKRESUMO
The three main types of marginal zone lymphoma (MZL), recognized by the current lymphoma classifications are the extranodal MZL of mucosa-associated lymphoid tissue, the splenic MZL, and the nodal MZL. They share some karyotype lesions (trisomies of chromosomes 3 and 18, deletions at 6q23), and alterations of the nuclear factor kappa B (NFkB) pathway are also common in all of them. However, they differ in the presence of recurrent translocations, mutations affecting the Notch signaling pathway (NOTCH2 and less commonly NOTCH1), the transcription factors Kruppel-like factor 2 (KLF2) or the receptor-type protein tyrosine phosphatase delta (PTPRD). This review summarizes the most recent and significant advances in our understanding of the epidemiology, genetics, and biology of MZLs and outlines the current principles of the standard management of MZL at different anatomic sites.
Assuntos
Leucemia Linfocítica Crônica de Células B , Linfoma de Zona Marginal Tipo Células B , Humanos , Linfoma de Zona Marginal Tipo Células B/diagnóstico , Linfoma de Zona Marginal Tipo Células B/genética , Linfoma de Zona Marginal Tipo Células B/terapia , Fatores de Transcrição/genética , Translocação Genética , Leucemia Linfocítica Crônica de Células B/genética , Rearranjo GênicoRESUMO
Marginal zone lymphoma (MZL) is a relatively uncommon subtype of non-Hodgkin lymphoma consisting of extranodal, nodal, and splenic MZL. Mucosa-associated lymphoma constitutes the majority of extranodal MZL including but not limited to the stomach, lung, salivary gland, ocular adnexa, skin, and thyroid. Depending on the site of origin, maltoma may present with different symptoms. Here we present a patient who presented to the pulmonary clinic for further evaluation of a right middle lobe consolidation. She was treated with a course of antibiotics empirically with no interval change in imaging. She underwent bronchoscopy with biopsy. Pathology was remarkable for extensive lymphoid infiltrates consisting of mixed B and T lymphocytes. Positron emission tomography (PET) CT demonstrated mild uniform uptake in consolidation with no evidence of PET avid distant metastasis. CT-guided biopsy was consistent with extranodal marginal zone lymphoma. She underwent right middle lobectomy with no complications. As mentioned in our case, bronchoscopy is usually nondiagnostic and a lung biopsy is needed in pulmonary maltoma. Treatment is based on the tumor location and extent of the disease. Prognosis is good with an 86-95% five-year survival.
RESUMO
There are three different marginal zone lymphomas (MZLs): the extranodal MZL of mucosa-associated lymphoid tissue (MALT) type (MALT lymphoma), the splenic MZL, and the nodal MZL. The three MZLs share common lesions and deregulated pathways but also present specific alterations that can be used for their differential diagnosis. Although trisomies of chromosomes 3 and 18, deletions at 6q23, deregulation of nuclear factor kappa B, and chromatin remodeling genes are frequent events in all of them, the three MZLs differ in the presence of recurrent translocations, mutations affecting the NOTCH pathway, and the transcription factor Kruppel like factor 2 ( KLF2) or the receptor-type protein tyrosine phosphatase delta ( PTPRD). Since a better understanding of the molecular events underlying each subtype may have practical relevance, this review summarizes the most recent and main advances in our understanding of the genetics and biology of MZLs.
