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Nodal marginal zone lymphoma

Angela Brown, Isabella Sciascia-Visani, Dianna Farrell, Meg Smith, Clive Felix, Vanaja Mutharajah, Jackie Ruell, Graeme Taylor
Background: Nodal marginal zone B-cell lymphoma is a rare entity in which the cytogenetic findings are not well defined. The t(2;14)(p24;q32) has previously been reported in three patients with blastic mantle cell lymphoma and one patient with follicular lymphoma. This rearrangement has not been reported previously in a patient with a diagnosis of nodal marginal zone B-cell lymphoma. Case presentation: We present a male patient who presented with lymphadenopathy...
2019: Molecular Cytogenetics
Muhammad J Khalil, Mustafa N Malik, Maryam Ahmed, Abdul Rafae, Faiz Anwer
Marginal zone B cell lymphomas are divided into nodal, extranodal and splenic types. Renal involvement by extranodal B cell lymphoma is extremely rare with an incidence of about 0.1%. We present a case of a 79-year-old Caucasian male with progressive renal failure and isolated left renal extranodal marginal zone lymphoma. Asymptomatic immunoglobulin (Ig) M monoclonal gammopathy along with bone marrow involvement by lymphoma was observed. Contemporary management options including radiotherapy (RT), chemotherapy, immune-modulating agents and novel chemotherapy-free regimens...
November 8, 2018: Curēus
Xiang Meng Li, Hai Song Zhang, Xiao Li Dai, Jian Hong Shi
RATIONALE: Primary extra-nodal non-Hodgkin lymphoma (PE-NHL) arising in the region of the buttocks is rare. After reviewing the literature from the last 20 years, we found only 3 reported lymphomas originating from soft tissue of the buttocks. In our case, positron emission tomography/computed tomography (PET/CT) was performed for the first time, both before and after treatment, to determine the initial stage of PE-NHL and the curative effects of treatment. PATIENT CONCERNS: We report the case of a 71-year-old woman who was admitted to our hospital due to pain, skin redness, rising skin temperature, and swelling in the right hip...
December 2018: Medicine (Baltimore)
Eri Ishikawa, Yasuhiro Takahashi, Ma Regina Paula Valencia, Marian Grace Ana-Magadia, Hirohiko Kakizaki
PURPOSE: To examine the clinicopathological characteristics of patients with thyroid eye disease (TED) who showed asymmetric lacrimal gland enlargement. METHODS: Sixteen patients were included in this retrospective, observational case series. The following data were collected: sex, age, laterality, past medical history, laboratory results for thyroid function and other autoimmune disorders, pathological results, other systemic lesions related to lacrimal gland pathology, and the clinical course after treatment...
November 28, 2018: Graefe's Archive for Clinical and Experimental Ophthalmology
Aliki Xochelli, Vasilis Bikos, Eleftheria Polychronidou, Chrysi Galigalidou, Andreas Agathangelidis, Frédéric Charlotte, Panagiotis Moschonas, Zadie Davis, Monica Colombo, Maria Roumelioti, Lesley-Ann Sutton, Patricia Groenen, Michiel van den Brand, Myriam Boudjoghra, Patricia Algara, Alexandra Traverse-Glehen, Ana Ferrer, Evangelia Stalika, Maria Karypidou, George Kanellis, Christina Kalpadakis, Manuella Mollejo, Gerasimos Pangalis, Panayiotis Vlamos, Rose-Marie Amini, Sarka Pospisilova, David Gonzalez, Maurilio Ponzoni, Achilles Anagnostopoulos, Véronique Giudicelli, Marie-Paule Lefranc, Blanca Espinet, Panagiotis Panagiotidis, Miguel Angel Piris, Ming-Qing Du, Richard Rosenquist, Theodora Papadaki, Chrysoula Belessi, Manlio Ferrarini, David Oscier, Dimitrios Tzovaras, Paolo Ghia, Frederic Davi, Anastasia Hadzidimitriou, Kostas Stamatopoulos
The B cell receptor immunoglobulin (BcR IG) gene repertoires of marginal zone (MZ) lymphoproliferations were analyzed in order to obtain insight into their ontogenetic relationships. Our cohort included cases with MZ lymphomas (n=488) i.e. splenic (SMZL), nodal (NMZL) and extranodal (ENMZL) as well as provisional entities (n=76) according to the World Health Organization classification. The most striking IG gene repertoire skewing was observed in SMZL. However, restrictions were also identified in all other MZ lymphomas studied, particularly ENMZL, with significantly different IG gene distributions depending on the primary site of involvement...
November 28, 2018: Journal of Pathology
Stephanie L Skala, Boris Hristov, Alexandra C Hristov
CONTEXT.—: Primary cutaneous follicle center lymphoma is a low-grade B-cell lymphoma that is limited to the skin at diagnosis. It has a differential diagnosis that includes systemic/nodal follicular lymphoma secondarily involving the skin; primary cutaneous diffuse large B-cell lymphoma leg type; reactive lymphoid hyperplasia; and primary cutaneous marginal zone lymphoma. OBJECTIVE.—: To review the clinical, morphologic, immunophenotypic, and genetic features of primary cutaneous follicle center lymphoma; its differential diagnosis; and the evidence that supports use of immunohistochemistry and genetic testing in the diagnosis and prognosis of this entity...
November 2018: Archives of Pathology & Laboratory Medicine
Andrej Belančić, Luka Vranić, Ivan Ševeljević, Ita Hadžisejdić, Antica Duletić Načinović, Nives Jonjić
An association between autoimmune events, as well as the development of antiphospholipid (aPL) antibodies and lymphoproliferative disorders is well recognized. We present the patient with coagulation abnormalities and non-Hodgkin lymphoma (NHL), primarily diagnosed as nodal marginal zone B-cell lymphoma (NMZL), and in relapse as diffuse large B-cell lymphoma (DLBCL). In the follow-up period, the patient simultaneously developed different aPL antibodies. The presence of aPL antibodies in NHL is frequent but it is not common in the NMZL...
October 23, 2018: Pathology, Research and Practice
Juan Pablo Alderuccio, Wei Zhao, Amrita Desai, Nicolas Gallastegui, Jeremy Ramdial, Erik Kimble, Macarena I de la Fuente, Joseph D Rosenblatt, Jennifer R Chapman, Francisco Vega, Isildinha M Reis, Izidore S Lossos
PURPOSE: Given the paucity of data on higher-grade transformation (HGT) to aggressive lymphoma in patients with marginal zone lymphoma (MZL), we report on a large cohort of patients, identify risk factors, and determine HGT impact on overall survival (OS). METHODS: We analyzed 453 patients with biopsy-proven MZL seen at our institution between 1995 and 2016. Kaplan-Meier, Cox proportional hazards regression, and competing risk methods were used in analyses of time-to-event outcomes...
October 12, 2018: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
Vadim R Gorodetskiy, Wolfram Klapper, Natalya A Probatova, Vladimir I Vasilyev, Elena V Rozhnova
We present an exceptionally rare case of co-occurrence of Rosai-Dorfman disease (RDD) and nodal marginal zone lymphoma (NMZL) in a 60-year-old Caucasian female with a 20-year course of Sjögren's syndrome (SS). In response to treatment for lymphoma, the patient presented a short positive response, followed by a rapid progression of the disease accompanied by the development of the peripheral facial nerve palsy. We failed to detect Epstein-Barr virus (EBV) in the NMZL/RDD sample by EBV-encoded RNA (EBER) in situ hybridization but identified genomic DNA of EBV by polymerase chain reaction...
2018: Case Reports in Hematology
Paolo K Soriano, Taylor Stone, Junaid Baqai, Sherjeel Sana
BACKGROUND Leukemias and lymphomas can arise from myeloid or lymphoid stem cells. Combined myeloid leukemia and non-Hodgkin's lymphoma (NHL), either synchronous or metachronous, rarely occur in the same patient. This report is of a 67-year-old man with a synchronous diagnosis of both bone marrow chronic myelomonocytic leukemia (CMML) and nodal marginal zone lymphoma (NMZL), which a peripheral low-grade B-cell NHL. CASE REPORT A 67-year-old Caucasian man, who was a long-term cigarette smoker, presented with a five-year history of leukocytosis and cervical lymphadenopathy...
September 26, 2018: American Journal of Case Reports
Irit Avivi, Luca Arcaini, Virginia V Ferretti, Ariane Boumendil, Hervé Finel, Giuseppe Milone, Francesco Zaja, Devizzi Liliana, Maurizio Musso, Blaise Didier, Emmanuel Bachy, Mohammed Wattad, Emmanuelle Nicolas-Virelizier, Martin Gramatzki, Jean-Henri Bourhis, Denis Caillot, Anette Haenel, Gerhard Held, Catherine Thieblemont, Pavel Jindra, David Pohlreich, François Guilhot, Frank Kroschinsky, Björn Wahlin, Christof Scheid, Norbert Ifrah, Christian Berthou, Peter Dreger, Silvia Montoto, Annarita Conconi
The role of autologous stem cell transplantation (ASCT) in patients with marginal zone lymphoma (MZL) is debatable. This study investigated the outcome and prognostic factors affecting the outcome of patients undergoing ASCT for MZL. Eligible patients had non-transformed nodal, extra-nodal (MALT) or splenic MZL (SMZL), aged ≥18 years, who underwent a first ASCT between1994 and 2013 and were reported to the European Society for Blood and Marrow Transplantation, Fondazione Italiana Linfomi or Gruppo Italiano Trapianto Di Midollo Osseo registries...
September 2018: British Journal of Haematology
Hiroe Itami, Hirokazu Nakamine, Maiko Takeda, Tokiko Nakai, Tomoya Myojin, Minami Matsuoka, Shoh Sasaki, Tomoko Uchiyama, Kohei Morita, Tomomi Fujii, Kinta Hatakeyama, Chiho Ohbayashi
Although it has been described that extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT lymphomas) are the most common type among primary salivary gland lymphomas (SGLs), some studies revealed that the frequency of follicular lymphomas (FLs) was as high as that of MALT lymphomas. However, it has been reported that many of these FLs may have developed in lymph nodes attached to the capsule of the glands or intraglandular lymph nodes. Clinical, histological, immunohistochemical, and cytogenetic features of 11 SGL cases, which were extracted from our surgical pathology file consisting of consecutive pathology cases, were reevaluated to further characterize whether they were actually primary SGLs...
July 1, 2018: International Journal of Surgical Pathology
Chi Yuen Cheung, Wing Hung Lau, Wah Cheuk
A 56-year-old man, who received deceased kidney transplant 20 years ago, presented with an enlarged submandibular lymph node. Histologic examination revealed nodal marginal zone lymphoma in which the neoplastic lymphoid cells showed diffuse positivity for Epstein-Barr virus early RNA by in situ hybridization. Systemic lymphoma workup showed stage I disease. The tumor was managed as a posttransplant lymphoproliferative disorder and the immunosuppression was modified. There was no evidence of lymphoma at follow-up 6 years after excision alone...
June 1, 2018: International Journal of Surgical Pathology
Francesco Bertoni, Davide Rossi, Emanuele Zucca
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 )...
2018: F1000Research
Nathan M Denlinger, Narendranath Epperla, Basem M William
Marginal zone lymphomas (MZLs) consist of a diverse family of malignancies, which are derived from B-cells. The disease subtypes are recognized extranodal, nodal, and splenic MZLs. The disease characteristics, clinical course, and treatment vary considerably based on the site of involvement. In 2017, the US Food and Drug Administration approved ibrutinib, a first in class Bruton's tyrosine kinase inhibitor that revolutionized the care of chronic lymphocytic leukemia patients; for, the treatment of relapsed/refractory MZL based on pivotal open-label Phase II trial demonstrated an overall response rate of 48%, with a complete response rate of 3%, median progression-free survival of 14...
2018: Cancer Management and Research
Yu Uemura, Hirotaka Sakai, Yusuke Saiki, Akiko Uchida, Kazuyuki Sato, Yuka Tsuruoka, Satoshi Yokoi, Yuji Nishio, Manabu Matsunawa, Yoshinori Suzuki, Yasushi Isobe, Masayuki Kato, Naoto Tomita, Yasuyuki Inoue, Ikuo Miura
A 73-year-old man with left parotid gland swelling over 2 months was referred to our hospital in March 201X. Purpura on the lower legs had been recurrent for >20 years. Biopsy of the parotid gland demonstrated diffuse infiltration of abnormal lymphocytes that were negative for CD10 and positive for CD19, CD20, and κ-chain. The Ki-67 positivity was <10%; lymphoepithelial lesions were observed. The patient was diagnosed with extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma)...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Sabarish Ayyappan, Basem M William
PURPOSE OF REVIEW: The purpose of the study is to summarize the current conundrums in the management of marginal zone lymphomas (MZL). RECENT FINDINGS: In 2017, the US Food and Drug Administration (FDA) approved ibrutinib, a first in class Bruton Tyrosine Kinase inhibitor, for the treatment of relapsed/refractory MZL based on pivotal open-label phase II trial demonstrating an overall response rates of 48%. Clinical trials design utilizing chemotherapy-free regimens for relapsed/refractory disease are gaining popularity...
March 23, 2018: Current Oncology Reports
V Pillonel, D Juskevicius, C K Y Ng, A Bodmer, A Zettl, D Jucker, S Dirnhofer, A Tzankov
Nodal marginal zone lymphoma (NMZL) is a rare small B-cell lymphoma lacking disease-defining phenotype and precise diagnostic markers. To better understand the mutational landscape of NMZL, particularly in comparison to other nodal small B-cell lymphomas, we performed whole-exome sequencing, targeted high-throughput sequencing, and array-comparative genomic hybridization on a retrospective series. Our study identified for the first time recurrent, diagnostically useful, and potentially therapeutically relevant BRAF mutations in NMZL...
November 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Gemma Ann Joan Harrop, Jane Tighe, Alexander MacKenzie
A 68-year-old man presented with a 4-day history of worsening knee and arm pain. On examination, there was erythema and swelling of the left knee and both wrists. Joint aspiration grew Neisseria meningitidis Blood tests showed an unusually high total protein level (100 g/L) and an IgM kappa paraprotein band of 45 g/L on protein electrophoresis. CT showed widespread lymphadenopathy, hepatosplenomegaly and multilevel thoracic vertebral collapse. A bone marrow biopsy revealed a lymphoplasmacytic infiltrate and a lymph node biopsy showed features of nodal marginal zone lymphoma with plasmacytic differentiation...
January 23, 2018: BMJ Case Reports
Roberto Castelli, Luigi Bergamaschini, Giorgio Lambertenghi Deliliers
Splenic marginal zone lymphomas (SMZLs) are rare indolent B cell neoplasms that affect the spleen, bone marrow, and blood. Although they have an indolent course in the majority of patients, who have a median survival of 8-10 years, ∼ 30% may experience a worse outcome. The prognostic criteria of progression are lymph node and extra-nodal involvement, high lymphocyte counts, anaemia, and thrombocytopenia. The treatment of SMZLs include a "wait and watch strategy", splenectomy, and alkylating agents ± rituximab...
December 29, 2017: Medical Oncology
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