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Splenic B-cell lymphoma

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https://www.readbyqxmd.com/read/28321090/splenic-marginal-zone-lymphoma-complicated-by-cold-agglutinin-disease
#1
Kiyosumi Ochi, Kazuaki Yokoyama, Nobuhiro Ohno, Yasunori Ota, Arinobu Tojo
Splenic marginal zone lymphoma (SMZL) is a rare low-grade B-cell lymphoma accounting for less than 1% of lymphoid neoplasms and is often associated with autoimmune disorders. A 48-year-old woman presented with severe anemia due to steroid-refractory cold agglutinin disease (CAD), and was referred to our hospital for management of progressive systemic illness and high fever. On admission, she showed elevated serum soluble IL-2R and mild splenomegaly. PET/CT revealed FDG accumulation in the spleen and bone. She was pathologically diagnosed as having splenic marginal zone lymphoma by splenectomy and received 8 cycles of rituximab every 2 weeks, resulting in marked improvement of anemia...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28297625/genomics-of-hairy-cell-leukemia
#2
Enrico Tiacci, Valentina Pettirossi, Gianluca Schiavoni, Brunangelo Falini
Hairy cell leukemia (HCL) is a chronic mature B-cell neoplasm with unique clinicopathologic features and an initial exquisite sensitivity to chemotherapy with purine analogs; however, the disease relapses, often repeatedly. The enigmatic pathogenesis of HCL was recently clarified by the discovery of its underlying genetic cause, the BRAF-V600E kinase-activating mutation, which is somatically and clonally present in almost all patients through the entire disease spectrum and clinical course. By aberrantly activating the RAF-MEK-ERK signaling pathway, BRAF-V600E shapes key biologic features of HCL, including its specific expression signature, hairy morphology, and antiapoptotic behavior...
March 20, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28295729/targeting-of-b-cell-receptor-signalling-in-b-cell-malignancies
#3
M Jerkeman, M Hallek, M Dreyling, C Thieblemont, E Kimby, L Staudt
Pharmacological agents that inhibit enzymes of the B-cell receptor (BCR) pathway are of increasing importance in the treatment of B-cell malignancies. These include inhibitors of Bruton tyrosine kinase (BTK), phosphatidylinositol 3-kinase (PI3K), splenic tyrosine kinase and protein kinase Cβ. Two agents are already approved in the USA and Europe: ibrutinib, a BTK inhibitor, for the treatment of chronic lymphatic leukaemia (CLL), mantle cell lymphoma (MCL) and Waldenström's macroglobulinemia; and idelalisib, a PI3Kδ inhibitor, for the treatment of CLL and follicular lymphoma...
March 14, 2017: Journal of Internal Medicine
https://www.readbyqxmd.com/read/28288722/nodal-marginal-zone-lymphoma-clinical-features-diagnosis-management-and-treatment
#4
REVIEW
Tamar Tadmor, Aaron Polliack
Nodular marginal zone lymphoma (NMZL) is a small B-cell lymphoma involving only lymph nodes and is the least common form of MZL constituting about 10% of cases. Patients usually present with advanced disease which must be distinguished from extranodal MZL with lymph node spread. NMZL shares cytological and immunophenotypic features with MALT and splenic MZL, but has a less favorable prognosis than these two categories. It occurs mostly in adults and pediatric cases are rare. Different therapeutic approaches have been used in NMZL, but because of the small patient numbers involved, more definitive treatment is still anticipated...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288718/splenic-marginal-zone-lymphoma
#5
REVIEW
Miguel A Piris, Arantza Onaindía, Manuela Mollejo
Splenic marginal zone lymphoma (SMZL) is an indolent small B-cell lymphoma involving the spleen and bone marrow characterized by a micronodular tumoral infiltration that replaces the preexisting lymphoid follicles and shows marginal zone differentiation as a distinctive finding. SMZL cases are characterized by prominent splenomegaly and bone marrow and peripheral blood infiltration. Cells in peripheral blood show a villous cytology. Bone marrow and peripheral blood characteristic features usually allow a diagnosis of SMZL to be performed...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288717/pathology-of-nodal-marginal-zone-lymphomas
#6
REVIEW
Stefano Pileri, Maurilio Ponzoni
Nodal marginal zone B cell lymphomas (NMZLs) are a rare group of lymphoid disorders part of the spectrum of marginal zone B-cell lymphomas, which encompass splenic marginal one B-cell lymphoma (SMZL) and extra nodal marginal zone of B-cell lymphoma (EMZL), often of MALT-type. Two clinicopathological forms of NMZL are recognized: adult-type and pediatric-type, respectively. NMZLs show overlapping features with other types of MZ, but distinctive features as well. In this review, we will focus on the salient distinguishing features of NMZL mostly under morphological/immunophenotypical/molecular perspectives in views of the recent acquisitions and forthcoming updated 2016 WHO classification of lymphoid malignancies...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288716/molecular-pathogenesis-of-splenic-and-nodal-marginal-zone-lymphoma
#7
REVIEW
Valeria Spina, Davide Rossi
Genomic studies have improved our understanding of the biological basis of splenic (SMZL) and nodal (NMZL) marginal zone lymphoma by providing a comprehensive and unbiased view of the genes/pathways that are deregulated in these diseases. Consistent with the physiological involvement of NOTCH, NF-κB, B-cell receptor and toll-like receptor signaling in mature B-cells differentiation into the marginal zone B-cells, many oncogenic mutations of genes involved in these pathways have been identified in SMZL and NMZL...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28280994/detection-of-myd88-l265p-and-whim-like-cxcr4-mutation-in-patients-with-igm-monoclonal-gammopathy-related-disease
#8
Xin-Xin Cao, Qi Meng, Hao Cai, Tian-Hua He, Cong-Li Zhang, Wei Su, Jian Sun, Yue Li, Wei Xu, Dao-Bin Zhou, Jian Li
A broad spectrum of diseases are associated with IgM monoclonal gammopathy, including Waldenstrom macroglobulinemia (WM), various types of B cell non-Hodgkin's lymphoma (NHL), multiple myeloma (MM), primary amyloidosis (AL), and monoclonal gammopathy of undetermined significance (MGUS); these are called IgM monoclonal gammopathy related diseases (IgM-RD). We investigated MYD88 L265P and WHIM-like CXCR4 mutations in various IgM-RD. Patients with serum immunofixation electrophoresis confirmed IgM monoclonal gammopathy who had enough material for DNA extraction and presented between January 2008 and October 2016 at Peking Union Medical College Hospital were enrolled in this cohort...
March 9, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/28259031/follicular-lymphoma-transforming-into-diffuse-large-b-cell-lymphoma-in-spleen-simultaneous-appearance-of-both-on-18-f-fdg-pet-ct-and-histology
#9
William Makis, Anthony Ciarallo, Tina Petrogiannis-Haliotis, Arthur Rosenberg, Stephan Probst
Low grade lymphoma may transform into a more aggressive lymphoma and this transformation is usually associated with a poor outcome. A 65year old man presented with two metabolically active splenic lesions on a staging [18F] fluoro-2-deoxy-d-glucose ((18)F-FDG) positron emission tomography/computed tomography (PET/CT). Histologic evaluation post splenectomy confirmed the presence of two clonally related lymphomas: a follicular lymphoma (FL) and a diffuse large B-cell lymphoma (DLBCL). Molecular genetic studies confirmed that the DLBCL lesions arose from a pre-existing FL...
February 21, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28230270/a-phase-2-study-of-lenalidomide-rituximab-cyclophosphamide-and-dexamethasone-lr-cd-for-untreated-low-grade-non-hodgkin-lymphoma-requiring-therapy
#10
Allison Rosenthal, Amylou C Dueck, Stephen Ansell, Katherine Gano, Christopher Conley, Grzegorz S Nowakowski, John Camoriano, Jose F Leis, Joseph R Mikhael, A Keith Stewart, David Inwards, David Dingli, Shaji Kumar, Pierre Noel, Morie Gertz, Luis Porrata, Stephen Russell, Joseph Colgan, Rafael Fonseca, Thomas M Habermann, Prashant Kapoor, Francis Buadi, Nelson Leung, Rodger Tiedemann, Thomas E Witzig, Craig Reeder
Patients with indolent NHL have multiple treatment options yet there is no consensus as to the best initial therapy. Lenalidomide, an immunomodulatory agent, has single agent activity in relapsed lymphoma. This trial was conducted to assess feasibility, efficacy and safety of adding lenalidomide to rituximab, cyclophosphamide, and dexamethasone (LR-CD) in untreated indolent NHL patients requiring therapy. This was a single institution phase II trial. Treatment consisted of IV rituximab 375mg/m(2) day 1, oral lenalidomide 20mg days 1-21, cyclophosphamide 250mg/m(2) days 1, 8, 15, and dexamethasone 40mg days 1, 8, 15, 22, of a 28-day cycle...
February 23, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/28203580/unusual-presentation-of-diffuse-large-b-cell-lymphoma-with-splenic-infarcts
#11
Vivek Kumar, Parita Soni, Vishangi Dave, Jonathan Harris
A 67-year-old man presented with a 3-day history of abdominal pain, fever, and significant weight loss over 2 months. Physical examination revealed left upper quadrant tenderness, hepatomegaly, splenomegaly, and bilateral pitting edema but peripheral lymphadenopathy was absent. Laboratory tests showed anemia, thrombocytopenia, elevated prothrombin time (PT), partial thromboplastin time (PTT), and increased lactate dehydrogenase (LDH). PTT was corrected completely in mixing study. Further workup for the cause of coagulopathy revealed decreased levels of all clotting factors except factor VIII and increase fibrinogen levels, which ruled out disseminated intravascular coagulation (DIC)...
January 2017: Journal of Investigative Medicine High Impact Case Reports
https://www.readbyqxmd.com/read/28195266/splenic-histiocyte-rich-pseudotumor-following-chemotherapy-for-non-hodgkin-diffuse-large-b-cell-lymphoma
#12
A G Abdou, M Kandil, M S Eldien, R Abdallah
Chemotherapy may induce mass lesion in rare conditions, which can be easily mistaken as a residual tumor mass. In this report, we describe a mass affecting spleen in a patient received chemotherapy for non Hodgkin diffuse large B cell lymphoma. This mass proved histologically to be non neoplastic formed of sheets of histiocytes and xanthoma cells, which is called histiocyte-rich pseudotumor. This report describes this rare lesion and the possible differential diagnosis.
September 2016: Pathologica
https://www.readbyqxmd.com/read/28190866/splenic-marginal-zone-lymphoma-uncovered-after-a-10-year-follow-up-as-anemia-of-unknown-cause
#13
Asumi Koyama, Chieko Shiotani, Toshio Kurihara, Toshiki Mushino, Yukiharu Okamoto, Tatsunori Tamaki, Takashi Ozaki, Kouichi Ohshima, Shinobu Tamura
A 75-year-old man was referred to our hospital for evaluation of persistent anemia. Despite repeated diagnostic tests, including bone marrow aspiration, the cause of his anemia remained unknown. On each occasion, computed tomography had revealed neither swollen lymph nodes nor splenomegaly. After a 10-year follow-up period, he was admitted with general fatigue and had developed splenomegaly as well as the anemia. Bone marrow biopsy revealed increased abnormal lymphocytes with short villi that were positive for CD11c, CD19, CD20, and kappa chain, but not for CD5, CD10, CD23, or cyclin D1, according to flow cytometry...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28105889/splenic-marginal-zone-lymphoma-excellent-outcomes-in-64-patients-treated-in-the-rituximab-era
#14
Adam G Starr, Paolo F Caimi, PingFu Fu, Mira R Massoud, Howard Meyerson, Eric D Hsi, David B Mansur, Sheen Cherian, Brenda W Cooper, Marcos J G De Lima, Hillard M Lazarus, Stanton L Gerson, Deepa Jagadeesh, Mitchell R Smith, Robert M Dean, Brad L Pohlman, Brian T Hill, Basem M William
OBJECTIVES AND METHODS: Splenic marginal zone lymphoma (SMZL) is a rare non-Hodgkin lymphoma. We sought to identify prognostic factors and define outcomes in a cohort of 64 patients with SMZL who were treated at two large academic medical centers in North America in the rituximab era. RESULTS: Over a median follow-up of 37.8 (range 6-167.1) months, Kaplan-Meier estimate of median OS was 156.3 months and median PFS was 52.9 months. On univariate analysis, baseline hemoglobin <12 g/dl was associated with inferior OS (p = 0...
January 20, 2017: Hematology (Amsterdam, Netherlands)
https://www.readbyqxmd.com/read/28087391/clonal-cytogenetic-abnormalities-are-predictor-in-developing-non-hodgkin-lymphomas
#15
Ying Wang, Yi Xiao, Xiangyu Meng, Heng Zhang, Qinlu Li, Fankai Meng, Lifang Huang, Chunrui Li, Jianfeng Zhou
Pathological analysis is the cornerstone for diagnosing malignant lymphoma. Status of cytogenetic abnormalities is frequently left unexamined if no evidence of malignancy is found in pathological analysis. In this study, we presented 3 cases in which clonal cytogenetic abnormalities were detected but morphological alterations of the same tissue did not support malignant non Hodgkin lymphoma at the first lymph node biopsy. Case 1 is a 55-year-old female with lymphadenopathy neoplastic process confirmed by flow cytometry and polymerase chain reaction (PCR)...
January 11, 2017: Experimental and Molecular Pathology
https://www.readbyqxmd.com/read/28077481/splenomegaly-in-the-returning-traveller-a-diagnostic-workup
#16
Priya Umesh Patel, Padmini Sastry, Muhammad Jawad
Isolated splenomegaly is an unusual condition encompassing a broad range of diagnoses. We report a case of a 38-year-old Asian man who presented with insidious abdominal discomfort and night sweats following recent travel to India. Massive splenomegaly was the only prominent feature on clinical examination and on subsequent imaging. Extensive investigations were performed, ultimately resulting in transfer to a tertiary centre for definitive diagnosis via a splenic biopsy. A fine-needle aspiration was performed, and revealed diffuse large B-cell lymphoma (DLBCL)...
January 11, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28069605/splenic-diffuse-red-pulp-small-b-cell-lymphoma-displays-increased-expression-of-cyclin-d3-and-recurrent-ccnd3-mutations
#17
LETTER
Soraya Curiel-Olmo, Rufino Mondéjar, Carmen Almaraz, Manuela Mollejo, Laura Cereceda, Roso Marès, Sophia Derdak, Yolanda Campos-Martín, Ana Batlle, Sonia González de Villambrosía, Marta Gut, Julie Blanc, Alexandra Traverse-Glehen, Aurelie Verney, Lucile Baseggio, Francisca I Camacho, Andrew Wotherspoon, Kostas Stamatopoulos, Aliki Xochelli, Theodora Papadaki, George Kanellis, Maurilio Ponzoni, Monica García-Cosío, Jose P Vaqué, Sergi Beltrán, Ivo Gut, Miguel Angel Piris, Nerea Martínez
No abstract text is available yet for this article.
February 23, 2017: Blood
https://www.readbyqxmd.com/read/28018857/murine-models-of-splenic-marginal-zone-lymphoma-a-role-for-cav1
#18
REVIEW
Chelsey L Patten, Christine E Cutucache
Dozens of murine models of indolent and aggressive B-cell lymphomas have been generated to date. These include those manifesting chronic lymphocytic leukemia (CLL), diffuse large B-cell lymphoma (DLBCL), as well as xenografts of mantle cell lymphoma (MCL). These models have led to an improved understanding of disease etiology, B-cell biology, immunomodulation, and the importance of the tumor microenvironment. Despite these efforts in CLL, DLBCL, and MCL, considerably little progress toward a model of splenic marginal zone lymphoma (SMZL) has been accomplished...
2016: Frontiers in Oncology
https://www.readbyqxmd.com/read/27882275/a-case-of-an-enigmatic-pulmonary-infiltrate
#19
Uroosa Ibrahim, Amina Saqib, Michel Chalhoub, Jean Paul Atallah
The differential diagnosis of a pulmonary mass in an immunosuppressed host with a history of cancer is broad and includes malignant, infectious and inflammatory etiologies. Mycobacterium avium complex (MAC) is a rare cause of opportunistic infection in susceptible individuals that can present as either localized or disseminated disease. On radiologic studies, the pulmonary disease can manifest as heterogeneous linear or nodular densities, a mass-like lesion, or thin-walled cavitary lesions. We present the case of pulmonary MAC in a patient with a history of lung cancer requiring lobectomy, and splenic lymphoma being treated with chemotherapy, presenting with extreme fatigue and a fludeoxyglucose (FDG)-avid mass on positron emission tomography-computed tomography (PET-CT)...
October 13, 2016: Curēus
https://www.readbyqxmd.com/read/27867670/lymphoma-heterogeneity-three-different-histological-pictures-and-one-unique-clone
#20
Sara Alonso-Alvarez, Alba Redondo-Guijo, Óscar Blanco, Miguel Alcoceba, Ana Balanzategui, Juan C Caballero, Julio Dávila, Marcos González, María D Caballero, Alejandro Martín, Ramón García-Sanz
We report a patient who developed up to three different lymphomas with the same clonal IGH rearrangement. She was first diagnosed of splenic zone marginal lymphoma and relapsed for the first time with Hodgkin lymphoma histology and later with diffuse large B-cell lymphoma histology. Subsequent biopsies and analysis of clonally rearranged IGH genes helped to elucidate the clonal relationship between the three histologies and to confirm a common origin from the three tissue histologies. An integrated diagnosis should always be performed in order to achieve the most accurate diagnosis and be able to choose the best therapeutic options for our patients...
2016: Case Reports in Hematology
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