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splenic 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/28291005/the-correlation-between-pre-treatment-fluorodeoxyglucose-positron-emission-tomography-computed-tomography-parameters-and-clinical-prognostic-factors-in-pediatric-hodgkin-lymphoma
#4
Ebru Tatcı, İnci Uslu Biner, Suna Emir, Hikmet Gülşah Tanyıldız, Özlem Özmen, Engin Alagöz, Atila Gökçek, Gürses Şahin
OBJECTIVE: To compare standardized uptake values (SUV) derived from pre-treatment (18)F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) imaging and clinical prognostic factors in pediatric patients with Hodgkin lymphoma (HL). METHODS: Pre-treatment FDG PET/CT findings of 28 children with HL were evaluated in this retrospective study. Metabolic tumor volume (MTV), SUVmax normalized by weight (SUVweight), lean body mass (SUVlbm), body surface area (SUVbsa) and plasma glucose levels of tumors (SUVglucose) were calculated using pre-treatment FDG PET/CT scan images...
February 5, 2017: Molecular Imaging and Radionuclide Therapy
https://www.readbyqxmd.com/read/28288722/nodal-marginal-zone-lymphoma-clinical-features-diagnosis-management-and-treatment
#5
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/28288721/epidemiology-and-environmental-aspects-of-marginal-zone-lymphomas
#6
REVIEW
Priya Sriskandarajah, Claire E Dearden
Marginal zone lymphomas (MZLs) account for between 5% and 17% of all non-Hodgkin's lymphomas. MZLs consist of 3 different subtypes with extranodal being the most commonly reported, representing 50-70% of MZL, followed by splenic (20%) and nodal (10%). Median age at presentation varies between these lymphoma sub-types, ranging between 50 and 69 years, with an overall greater incidence noted in males compared to females. Given the rarity of these lymphomas, epidemiologic data has been sparse, although it has been suggested the aetiology is multi-factorial including ethnicity and geographical factors...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288718/splenic-marginal-zone-lymphoma
#7
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
#8
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
#9
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/28288714/bacteria-associated-with-marginal-zone-lymphomas
#10
REVIEW
Maurilio Ponzoni, Andrés J M Ferreri
In the last decades some bacteria have been associated with a various extent with marginal zone (extra nodal, nodal and splenic types) lymphomas are frequently associated with chronic infections, with important clinical, molecular, biological, and therapeutic implications. The well-known correlation between Helicobacter pylori and gastric MALT-lymphoma, the recently reported links between Chlamydia psittaci and ocular adnexal MALT-lymphoma and Borrelia burgdorferi and cutaneous MALT lymphoma constitute the most studied examples; in addition, Campylobacter jejuni and some more recent associations encompassing Achromobacter xylosoxidans and Haemophilus influenzae will be further reported...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288713/methylation-patterns-in-marginal-zone-lymphoma
#11
REVIEW
Alberto J Arribas, Francesco Bertoni
Promoter DNA methylation is a major regulator of gene expression and transcription. The identification of methylation changes is important for understanding disease pathogenesis, for identifying prognostic markers and can drive novel therapeutic approaches. In this review we summarize the current knowledge regarding DNA methylation in MALT lymphoma, splenic marginal zone lymphoma, nodal marginal zone lymphoma. Despite important differences in the study design for different publications and the existence of a sole large and genome-wide methylation study for splenic marginal zone lymphoma, it is clear that DNA methylation plays an important role in marginal zone lymphomas, in which it contributes to the inactivation of tumor suppressors but also to the expression of genes sustaining tumor cell survival and proliferation...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288711/marginal-zone-lymphoma-in-elderly-and-geriatric-patients
#12
REVIEW
Valentin Goede
Approximately 50% of patients with newly diagnosed marginal zone lymphoma (MZL) are of advanced age. For the three subtypes of MZL (extranodal MZL of mucosa-associated lymphoid tissue, splenic MZL, nodal MZL), the median age at diagnosis is around 65-70 years. Due to the lack of larger studies in MZL, little is known of the prevalence of comorbidity, polypharmacy, or geriatric syndromes in older patients with MZL. The impact of these concurrent conditions on the tolerability and feasibility of diagnostic or therapeutic procedures used in MZL has not been specifically investigated...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288709/treatment-of-splenic-marginal-zone-lymphoma
#13
REVIEW
Christina Kalpadakis, Gerassimos A Pangalis, Maria K Angelopoulou, Theodoros P Vassilakopoulos
Splenic marginal zone lymphoma (SMZL) is a distinct lymphoma entity characterized by an indolent clinical course and prolonged survival. Treatment is not standardized, since there are no prospective randomized trials in large series of SMZL patients. Splenectomy and rituximab represent the most effective treatment strategies used so far. The addition of chemotherapy to rituximab has not further improved the outcome, although this issue requires further investigation. Rituximab monotherapy has been associated with high response rates (∼90%), with approximately half of these responses being complete, even at the molecular level...
March 2017: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/28288708/transformation-of-marginal-zone-lymphoma-and-association-with-other-lymphomas
#14
REVIEW
Carla Casulo, Jonathan Friedberg
Marginal zone lymphomas (MZL) are a diverse group of indolent lymphoproliferative disorders that comprise three subtypes: nodal, splenic and mucosal associated marginal zone lymphomas (MALT). Histologic transformation (HT) to an aggressive lymphoma is a rare event that can occur in any subtype, and at lower frequency compared to other indolent non Hodgkin lymphomas (NHL) like follicular lymphoma. There are few data directly associated with risk and prognosis of transformation in MZL. However, recent advances in the understanding of molecular and genetic features of MALT have contributed to an evolving appreciation of HT in this disease...
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
#15
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
#16
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/28250305/splenic-marginal-zone-lymphoma-with-acquired-von-willebrand-syndrome-diagnosed-via-splenic-bleeding
#17
Yukiko Komeno, Naoki Shibuya, Hideki Uryu, Haruki Yamada, Takeo Toda, Masayuki Shibasaki, Shinji Kunishima, Kuniko Iihara, Tomiko Ryu
An 85-year-old woman underwent emergent splenectomy due to left abdominal pain and active bleeding in a massively enlarged spleen. The histological diagnosis was splenic marginal zone lymphoma (SMZL). A prolonged activated partial thromboplastin time (APTT) was noted, and additional tests led to the diagnosis of type 2A-like acquired von Willebrand syndrome (AVWS). An APTT cross mixing test ruled out the presence of inhibitors. She received eight courses of rituximab monotherapy. The coagulation data showed no improvement, possibly because the lymphoma showed a poor response to the treatment...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28235627/florid-splenic-gamma-delta-t-cell-proliferation-in-patients-with-splenomegaly-and-cytopenias-a-high-stakes-diagnostic-challenge
#18
Shanxiang Zhang, Michael G Bayerl
OBJECTIVES: Splenic gamma/delta T-cell proliferation is rare and correct diagnosis is critical for adequate clinical management. METHODS: Two splenectomy cases from patients with splenomegaly and cytopenias were studied by morphological evaluation, extensive immunophenotyping, FISH and molecular studies. The clinicopathologic findings were compared with splenic T gamma/delta neoplasia, notably hepatosplenic T-cell lymphoma (HSTL) and T-cell large granular lymphocytic leukemia (TLGL) of the variety T gamma/delta...
February 21, 2017: Human Pathology
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
#19
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
#20
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
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