lymphoma NHL SMZL

Introduction : Marginal zone lymphoma (MZL) accounts for approximately 10% of all cases of non-Hodgkin lymphoma and includes 3 clinically distinct subtypes: extranodal (MALT), splenic (SMZL), and nodal (NMZL). Though commonly grouped in trials of iNHL the clinical behavior, molecular features, and response to therapy of MZL is distinct from other iNHL subtypes and varies among MZL subtypes. Areas covered : This review focuses on the contemporary management of NMZL and SMZL. Treatment with monoclonal antibodies, chemoimmunotherapy, BTK inhibitors, PI3K/mTOR inhibitors, Bcl2 inhibitors, lenalidomide, and CAR-T cell therapy will be covered...

BACKGROUND Rituximab is a chimeric monoclonal antibody to CD20 that is used to treat vasculitis, B-cell lymphoproliferative disorders, and B-cell non-Hodgkin lymphoma (NHL). A report is presented of a case of rituximab-induced acute thrombocytopenia (RIAT) in a woman with splenic marginal zone lymphoma (SMZL) and chronic hepatitis C virus (HCV) infection. CASE REPORT A 46-year-old woman with SMZL complicated by chronic HCV infection presented with worsening B symptoms of fever, night sweats, and loss of weight...

Angioedema due to acquired deficiency of the inhibitor of the first component of complement (C1-INH) is a rare disease known as acquired angioedema (AAE). About 70% of patients with AEE display autoantibodies to C1-INH, the remaining patients have no antibodies to C1-INH. The clinical features of C1-INH deficiency include recurrent, self-limiting local swellings involving the skin, the gastrointestinal tract, and the upper respiratory tract. Swelling is due to accumulation of bradykinin released from high molecular weight kininogen...

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...

OBJECTIVE: To investigate the expression level of large tumor suppressor (LATS)1, LATS2 mRNA and its prognostic value in mantle cell lymphoma (MCL). METHODS: A total of 36 B-NHL cases (including MCL 16 cases, chronic lymphoblastic leukemia (CLL) 11 cases, splenic marginal zone cell lymphoma (SMZL) 9 cases) and 8 healthy donors were enrolled in this study from January 2008 to April 2011 in our Lymphoma Clinic Center. The mRNA level of Yap (effector of Hippo pathway) and LATS1, LATS2 mRNA were detected by using real-time quantitative PCR...

Splenic marginal zone lymphoma (SMZL) accounts for less than 2% of all non-Hodgkin lymphomas. We identified 107 cases diagnosed with SMZL between 1985 and 2012 from the British Columbia Cancer Agency Centre for Lymphoid Cancer and Lymphoma Pathology Databases. Patient characteristics were: median age 67 years (range 30-88), male 40%, stage IV 98%, splenomegaly 93%, bone marrow involvement 96%, peripheral blood involvement 87%. As initial treatment, 52 underwent splenectomy (10 with chemotherapy), 38 chemotherapy alone (21 chemoimmunotherapy containing rituximab, 1 rituximab alone), two antivirals for hepatitis C, and 15 were only observed...

Chronic infection with hepatitis C virus (HCV) may lead to B cell activation and transformation into non-Hodgkin lymphoma (NHL). Molecular mechanisms of B cell transformation by HCV are poorly understood. One of the most common lymphoproliferative disorders in HCV-infected patients is splenic marginal zone lymphoma (SMZL). A case of a 42-years old man, affected by HCV-related SMZL, effectively treated with an IFN-free, NS3-NS4A inhibitor-based regimen, is hereby described. The patient was treated for 16 weeks with faldaprevir, deleobuvir, and ribavirin, achieving a very rapid viral eradication without relevant toxicities...

INTRODUCTION: Splenic marginal zone lymphoma (SMZL) is a rare low-malignant Non-Hodgkin lymphoma (NHL), in which immune mediated paraneoplastic phenomena such as autoimmune hemolytic anemia (AIHA), autoimmune thrombocytopenia (ITP) and C1 esterase inhibitor deficiency are relatively common. MATERIALS AND METHODS: We performed a multicenter retrospective study in 70 patients on the prevalence and clinical features of antiphospholipid antibodies (aPLA) in SMZL. RESULTS AND CONCLUSIONS: Nine patients (13%) had the diagnosis of a lupus anticoagulant (LA)...

BACKGROUND: Marginal zone lymphoma (MZL), comprised of nodal, extranodal, and splenic subtypes, accounts for 5%-10% of non-Hodgkin lymphoma cases. A detailed evaluation of the independent effects of risk factors for MZL and its subtypes has not been conducted. METHODS: Data were pooled from 1052 MZL cases (extranodal [EMZL] = 633, nodal [NMZL] = 157, splenic [SMZL] = 140) and 13766 controls from 12 case-control studies. Adjusted unconditional logistic regression was used to compute odds ratios (ORs) and 95% confidence intervals (CIs)...

Rupture of the spleen is a potentially life-threatening condition, which most often occurs secondary to abdominal trauma. Spontaneous rupture of the spleen is a much rarer event, usually occurring secondary to infections and less frequently secondary to haematological malignancies causing massive splenomegaly. We present a case of a 71-year-old woman who presented in the emergency department with acute abdominal and back pain and no history of trauma, with a CT scan diagnosis of splenic rupture. Splenectomy was performed and the histological examination of the specimen revealed splenic marginal cell lymphoma (SMZL), which is classified under the non-Hodgkins lymphomas (NHL) and accounts for <1% of NHL...

PURPOSE: This study sought to characterize the imaging features of primary splenic lymphoma (PSL). MATERIALS AND METHODS: Pathological and imaging data from 17 patients with primary splenic lymphoma initially diagnosed at splenectomy were retrospectively analyzed. Pretreatment computed tomography (CT) imaging was available for 16 patients, and magnetic resonance imaging (MRI) data were available for 4 patients. Splenic lymphoma imaging data were categorized based on the gross pathological presentation in the following manner: type 1, homogeneous enlargement; type 2, miliary nodules; type 3, multifocal masses of varying size; and type 4, solitary large mass...

Marginal zone lymphoma (MZL) is an indolent B-cell lymphoma arising from marginal zone B-cells present in lymph nodes and extranodal tissues. MZL comprises 5-17% of all non-Hodgkin's lymphomas in adults. The World Health Organization categorizes MZL into three distinct types based on their site of impact: (1) splenic marginal zone lymphoma (SMZL); (2) nodal marginal zone lymphoma (NMZL); (3) extranodal mucosa-associated lymphoid tissue (MALT) lymphoma, which can be subdivided into gastric and nongastric. The subgroups of MZL share some common features but are different in their biology and behavior...

Therapy of indolent lymphomas with involvement of the central nervous system (CNS) has not been standardized so far. A 42-year old male patient presented with neurological signs because of leukemic splenic marginal zone lymphoma (SMZL) manifested in bone marrow, lymph nodes and CNS. Due to the aggressiveness of the disease and the young age of the patient, an intensive immunochemotherapy followed by high-dose therapy with busulfan, thiotepa and fludarabine and subsequent unrelated allogeneic stem cell transplantation (alloSCT) was performed...

The epidemiology of splenic marginal zone lymphoma (SMZL) in the United States has not been addressed. Eight years of data (2001-2008) from 17 registries of the Surveillance, Epidemiology and End Results (SEER) program were used for this study. Of the 116,411 cases of non-Hodgkin lymphoma (NHL) in the registries, 763 (0.6%) were SMZL. The overall annual age-adjusted incidence was 0.13 per 100,000 persons per year. The annual percent change in age-adjusted incidence was 4.81% overall (p < 0.05), and significantly increasing trends were found for patients who were white, male or aged 70 years and older (p < 0...

Follicular lymphoma (FL) is an indolent lymphoma with long median survival. Many studies have been performed to build up prognostic scores potentially useful to identify patients with poorer outcome. In 2004, an international consortium coordinated by the International Follicular Lymphoma Prognostic Factor project was established and a new prognostic study was launched (FLIPI2) using progression-free survival (PFS) as main endpoint and integrating all the modern parameters prospectively collected. Low-grade non-Hodgkin lymphomas were once considered as a heterogenous group of lymphomas characterized by an indolent clinical course...

Splenic marginal zone lymphomas (SMZL) are an uncommon type of B-cell non-Hodgkin's lymphoma (NHL-B) in which no specific chromosomal translocations have been described. In contrast, the most frequent cytogenetic abnormality is the loss of the long arm of chromosome 7 (7q). Previous reports have located this loss in the 7q32 region. In order to better characterize the genomic imbalances in SMZL, molecular studies were carried out in 73 patients with SMZL. To gain insight into the mapping at 7q a tiling array was also used...

BACKGROUND: Kaposi's sarcoma-associated herpes virus is associated with primary effusion lymphoma and multicentric Castleman's disease. METHODS: Seropositivity to lytic and latent Kaposi's sarcoma herpes virus (KSHV) antigens were examined in 2083 lymphomas and 2013 controls from six European countries. RESULTS: Antibodies against KSHV latent and lytic antigens were detectable in 4.5% and 3.4% of controls, respectively, and 3.6% of cases (P>0...

Splenic marginal zone lymphoma (SMZL) is a rare malignant lymphoma involving marginal zone B cells, accounting for only 1% of non-Hodgkin lymphomas. No previous reports have documented SMZL accompanied by massive bloody pleural effusion. Herein, the case of a patient with SMZL that was only diagnosed after splenectomy and thoracoscopic pleural biopsy is presented. SMZL in this patient was accompanied by massive bloody pleural effusion. The characteristics of the patient's pleural effusion changed from bloody to transudative after splenectomy, and rapid improvement was observed...

New therapeutic modalities for B-cell non-Hodgkin's lymphomas (B-NHL) are needed, especially for relapsing and aggressive subtypes. Toward this end, we previously generated a fully CD20-targeted and armed measles virus, and tested its efficacy in a xenograft model of mantle cell lymphoma (MCL). Here, we quantify its spread in peripheral blood mononuclear cells and/or tissue of patients with different histological subtypes of B-NHL, including splenic marginal zone lymphoma (SMZL). CD20-targeted MV efficiently infects lymphoma cells from SMZL and MCL while sparing most cells in the CD20-negative population, in contrast to the parental vaccine-lineage MV, which infects CD20-positive and CD20-negative cells equally...

Antigen stimulation may be important for splenic marginal zone lymphoma pathogenesis. To address this hypothesis, the occurrence of stereotyped B-cell receptors was investigated in 133 SMZL (26 HCV+) compared with 4,414 HCDR3 sequences from public databases. Sixteen SMZL (12%) showed stereotyped BCR; 7 of 86 (8%) SMZL sequences retrieved from public databases also belonged to stereotyped HCDR3 subsets. Three categories of subsets were identified: i) "SMZL-specific subsets" (n=5), composed only of 12 SMZL (9 HCV-from our series); ii) "Non-Hodgkin's lymphoma-like subsets" (n=5), comprising 5 SMZL (4 from our series) clustering with other indolent lymphomas; iii) "CLL-like subsets" (n=6), comprising 6 SMZL (3 from our series) that belonged to known CLL subsets (n=4) or clustered with public CLL sequences...