Extranodal Marginal Zone B-cell Research Articles

IBCL-151: Epidemiological Data on Marginal Zone B-Cell Lymphoma in the Republic of Kosovo During the Period of 2016-2020

Context Marginal zone B-cell lymphomas (MZLs) are a group of clinically indolent B-cell lymphomas postulated to derive from memory B lymphocytes in the 'marginal zone’ of secondary lymphoid tissue. It has several distinct entities including extra nodal marginal zone B-cell lymphoma (EMZL) or mucosa-associated lymphoid tissue (MALT) lymphoma, splenic MZL, and nodal MZL. This type of lymphoma is one of the most common lymphoma types in Kosovo. This epidemiological study will reveal new data on the success of treatment of this disease in Kosovo and how we can improve our treatment in the future. Design Retrospective descriptive epidemiological study. The data was collected during the 5 year period of 2016-2020 from patient files. Setting The data was collected from the patient files in the hematology clinic of the university clinical center of Kosovo. Patients Adults, age 18 and older, both genders diagnosed with marginal zone B-cell lymphoma in the hematology clinic of the university clinical center of Kosovo. Interventions None. Main Outcomes Measures No main outcome because it is an epidemiological study. Results During the period of January 2016 to December 2020, 31 patients were diagnosed and treated with marginal zone B-cell lymphoma. The median age of the patients was 59.2 years old. 14 (45.2%) were male and 17 (54.8%) were female. MALT marginal zone B-cell lymphoma was the most common entity with 10 patients, 10 were splenic marginal zone lymphoma, 6 were nodal and 4 were EMZL. Conclusions The majority of patients (30) were treated with R-CHOP chemotherapy protocol. Only one was not treated at all after a PET scan showed no metabolically active lesions and the watch and wait method was applied. 24 patients went into remission and received rituximab maintenance therapy every 3 months, 3 patients had progressive disease after 6 cycles of R-CHOP, and there is no data on the outcome of 2 patients.

Rare case of gastric extranodal marginal zone lymphoma

True histiocytic lymphoma is considered a rare entity, and its diagnosis requires the concordance of morphological, immunophenotypic, and molecular findings. Gastric extra nodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) is a B-cell non-Hodgkin lymphoma that arises in the stomach and has a perifollicular/marginal zone growth pattern. The lymphoma is derived from marginal zone B-cells and recapitulates the architecture and organization of native MALT exemplified by the Peyers’ patches in the terminal ileum. Marginal zone lymphoma of MALT (MALT lymphoma) is the most common indolent subtype and represents 7% of all non-Hodgkin lymphomas.

Mucosa-Associated Lymphoid-Tissue Lymphoma of the Descending Colon in a Patient with Blastocystis hominis Infection: A Case Report and Review of Literature

Mucosa-associated lymphoid tissue (MALT) lymphoma is an extra nodal marginal zone B-cell lymphoma that has been associated with chronic infections. It affects especially stomach, but other organs of gastrointestinal tract can be also involved. Colon MALT lymphoma is a very rare disease. We report a case of large bowel MALT lymphoma diagnosed as a result of weight loss and positive occult fecal blood test. Blastocystis hominis infection was detected in fecal analysis. We hypothesize chronic parasitic infection could be related with the development of the lymphoma.

Targeted Metabolomics Analysis in Follow-up of Classic and Mild PKU using LC-ESI-MS/MS: Assessment of Metabolic Control through PHE/TYR ratio

MALT lymphoma, also referred to as extra nodal marginal zone B-cell lymphoma, is a low-grade malignant non-Hodgkin lymphoma...

Mucosa-Associated Lymphoid-Tissue Lymphoma of the Cecum and Rectum: A Case Report.

A colonic mucosa-associated lymphoid-tissue (MALT) lymphoma is relatively rare compared to lymphomas of the stomach or small intestine. We present a case of a MALT lymphoma in the cecum and rectum found during screening colonoscopy. A 54-year-old female, who had undergone right-breast-conserving surgery with axillary dissection due to an invasive ductal carcinoma and a left-breast excisional biopsy due to microcalcification following adjuvant chemoradiation therapy 3 years earlier, was found to have 3-mm-sized smooth elevated lesions in both the cecum and rectum. No pathologic lesion or lymphadenopathy was found at any other site, but chronic gastritis negative for Helicobacter pylori infection was found. The polyps were removed by using an endoscopic biopsy and revealed an extra nodal marginal zone B-cell MALT lymphoma, showing positive for CD3 and CD20 by immunohistochemical staining. The patient underwent close observation without any additional treatment and has shown no evidence of recurrence as of her last visit.

Distribution of 9p24 Abnormalities in Hematologic Malignancies with an Emphasis on Diffuse Large B-Cell Lymphoma

Introduction: 9p24 contains genes that are critical for immune evasion and propagating cell division. The loci for PD-L1, PD-L2, JAK2, and the histone demethylases KDM4C/JMJD2C are linked on 9p24 (Van Roosbroeck et al. Genes, Chromosomes and Cancer 2016). Amplification or rearrangements of this region have been described in the pathogenesis of classical Hodgkin lymphoma (cHL) and primary mediastinal large B-cell lymphoma (Ansell et al. New England Journal of Medicine 2015). Additionally, JAK2 amplification up-regulates PD-L1 and L2, which leads to increased T-cell inactivation and suggests synergy between these drug targets. The recent success of PD-1 blockade in numerous malignancies has led to the development and approval of PD-1 inhibitors in cHL as well as other cancers. Targeted therapies are approved for JAK2 inhibition, such as ruxolitinib, and are in development for histone demethylases, which illustrates the utility of identifying the 9p24 amplicon in hematologic malignancies (HM) (Van Roosbroeck et al. Genes, Chromsomes and Cancer 2016). The goal of this analysis is to better understand the distribution of 9p24 abnormalities across a broader range of leukemias and lymphomas in order to facilitate future studies of targeted therapy.Methods: The National Cancer Institute's Mitelman Database of Chromosome Aberrations and Gene Fusions in Cancer was queried for 9p24 breakpoint abnormalities within HM. Incidence of additions and rearrangements in chromosome 9p24 for all subtypes of HM listed in the Mitelman Database were calculated. Individual references were manually reviewed and pathologic data was extracted as available from the primary sources. Diffuse large B-cell lymphoma (DLBCL) cases were further assessed for co-incident rearrangement of MYC (8q24), BCL2 (18q21) and BCL6 (3q27) with 9p24. All subtypes with greater than 2% incidence of additions and/or rearrangements in chromosome 9p24 were reported.Results: 48,761 patients (pts) with HM across 74 lymphoid and myeloid subtypes were identified. 361 (<1%) pts had additions or rearrangements involving 9p24 across 53 subtypes of HM. 21 subtypes of HM had no 9p24 abnormalities. PTCL NOS had the highest incidence of aberrations (3.1% (6/193), 1.1% with additions and 2% with rearrangements) followed by cHL (2.7% (5/186), all additions) and DLBCL (2.7% (37/1413), 1.5% additions and 1.2% rearrangements) in malignancies with over 100 cases. Among DLBCL cases 9p24 was co-amplified/rearranged with MYC in 22% (8 cases), BCL6 27% (10), BCL2 14% (5) and in 3% (1) double-hit lymphomas. Minimal data was available on cell of origin; therefore, further analysis was not performed. No cases of DLBCL with 9p24 aberrations were T-cell rich DLBCL’s (0/37). Among the rarer lymphomas, extranodal NK/T cell lymphoma (11%, 2/18) and intravascular B cell lymphoma (7%, 1/14) had higher incidences of 9p24 additions or rearrangements (Table 1).Conclusion: Amplifications and rearrangement of 9p24 are rare in HM within the Mitelman database. Our study suggests that patients with DLBCL, adult T-cell leukemia/lymphoma, extranodal NK/T cell lymphoma, intravascular B cell lymphoma, peripheral T-cell lymphoma, and extranodal marginal zone B-cell may be considered for further studies with FISH and aCGH to further define the incidence of 9p24 alterations and potentially for targeted clinical trials. 9p24 abnormalities did not correlate with previously described phenotypic subtypes of DLBCL. [Display omitted] DisclosuresNo relevant conflicts of interest to declare.

Acute Kidney Injury in a Patient With Type II Cryoglobulinemia From Extra Nodal Marginal Zone B-Cell Lymphoma (MALT): Case Report and Review of the Literature

Acute Kidney Injury in a Patient With Type II Cryoglobulinemia From Extra Nodal Marginal Zone B-Cell Lymphoma (MALT): Case Report and Review of the Literature

EPID-29DURAL PRIMARY MARGINAL ZONE LYMPHOMA WITH LARGE B-CELLS PRESENT: A CASE REPORT AND REVIEW OF THE LITERATURE

A 64-year-old female presented with personality changes, acalculia, falls, headache and nausea. CT and MR imaging of the head revealed a homogeneously contrast-enhancing 7 cm x 4.4 cm x 4.4 cm right frontal dural-based mass, concerning for a meningioma. She underwent a right frontal craniotomy with complete resection. Post-operative pathology demonstrated sheets of lymphocytes and plasma cells with small numbers of germinal cells present. The infiltrate was limited to the dura and leptomeninges. Immunophenotype as demonstrated by immunohistochemistry and flow cytometry was diagnostic of an extranodal marginal zone lymphoma with extensive plasmacytic differentiation. Given the normal bone marrow biopsy and absence of disease on CT chest, abdomen, and pelvis, she was diagnosed with an extranodal primary dural-based marginal zone B-cell lymphoma (MZL) with plasmacytic differentiation. Primary dural lymphomas are a well-described subset of primary central nervous system (CNS) lymphomas that most commonly present as low grade lymphomas. MZLs generally carry a much better prognosis than typical diffuse large B-cell lymphomas. This case illustrates that hematopoietic malignancies should be considered in the differential diagnosis of dural based masses in addition to meningiomas or less common dural metastases, solitary fibrous tumors, leiomyosarcomas, and hemangiopericytomas. Typically dural based lymphomas have increased vasogenic edema compared to meningiomas. Unlike other extranodal MZLs, dural based MZLs have no strong association with chronic inflammatory conditions, infections, or immunosuppression. The literature supports that MLZs are very radiosensitive; with excision and low dose-localized radiation therapy, patients may remain disease free locally. However, systemic recurrence may occur, possibly because the dura is outside the blood brain barrier. Appropriate diagnosis is important as it has both prognostic and treatment implications.

Clinical characteristics and treatment analysis of primary ocular adnexal lymphoma

Objective To study the clinical characteristics and analyze the treatment of primary ocular adnexal lymphoma (POAL). Methods A retrospective review was performed based on the clinical records of 26 POAL cases, who were treated from June 1999 to June 2009. The clinical manifestations,imaging features, diagnosis approaches and treatment of them were analyzed. Results The main symptoms were a slowly growing painless mass, exophthalmia and red eyelid. Mostly, imaging finding shows casting like. 50 % of POAL was at the orbit. 22 patients w ere B-cell origin [13 patients were extranodal marginal zone B-cell lymphoma-mucosal-associated lymphoid tissue (MALT) lymphoma], 2 patients were NK/T cell lymphoma. 76.9 % patients were in stage Ⅰ -Ⅱ. Surgery, radiotherapy and chemotherapy were most common treatment modalities for POAL. Conclusion Most POAL were low-grade B-cell lymphoma, with MALT by far the most common type. POAL was easy to be misdiagnosed due to non specific clinical manifestations.Imaging finding was helpful to diagnosis, but could not reliably distinguish between benign and malignant. The invasive biopsy is the main diagnosis approach. Radiotherapy had been the standard treatment for low-grade POAL. High-grade or infiltrating peri-orbit need combine chemotherapy. lmmunotherapy and molecular targeted therapy were the direction of future research. Key words: Lymphoma; Eye neoplasms; Therapy

Reduced-dose (two-thirds) R-CHOP chemotherapy for elderly patients with non-Hodgkin lymphoma

Elderly patients with non-Hodgkin lymphoma (NHL) have a poor prognosis. Owing to treatment-related toxicities, there is no standard chemotherapy for the elderly patients, especially those aged 70 years or older. In this study, we retrospectively evaluated the efficacy and toxicity of reduced-dose (two-thirds) R-CHOP chemotherapy as an initial chemotherapy for 45 patients aged 70 years or older with B-cell NHL. The WHO classification of NHL included diffuse large B-cell lymphoma (DLBCL) (31), mantle cell lymphoma (5), follicular lymphoma (4), extranodal marginal zone lymphoma (1), Burkitt lymphoma (1), and B-cell lymphoma whose further types were unclassified (3). The incidences of grade 4 neutropenia and febrile neutropenia (FN) were 51·1 and 15·6%, respectively. Efficacy was evaluated in patients with DLBCL. The overall and complete response (CR) rates were 96·7 and 90·0%, respectively. Two-year event-free survival (EFS) and overall survival (OS) were 84·4 and 89·2%, respectively. There was no treatment-related mortality. In conclusion, two-thirds R-CHOP chemotherapy is a promising treatment for elderly patients with B-cell NHL in terms of its efficacy and toxicity.

Cavitary pulmonary involvement of diffuse large B-cell lymphoma transformed from extra nodal marginal zone B-cell lymphoma MALT type

We describe a case of pulmonary diffuse large B-cell lymphoma (DLBCL), which was thought to arise from extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma). A 68-year-old woman presented with a 2-month history of cough and bloody sputum. The chest X-ray and computed tomography revealed a mass with cavitation in the right lower lobe. Transbronchial biopsy specimens revealed a granulomatous infiltration without malignant cells. However, diagnosis of MALT lymphoma was established from gastric biopsy specimen. Subsequently, a right lower lobectomy was performed because of hemoptysis. Examination of the resected specimen revealed a diffuse large B-cell lymphoma, which was considered to have transformed from MALT lymphoma, because both lung and stomach lesions had the chromosomal translocation t(11;18)(q21;q21) in common. In addition, there were no nodules, masses, alveolar or interstitial infiltrates in the lung fields, which are usually observed in the case of marginal zone B-cell lymphoma of bronchial mucosa-associated lymphoid tissue. These findings indicate that involvement of DLBCL have to be considered in patients with MALT lymphoma and cavitary lesion of the lung.