Multiple lymphomatous polyposis(MLP) is an interesting clinical entity of non-Hodgkin's lymphoma(NHL) and is defined as B-cell lymphoma characterized by the presence of multiple lymphomatous polyps along the gastrointestinal tract. Recently MLP has been considered as a variant form of mantle cell lymphoma(MCL). The median survival of patients with MCL is only 3 years, and none of the available conventional chemotherapy regimens appears curative. Encouraging results have been reported with high dose chemotherapy with autoSCT and alloSCT for its treatment. We introduce 4 cases of MLP diagnosed as MCL by morphologic and immunologic method. The common clinical findings of these cases were splenomegaly (4/4), multiple intraabdominal lymphadenopathy (4/4), and advanced stage (3/4) at presentation. The overall remission duration was relatively short (5-27 months) and three of four cases relapsed after conventional chemotherapy or autologous stem cell transplant. Our report suggests that MCL presented as MLP is a high risk subgroup of NHL and more aggressive approach may be needed for cure.
Drug Therapy ; Gastrointestinal Tract ; Humans ; Lymphatic Diseases ; Lymphoma ; Lymphoma, B-Cell ; Lymphoma, Mantle-Cell* ; Polyps ; Splenomegaly ; Stem Cells

Multiple lymphomatous polyposis (MLP) is a term used to describe malignant lymphoma of the gastrointestinal tract that manifest as polyposis. MLP is believed to represent gastrointestinal involvement by mantle cell lymphoma (MCL), primarily based on its histologic and immunophenotypic similarities with MCL. We have experienced a case of MCL presented as a peculiar feature of multiple lymphomatous polyposis in a 49-year old male patient. He had been diagnosed diffuse large B cell lymphoma of nasopharynx and treated by radiation therapy and CHOP chemotherapy. He newly developed MCL after 10 years in complete remission of diffuse large B cell lymphoma. After newly developed lymphoma was compared with former diagnosed lymphoma, we concluded that they were not same type each other. We reviewed a total of 13 patients including this case, of MLP, which were reported in various Korean literature, and literatures are reviewed.
B-Lymphocytes* ; Drug Therapy ; Gastrointestinal Tract ; Humans ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, Mantle-Cell ; Male ; Middle Aged ; Nasopharynx

Congresses as Topic ; Drug Delivery Systems ; Hematology ; Humans ; Lymphoma, Mantle-Cell ; drug therapy ; United States

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Humans ; Interferons ; Lymphoma, Mantle-Cell ; drug therapy ; Recurrence ; Thalidomide

Mantle cell lymphoma is a subtype of aggressive non-Hodgkin's lymphoma and usually presents in advanced stages. When treated with a regimen such as CHOP, the median survival is about three years. More aggressive front-line therapy with the hyper-CVAD regimen or high-dose therapy with stem cell support has shown encouraging results in several trials. The addition of rituximab to the chemotherapy regimens, or high-dose therapy, increased the response and survival rates in patients with mantle cell lymphoma. We report a case of mantle cell lymphoma that was successfully treated with aggressive front-line treatment strategies. The patient achieved complete remission with initial hyper-CVAD regimen, and was consolidated with autologous stem cell transplantation and subsequent rituximab.
Drug Therapy ; Humans ; Lymphoma, Mantle-Cell* ; Lymphoma, Non-Hodgkin ; Stem Cell Transplantation* ; Stem Cells* ; Survival Rate ; Rituximab

Humans ; Cardiovascular Diseases/drug therapy* ; Consensus ; Lymphoma, B-Cell ; Lymphoma, Mantle-Cell ; Tyrosine Protein Kinase Inhibitors/therapeutic use*

PURPOSE: Mantle cell lymphoma (MCL) is known to have systemic dissemination with poor prognosis, but very few cases have been reported in Korea. Therefore, we analyzed clinical features and prognosis of MCL by comparing 3 cases of ocular adnexal MCL and mucosa-associated lymphoid tissue (MALT) lymphoma. METHODS: The medical records of patients with ocular adnexal MALT lymphoma and MCL from Chungnam National University Hospital and Pusan National University Hospital from January 1999 to April 2014 were retrospectively reviewed. Immunohistochemical examination was selectively used to diagnose lymphoma subtypes. Systemic dissemination, treatment response, and recurrence were identified using radiological testing and bone marrow aspiration. RESULTS: This study included 39 patients with ocular and ocular adnexal MALT lymphoma and 1 patient with MCL from Chungnam National University Hospital and 53 patients with MALT lymphoma and 2 patients with MCL from Pusan National University Hospital. All 3 (100%) patients diagnosed with ocular adnexal MCL were over 60 years of age. However, 28 of 92 (30.4%) patients diagnosed with MALT lymphoma were over 60 years of age. In MALT lymphoma, 3 of 92 patients presented with systemic dissemination and most patients recovered with radiotherapy. Conversely, all 3 patients with MCL showed systemic dissemination and recurrence after radiotherapy or chemotherapy, therefore, additional chemotherapy was required. CONCLUSIONS: Contrary to MALT lymphoma, ocular and ocular adnexal MCL usually presented with systemic dissemination and complete remission was difficult even though many regimens of chemotherapy were attempted. Because MCL can be misdiagnosed as MALT lymphoma, careful evaluation should be performed.
Bone Marrow ; Busan ; Chungcheongnam-do ; Drug Therapy ; Humans ; Korea ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Lymphoma, Mantle-Cell* ; Medical Records ; Prognosis ; Radiotherapy ; Recurrence ; Retrospective Studies

Humans ; Interferons ; administration & dosage ; therapeutic use ; Lymphoma, Mantle-Cell ; drug therapy ; Male ; Middle Aged ; Thalidomide ; administration & dosage ; therapeutic use

Objective: To examine the clinical characteristics and prognostic factors of elderly patients with mantle cell lymphoma (MCL) and the impact of nutrition and underlying diseases on the prognosis of elderly patients with MCL. Methods: retrospectively analyzed 255 elderly patients with MCL from 11 medical centers, including Peking University Third Hospital between January 2000 and February 2021. We analyzed clinical data, such as age, gender, Mantle Cell Lymphoma International Prognostic Index score, and treatment options, and performed univariate and multivariate prognostic analysis. We performed a comprehensive geriatric assessment on elderly MCL patients with medical records that included retraceable underlying disease and albumin levels, and we investigated the impact of basic nutrition and underlying disorders on MCL prognosis in the elderly. Results: There were 255 senior individuals among the 795 MCL patients. Elderly MCL was more common in males (78.4%), with a median age of 69 yr (ages 65-88), and the majority (88.6%) were identified at a late stage. The 3-yr overall survival (OS) rate was 42.0%, with a 21.2% progression-free survival (PFS) rate. The overall response rate (ORR) was 77.3%, with a 33.3% total remission rate. Elderly patients were more likely than younger patients to have persistent underlying illnesses, such as hypertension. Multivariate analysis revealed that variables related with poor PFS included age of ≥80 (P=0.021), Ann Arbor stage Ⅲ-Ⅳ (P=0.003), high LDH level (P=0.003), involvement of bone marrow (P=0.014). Age of ≥80 (P=0.001) and a high LDH level (P=0.003) were risk factors for OS. The complete geriatric assessment revealed that renal deficiency was associated with poorer OS (P=0.047) . Conclusions: Elderly MCL patients had greater comorbidities. Age, LDH, renal function, bone marrow involvement, and Ann Arbor stage are all independent risk factors for MCL in the elderly.
Male ; Adult ; Humans ; Aged ; Lymphoma, Mantle-Cell/drug therapy* ; Prognosis ; Retrospective Studies ; Bone Marrow/pathology* ; Risk Factors

There is growing evidence that crosstalk between mantle cell lymphoma (MCL) cells and stromal microenvironments, such as bone marrow and secondary lymphoid tissues, promotes tumor progression by enhancing survival and growth as well as drug resistance of MCL cells. Recent advances in the understanding of lymphoma microenvironment have led to the identification of crucial factors involved in the crosstalk and subsequent generation of their targeted agents. In the present study, we evaluated the combinatory effect of blocking antibodies (Ab) targeting CXCR4 and VLA-4, both of which were known to play significant roles in the induction of environment-mediated drug resistance (EMDR) in MCL cell line, Jeko-1. Simultaneous treatment with anti-CXCR4 and anti-VLA-4 Ab not only reduced the migration of Jeko-1 cells into the protective stromal cells, but also enhanced sensitivity of Jeko-1 to a chemotherapeutic agent to a greater degree than with either Ab alone. These combinatorial effects were associated with decreased phosphorylation of ERK1/2, AKT and NF-kappaB. Importantly, drug resistance could not be overcome once the adhesion of Jeko-1 to the stromal occurred despite the combined use of Abs, suggesting that the efforts to mitigate migration of MCLs should be attempted as much as possible. Our results provide a basis for a future development of therapeutic strategies targeting both CXCR4 and VLA-4, such as Ab combinations or bispecific antibodies, to improve treatment outcomes of MCL with grave prognosis.
Antibodies, Bispecific ; Antibodies, Blocking ; Bone Marrow ; Cell Line ; Drug Resistance ; Drug Therapy* ; Integrin alpha4beta1* ; Lymphoid Tissue ; Lymphoma ; Lymphoma, Mantle-Cell* ; NF-kappa B ; Phosphorylation ; Prognosis ; Stromal Cells