High-Grade B-Cell Lymphoma (HGBCL) with gene rearrangements in MYC and BCL2 and/or BCL6 is an aggressive malignancy usually presenting in advanced stages. Current recommendations suggest the use of regimens more intensive than R-CHOP (rituximab, cyclophosphamide, vincristine, doxorubicin, prednisone), which are based on retrospective studies and single-arm prospective trials that included patients who are mostly in the advanced stage, and did not receive consolidation radiotherapy.

The optimal approach and treatment of HGBCL, whether limited-stage (LS) or advanced-stage, remains to be determined. Here we describe the promising outcomes of three patients with LS and low IPI HGBCL with the use of R-CHOP as induction chemotherapy regimen, which was followed by consolidation radiotherapy.

Three women, 54-, 60-, and 64-years of age diagnosed to have HGBCL with MYC, and BCL2 and/or BCL6 rearrangements, with Ann Arbor stages I-IIE were included in this case series. All three patients had complete metabolic response to 6 cycles of R-CHOP and was subsequently treated with consolidation involved site radiotherapy (ISRT; total dose 30-36 Gy). Chemotherapy and radiotherapy were tolerated very well. All patients remain to be in remission, with the longest being at 23 months.

Outcomes of patients with HGBCL generally remain to be poor, but this may not be the case for patients with limited-stage disease and favorable clinicopathologic risk profile. Nevertheless, the treatment of HGBCL is currently evolving and more studies are needed to determine the ideal approach and preferred chemotherapy regimen. Also, more studies are needed to elucidate the potential role of consolidation radiotherapy in patients with limited-stage HGBCL to improve survival outcomes. Findings of this case series suggest that patients with LS HGBCL may still derive benefit from R-CHOP followed by consolidation ISRT, but prospective trials are needed to confirm this.

<b>Objective:b> This study aimed to see how different initial treatment regimens affected the long-term prognosis of patients with extranodal marginal zone mucosa-associated lymphoid tissue lymphoma confining to the ocular adnexal (OAML) . <b>Methods:b> Between April 2008 and April 2019, 109 patients with initial mucosa-associated lymphoid tissue confining to ocular adnexal were evaluated and followed-up, and the prognosis of various initial treatment regimens were examined. <b>Results:b> A total of 36 patients underwent complete surgical resection of the lesions, and 73 patients had residual lesions after surgery, of which 37 patients chose watchful waiting, and 36 patients chose treatment. The treatment regimen included local radiotherapy and systemic treatment (chemotherapy, immunochemotherapy, the combination of radiotherapy and chemotherapy, etc.) , and no serious toxic and side effects were observed in patients receiving systemic treatment. The median follow-up time was 61 (10-142) months. The 5-year and 10-year progression-free survival (PFS) of monocular involvement patients were 78.2% and 76.0% . The 5-year and 10-year PFS rates of patients with binocular involvement were 64.4% and 23.5%. There was significant diference in PFS between patients with monocular and binocular involvement (P=0.010) . Patients who received additional treatment had higher PFS than those patients in the watchful waiting group (P=0.046) . The 5-year PFS was 71.4% and 90.1% among patients in the watchful waiting group and those who received additional treatment, whereas the 10-year PFS was 63.5% and 75.1% , respectively. Patients with OAML were still a risk of disease progression after 5 years. <b>Conclusions:b> Patients with binocular involvement OAML at the start of the disease had a poor prognosis, but treatment could reduce the risk of recurrence/progression. Systemic therapy is one of the first-line treatment options for patients with OAML, who require long-term monitoring.
Eye Neoplasms/radiotherapy* ; Humans ; Lymphoid Tissue/pathology* ; Lymphoma, B-Cell, Marginal Zone/therapy* ; Prognosis ; Retrospective Studies ; Treatment Outcome

BACKGROUND/AIMS: The eradication of Helicobacter pylori (H. pylori) is an effective treatment in gastric mucosa-associated lymphoid tissue (MALT) lymphoma associated with H. pylori infection. However, the treatment strategy in gastric MALT lymphoma patients who are H. pylori-negative or unresponsive to H. pylori eradication therapy remains controversial. In this study, we investigated the clinical efficacy of treatments other than H. pylori eradication therapy in these groups of patients. METHODS: This was a retrospective single-center study based on the medical records of patients diagnosed with gastric MALT lymphoma at Yeungnam University Medical Center between January 2005 and December 2016. Patients were treated with H. pylori eradication therapy, chemotherapy, or radiotherapy according to their H. pylori infection status and stage of gastric MALT lymphoma. RESULTS: Of the 68 eligible patients, 50 were enrolled in the study. Of the 42 patients with H. pylori-positive gastric MALT lymphoma, 36 (81.7%) were treated with H. pylori eradication therapy as primary treatment and 25 (69.4%) achieved a complete response (CR). Patients without a CR after H. pylori eradication therapy (n=11, 30.6%) received radiotherapy as a secondary treatment. Two patients with H. pylori-positive gastric MALT lymphoma and eight with H. pylori-negative gastric MALT lymphoma received radiotherapy as the primary treatment. CR was achieved in all 21 patients treated with radiotherapy as primary or secondary treatment. The 5-year progression-free survival rate after radiotherapy was 92.9%. CONCLUSIONS: Radiotherapy may be a worthwhile treatment option in patients with H. pylori-negative MALT lymphoma or H. pylori-positive MALT lymphoma that is not responsive to H. pylori eradication therapy.
Academic Medical Centers ; Disease-Free Survival ; Drug Therapy ; Helicobacter pylori* ; Helicobacter* ; Humans ; Lymphoid Tissue ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Medical Records ; Radiotherapy* ; Retrospective Studies ; Stomach Neoplasms ; Treatment Outcome*

The stomach is the most common primary site of an extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) type, which is characterized by an indolent clinical course. A diagnosis of gastric MALT lymphoma requires an endoscopic biopsy that should be confirmed by an experienced pathologist. Gastric MALT lymphoma shows a variable endoscopic appearance, including erosion, erythema, discoloration, atrophy, ulcer, and subepithelial lesion. The distribution is often multifocal. Therefore, clinical suspicion and multiple biopsies are essential for an accurate diagnosis. Gastric MALT lymphoma is almost invariably associated with a Helicobacter pylori (H. pylori) infection. H. pylori eradication therapy is the mainstay of treatment, which must be delivered to all patients regardless of the H. pylori infection status or stage. For patients who have failed to achieve remission following eradication therapy, radiotherapy or chemotherapy can be considered. Radiotherapy is an effective treatment modality for a localized stage and shows excellent outcomes. In the presence of disseminated or advanced disease, chemotherapy and/or immunotherapy with the anti-CD20 monoclonal antibody, rituximab, can be applied. Treatment should be individualized according to the stage and symptoms, as well as the patients' preference. Given that the clinical course of gastric MALT lymphoma is usually indolent, watchful waiting may be an adequate strategy in selected cases where scheduled follow-up is guaranteed.
Atrophy ; Biopsy ; Diagnosis ; Drug Therapy ; Erythema ; Follow-Up Studies ; Helicobacter pylori ; Humans ; Immunotherapy ; Lymphoma, B-Cell, Marginal Zone ; Radiotherapy ; Rituximab ; Stomach ; Ulcer ; Watchful Waiting

PURPOSE: To retrospectively analyze dosimetric parameters of volumetric-modulated arc therapy (VMAT) and three-dimensional conformal radiotherapy (3D-CRT) delivered to extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue in the stomach (gastric MALT lymphoma) to find out advantages of VMAT and conditions for definite benefits of VMAT. MATERIALS AND METHODS: Fifty patients with stage I-II gastric MALT lymphoma received VMAT (n = 14) or 3D-CRT (n = 36) between December 2005 and April 2018. Twenty-seven patients were categorized according to whether the planning target volume (PTV) overlaps kidney(s). Dosimetric parameters were analyzed by dose-volume histogram. RESULTS: Radiation dose to the liver was definitely lower with VMAT in terms of mean dose (p = 0.026) and V15 (p = 0.008). The V15 of the left kidney was lower with VMAT (p = 0.065). For those with PTV overlapping kidney(s), the left kidney V15 was significantly lower with VMAT. Furthermore, the closer the distance between the PTV and kidneys, the less the left kidney V15 with VMAT (p = 0.037). Delineation of kidney(s) by integrating all respiratory phases had no additional benefit. CONCLUSIONS: VMAT significantly increased monitor units, reduced treatment time and radiation dose to the liver and kidneys. The benefit of VMAT was definite in reducing the left kidney V15, especially in geometrically challenging conditions of overlap or close separation between PTV and kidney(s).
Humans ; Kidney ; Liver ; Lymphoid Tissue ; Lymphoma, B-Cell, Marginal Zone ; Organs at Risk ; Radiotherapy, Conformal ; Radiotherapy, Intensity-Modulated ; Retrospective Studies ; Stomach

PURPOSE: The purpose of this study was to evaluate the clinical features and the long-term outcomes of primary conjunctival marginal zone B-cell lymphoma (MZBCL) patients who were treated with radiation therapy (RT). MATERIALS AND METHODS: Retrospective data of 79 patients with 121 primary conjunctival MZBCL lesions were collected from January 1, 2001 till June 30, 2014. All lesions were treated by local RT (26 Gy) with patient-specific customized lens-shielding device. RESULTS: The current Korean patients' cohort showed younger median age at diagnosis (38 years), great female preponderance (78.5%) and more frequent bilateral involvement (53.2%) than the previous studies. Following 26 Gy's RT, excellent clinical outcomes were achieved: 5-year rates of overall survival, local relapse-free survival, and contralateral relapse-free survival were 100%, 98.1%, and 91.5%, respectively. Two patients (2.5%) developed local relapse and five (6.3%) developed relapse at initially uninvolved contralateral conjunctiva with median interval of 52.9 months, and late adverse events of grade 2 and 3 occurred in seven (8.8%) and two (2.5%) patients, respectively. CONCLUSION: 26 Gy's RT was highly effective and safe, with the use of lens-shielding device, in treating patients with primary conjunctival MZBCL.
Cohort Studies ; Conjunctiva ; Diagnosis ; Female ; Humans ; Lymphoma, B-Cell, Marginal Zone* ; Radiotherapy ; Recurrence ; Retrospective Studies

Primary cutaneous plasmacytoma (PCP) is a very rare cutaneous B-cell lymphoma that arises primarily in the skin. A few cases of PCP have been reported previously. PCPs are histologically similar to cutaneous B-cell lymphoma and are characterized by dense monoclonal plasma cell infiltration in the dermis. PCP can manifest as a single lesion or as multiple lesions. A solitary lesion can be treated with surgical excision or local radiotherapy. We describe the case of a patient with primary cutaneous plasmacytoma that was treated with intralesional steroid injections.
Dermis ; Humans ; Lymphoma, B-Cell ; Plasma Cells ; Plasmacytoma* ; Radiotherapy ; Skin

Lymphoproliferative disorder in a posttransplant setting has emerged as a difficult problem in kidney transplantation (KT). Lymphoma involving adnexa of the eye has rarely been reported due to scarcity of lymphoreticular tissue in the ocular area. This report presents a case of a 37-year-old KT recipient who was diagnosed with conjunctival mucosa-associated lymphoid tissue lymphoma with a chief complaint of seeing black spots. Unlike other post-transplant lymphoproliferative diseases associated with the Epstein-Barr virus (EBV) reactivation via immunosuppression, the lesion was not related to the virus. The patient received radiotherapy with concomitant conversion from the tacrolimus to the sirolimus. Overall, the results presented herein indicate lymphoma may be an important differential diagnosis when KT recipients complain of ocular discomfort.
Adult ; Diagnosis, Differential ; Herpesvirus 4, Human ; Humans ; Immunosuppression ; Kidney Transplantation ; Kidney* ; Lymphoid Tissue* ; Lymphoma* ; Lymphoma, B-Cell, Marginal Zone ; Lymphoproliferative Disorders ; Radiotherapy ; Sirolimus ; Tacrolimus ; Transplant Recipients*

BACKGROUND: Primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (POML) is the most common subtype of lymphoma involving the eyes in Thailand. We sought to assess the characteristics and treatment outcomes of patients with POML in Thailand. METHODS: We retrospectively reviewed patient data and included patients diagnosed with POML between January 2004 and December 2016 at Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand. We collected and analyzed patients' clinical characteristics and treatment outcomes. RESULTS: Among 146 patients with lymphoma involving the eyes, 121 (82%) were diagnosed with POML. Sixty-four (52.9%) were women with median age 58 (range, 22–86) years. The most common presenting symptom was orbital mass (71.1%). Common sites of origin were the orbit (46.3%) and lacrimal gland (34.7%). At presentation, 22.3% of patients had bilateral eye involvement. About half of patients had stage I disease (N=59, 56.2%) and 20% had stage IV. Most patients (73.3%) had a low-risk International Prognostic Index. Radiotherapy was the main treatment for patients with limited-stage disease (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rates 80%, 77.3%, and 64.7% for stages I, II, and IV, respectively. Five-year progression-free survival (PFS) and overall survival were 66.1% and 94.0%, respectively. For patients with limited-stage disease, radiotherapy significantly improved PFS compared with treatment not involving radiotherapy (5-year PFS 89.9% vs. 37.3%, P=0.01). CONCLUSION: We revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.
Disease-Free Survival ; Female ; Humans ; Lacrimal Apparatus ; Lymphoma ; Lymphoma, B-Cell, Marginal Zone* ; Orbit ; Radiotherapy ; Retrospective Studies ; Thailand*

BACKGROUND: The rectum is a relatively uncommon site for lymphoma compared with other gastrointestinal sites; no consensus regarding management of primary rectal lymphoma (PRL) has been formed due to its limited frequency. We aimed to investigate clinical characteristics and treatment outcomes in patients with PRL in a single center patient cohort. METHODS: We retrospectively analyzed the results of 16 consecutive patients with PRL, identified and treated at the Asan Medical Center, Seoul, Korea between January 1993 and December 2014. RESULTS: These 16 patients with PRL constituted 0.8% of all non-Hodgkin's lymphoma patients (N=1,984). B-cell lymphomas (N=14) made up the majority of the series, and half of these were extranodal marginal zone lymphomas (ENMZL, N=7). Ten patients received systemic chemotherapy with (N=3) or without rituximab (N=7), and 4 of these received additional local therapy. The others received radiotherapy (N=3) or endoscopic mucosal resection (N=3). Twelve patients (75%) achieved complete response (CR) after first-line treatment. Event-free survival (EFS) and overall survival (OS) in stages IE and IIE were significantly longer compared with stages IVE (P=0.001 and P=0.001, respectively). All patients with ENMZL (N=7) achieved CR during or after initial treatment. CONCLUSION: PRL is very rare and seems to present mostly as B-cell type. Stage is the most important prognostic factor, with significantly better survival associated with localized diseases. ENMZL may be one of the most common types of PRL with favorable treatment outcomes.
B-Lymphocytes ; Chungcheongnam-do ; Cohort Studies ; Consensus ; Disease-Free Survival ; Drug Therapy ; Humans ; Korea ; Lymphoma* ; Lymphoma, B-Cell ; Lymphoma, Non-Hodgkin ; Prognosis ; Radiotherapy ; Rectum ; Retrospective Studies ; Rituximab ; Seoul