Improvement of Severe Colon Stricture after Rituximab Therapy for Concomitant Mucosa-associated Lymphoid Tissue Lymphoma in a Patient with Ulcerative Colitis.

Colorectal strictures are uncommon in patients with ulcerative colitis (UC). An extranodal marginal zone B-cell lymphoma of mucosa- associated lymphoid tissue (MALT) lymphoma is rarely involved in the colon but may be associated with inflammatory bowel diseases. A 41-year-old female with a six-year history of UC presented with a severe stricture of the sigmoid colon that prevented the passage of a colonoscope. A histological examination revealed non-specific inflammation and fibrosis without dysplasia or cancer. Despite conventional treatment, including mesalazine and azathioprine for one year after that visit, the stricture persisted. In addition, diffuse, edematous exudative inflammation and multiple shallow ulcers were observed in the distal rectum, revealing a MALT lymphoma testing positive for CD20, CD43, CD5, and Bcl-2, but negative for CD3, CD10, CD23, and cyclin-D1. Four weekly doses of rituximab were administered. Follow-up colonoscopy performed one month after treatment revealed slight improvement in the rectal lesion without remnant histological evidence of a MALT lymphoma. In addition, the stricture showed marked improvement, and the colonoscope could pass easily through the stricture site. This is the first case report on an improvement of a severe sigmoid colon stricture in a patient with UC after rituximab treatment for a concomitant rectal MALT lymphoma.

Diagnosis and management of rare paediatric Non-Hodgkin lymphoma.

Mature B-cell lymphomas, (B- or T-cell) lymphoblastic lymphomas (LBL), and anaplastic large cell lymphoma (ALCL) correspond to about 90% of all non-Hodgkin lymphoma (NHL) cases occurring in children and adolescents. The remaining 10% encompass a complex group of entities characterized by low/very low incidences, paucity of knowledge in terms of underlying biology in comparison to their adult counterparts, and consequent lack of standardization of care, information on clinical therapeutic efficacy and long-term survival. At the Seventh International Symposium on Childhood, Adolescent and Young Adult NHL, organized on October 20-23, 2022, in New York City, New York, US, we had the opportunity to discuss clinical, pathogenetic, diagnostic, and treatment aspects of certain subtypes of rare B- or T-cell NHL and they will be the topic of this review.

Surgical treatment and prognosis of 51 cases of splenic marginal lymphoma / 中华普通外科杂志

Objective:To explore the clinical characteristics, treatment and prognosis of splenic marginal zone lymphoma.Methods:The clinical data of 51 patients with splenic marginal zone lymphoma who underwent surgical treatment at Department of Hepatobiliary in Peking University People's Hosipital from Dec 2009 to Oct 2022 were retrospectively analyzed.Results:The patients 's average age was 60.5±11.8 years old, and the ratio of male to female rate was about 1:1. As of the last follow-up date of Feb 28, 2023, 8 patients died of the primary lymphoma and 14 patients suffered from disease progression. The 5-year progression free survival rate was 68%, and the overall survival rate was 79%. Univariate analysis showed that age ≥65 years old, decreased preoperative albumin, increased bilirubin level, and increased lactate dehydrogenase bode ill for overall survival time, but none of them were independent risk factors; Age ≥65 years old and diffuse large B cell transformation were independent risk factors.Conclusions:Surgery combined with comprehensive treatment which contained rituximab is currently the main treatment method. Splenectomy remains an important treatment and diagnostic method for patients with obvious symptoms or persistent unrelieved blood count abnormalities and unexplained splenomegaly. Advanced age, elevated bilirubin and lactate dehydrogenase, decreased albumin, and diffuse large B cell transformation, it may indicate poor prognosis.

[Treatment and prognosis of primary ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma: a report from a single center].

Objective: 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) . Methods: 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. Results: 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. Conclusions: 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.

Tratamento cirúrgico exclusivo de linfoma B primário cutâneo da zona marginal: relato de caso / Exclusive surgical treatment of a primary cutaneous marginal zone lymphoma: a case report

Os linfomas B primários cutâneos (LBPCs) são linfomas não Hodgkin, de acometimento exclusivamente cutâneo, e representam 25% dos linfomas primários cutâneos. São divididos, conforme comportamento clínico, em indolentes e intermediários. O tratamento das formas indolentes inclui a cirurgia, a radioterapia e, em casos extensos, o rituximabe. Relata-se o caso de mulher de 57 anos, com placa única no braço esquerdo, com diagnóstico de LBPC da zona marginal, tratado com excisão com margens de segurança de 5mm, sem recidiva após 36 meses de seguimento. A cirurgia é uma alternativa terapêutica com bom resultado clínico, sem impacto na sobrevida livre da doença.

Primary cutaneous B-cell lymphomas are non-Hodgkin lymphomas presenting only in the skin and represent 25% of all primary cutaneous lymphomas. Based on their clinical behavior, they are classified into indolent and intermediate forms. Treatment of indolent forms includes surgery, radiotherapy, and, in extensive disease, rituximab. We report a case of a 57-year-old woman with a single nodule in the left arm treated with surgical excision with 5-mm security margins, without relapse after 36 months. Surgery is a therapeutic option in these lymphomas without compromising disease-free survival.

Nodal marginal zone lymphoma with elevated monoclonal IgM: report of 1 case and review of literature / 白血病·淋巴瘤

Objective:To investigate the clinicopathological features, diagnosis, differential diagnosis and treatment of nodal marginal zone lymphoma (NMZL) with elevated monoclonal IgM.Methods:The clinical data of one NMZL patient with elevated monoclonal IgM treated at Yancheng No.1 People's Hospital in July 2020 were retrospectively analyzed, and the related literature was analyzed.Results:The patient was a 57-year-old female and the main clinical manifestations were fatigue and bone pain in left rib. Serum immunofixation electrophoresis showed IgM-κ type M proteinemia, bone marrow cytology showed a few plasmacytoid lymphocytes, bone marrow biopsy and immunohistochemistry showed B-cell non-Hodgkin lymphoma, bone marrow genetic testing showed MYD88 L265p and CXCR4 were both negative, postoperative pathology result of retroperitoneal lymph node biopsy was marginal zone lymphoma (mature small B type, prone to NMZL),and immunohistochemistry results: CD3, CD5, CD138, κ, λ, CD10, Cyclin D1 were negative, CD20, Pax-5, CD23 (FDC), bcl-2 were positive; Ki-67 positive index < 5%. The final diagnosis was NMZL with elevated monoclonal IgM. Partial remission was achieved after 8 cycles of reduced-dose CHOP regimen; thalidomide was used in the maintenance treatment, the disease condition was stable until August in 2021 and the follow-up was continuing.Conclusions:NMZL with elevated monoclonal IgM is relatively rare. Its diagnosis should be differentiated from Waldenstr?m macroglobulinemia and other inert B-cell lymphomas. Currently, there is no standard treatment and following the principle of individualized treatment can improve the prognosis of patients.

Value of 18F-FDG PET/CT in the interim therapeutic and prognostic evaluation of primary gastrointestinal lymphoma / 中华核医学与分子影像杂志

Objective:To assess the value of 18F-FDG PET/CT imaging and relevant factors in the interim therapeutic and prognostic evaluation of primary gastrointestinal lymphoma (PGIL) patients. Methods:From January 2008 to January 2018, 41 patients with B-cell PGIL (24 males, 17 females; age: 26-84 years) confirmed by pathology in Shanghai General Hospital, Shanghai Jiao Tong University School of Medicine were retrospectively included. 18F-FDG PET/CT was performed before chemotherapy and radiotherapy and after 3-4 courses of chemotherapy. There were 17 cases of mucosa-associated lymphoid tissue (MALT) lymphoma and 24 cases of diffuse large B-cell lymphoma (DLBCL). Mann-Whitney U test was used to compare the differences of metabolic parameters (SUV max, metabolic tumor volume (MTV), total lesion glycolysis (TLG)) before treatment between MALT lymphoma and DLBCL patients. ROC curve analysis was used to analyze the predictive abilities of different parameters for progression-free survival (PFS), and Cox regression analysis was used to analyze the influencing factors for PFS. Results:The median follow-up time of 41 patients was 25 (6-84) months, with the 3-year PFS rate of 55.9% and the overall survival (OS) rate of 80.2%. The baseline SUV max (23.2±11.9), MTV (260.7(66.2, 740.7) cm 3) and TLG (1 902.9(592.2, 8 418.1) g) in DLBCL were significantly higher than those in MALT lymphoma (7.9(6.2, 9.8), 45.9(28.4, 104.2) cm 3, 121.1(72.8, 295.6) g; z values: -4.02, -3.10, -3.92, all P<0.05). ΔSUV max in DLBCL patients (AUC=0.80, P=0.012), ΔSUV max% (AUC=0.89, P=0.007; AUC=0.80, P=0.012), ΔMTV%(AUC=0.91, P=0.005; AUC=0.77, P=0.026) and ΔTLG% (AUC=0.87, P=0.011; AUC=0.77, P=0.026) in MALT lymphoma and DLBCL patients before and after treatment were predictive factors of PFS. Multivariate analysis showed that ΔSUV max% was an independent factor for PFS of MALT lymphoma (hazard ratio ( HR)=17.192, 95% CI: 2.035-145.245, P=0.009), while ΔMTV% and ΔTLG% were factors for PFS of DLBCL (both HR=7.556, 95% CI: 1.968-29.016, P=0.003). Conclusions:There are significant differences in metabolic parameters between MALT lymphoma and DLBCL before treatment. Interim PET/CT is effective for the prediction of prognosis of MALT lymphoma and DLBCL.

Treatment and prognosis of primary ocular adnexal extranodal marginal zone mucosa-associated lymphoid tissue lymphoma: a report from a single center / 中华血液学杂志

Objective: 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) . Methods: 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. Results: 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. Conclusions: 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.

CT and MRI Features of Localized Peritumoral Amyloidosis in a Patient with Head and Neck Mucosa-Associated Lymphoid Tissue Lymphoma: A Case Report.

Amyloidosis has been reported to be associated with non-Hodgkin lymphoma. Amyloidosis and lymphoma can be related in two ways: lymphoma-associated systemic amyloidosis and peritumoral amyloidosis with lymphoma. We report a rare case of peritumoral amyloidosis in a patient with head and neck mucosa-associated lymphoid tissue lymphoma. On CT, the oropharyngeal mass showed an irregularly shaped soft-tissue density with multifocal amorphous calcifications and heterogeneous enhancement. On MRI, the mass showed heterogeneous low signal intensity on both T1- and T2-weighted images. On contrast-enhanced MR images, the mass showed good enhancement with several inner non-enhancing foci. Concurrent pathologies, such as peritumoral amyloidosis, should be considered when calcifications are noted in patients with pre-treatment lymphoma.

Comparison of 18F-FDG PET/CT and digestive endoscopy findings in gastric mucosa-associated lymphoid tissue lymphoma / 中华核医学与分子影像杂志

Objective:To investigate 18F-fluorodeoxyglucose (FDG) PET/CT imaging manifestations and digestive endoscopy of gastric mucosa-associated lymphoid tissue (MALT) lymphoma and evaluate whether maximum standardized uptake value (SUV max) can reflect the tumor proliferation activity and diagnose the diffuse large B cell transformation. Methods:18F-FDG PET/CT of 36 untreated histologically confirmed gastric MALT lymphoma patients (19 males, 17 females, age (46.4±18.1) years) between December 2012 and January 2019 in Nanjing Drum Tower Hospital were reviewed retrospectively. A positive or negative PET was defined based on visual analysis. 18F-FDG uptake above surrounding tissues in the regions of interest defined by the nuclear physician was considered positive, while negative was definited if the 18F-FDG uptake below surrounding tissues. Types of uptake included focal uptake and diffuse uptake. The characteristic findings of 18F-FDG PET/CT and digestive endoscopy (3 types: chronic gastritis-like type, depressed type and protruding type) in the consecutive patients were evaluated. The region of interest was drawn and the maximum standardized uptake value (SUV max) was measured. One-way analysis of variance and the least siginficant difference t test were used to compare the SUV max of 3 types of lesions and Mann-Whitney U test was used for comparison of SUV max between lesions with/without diffuse large B cell transformation. The correlation between SUV max and Ki-67 was assessed by Spearman rank correlation analysis. Receiver operating characteristic (ROC) curve analysis was performed to calculate the optimal cut-off value for the diagnosis of diffuse large B cell transformation. Results:Positive 18F-FDG PET/CT were found in 15 patients and the diagnostic accuracy was 41.7%(15/36). 18F-FDG uptake results were positive for all protruding tumors (5/5) mainly with focal uptake (4/5), but only 4/16 for chronic gastritis-like type tumors and 6/15 for depressed type tumors. SUV max of protruding type tumors (10.7±6.4) was significantly higher than chronic gastritis-like type tumors (2.1±0.8) and depressed type tumors (2.7±1.4; F=13.010, all P<0.05). SUV max (2.7(1.8, 5.0)) was associated with Ki-67 (10%(15%, 40%); rs=0.345, P=0.039). SUV max of tumors with diffuse large B cell transformation in 36 patients was significantly higher than that with no transformation (9.4(3.1, 14.8) vs 2.3(1.7, 3.9); z=-3.044, P=0.002), and the cut-off value of SUV max was 6.5 (area under the curve: 0.788, P=0.011). Conclusions:18F-FDG PET may be a useful method for evaluating protruding type gastric MALT lymphoma but not appropriate for chronic gastritis-like type or depressed type tumors. SUV max may be a useful biomarker for tumor proliferation activity and can be used for diffuse large B cell transformation diagnosis in gastric MALT lymphoma patients.

Diagnosis of splenic marginal zone lymphoma / 白血病·淋巴瘤

Objective:To explore the clinical features, diagnosis and differential diagnosis of splenic marginal zone lymphoma.Methods:The clinical diagnosis and differential diagnosis processes of 3 cases of CD5 - CD10 - B cell non-Hodgkin lymphoma with splenomegaly and cytopenia who were admitted to Tongji Hospital of Tongji Medical College of Huazhong University of Science and Technology in 2019 were retrospectively analyzed, and the relevant literature was reviewed. Results:The 3 cases were all elderly patients with varying degrees of splenomegaly and cytopenia. CD5 - CD10 - monoclonal B lymphocytes were found in the bone marrow or lymph nodes. Based on the patient's clinical characteristics, peripheral blood and bone marrow morphology, immunophenotype and genetic characteristics, 2 patients were diagnosed as splenic marginal zone lymphoma, and 1 patient was diagnosed as diffuse large B-cell lymphoma. Conclusions:The diagnosis of splenic marginal zone lymphoma requires comprehensive analysis of clinical characteristics, peripheral blood and bone marrow morphology, immunophenotype and genetic characteristics. Careful differentiation from other CD5 - CD10 - small B-cell lymphomas is also needed. The next-generation gene mutation high-throughput sequencing and mutational spectrum analysis will help the accurate diagnosis of atypical and difficult cases.

[The preliminary observation of intralesional rituximab administration in primary ocular adnexal mucosa-associated lymphoid tissue lymphoma].

Objective: To observe the efficacy of intralesional rituximab administration in primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (POAML). Methods: Retrospective case series study. The clinical and follow-up data of eight patients with POAML in the treatment group from September 2017 to December 2018 at the First Affiliated Hospital of Zhengzhou University were collected and analyzed retrospectively. The patients (3 males and 5 females) were 35 to 83 years old, with a median of 58 years. All patients received surgical treatment (most of the tumor were removed). The diagnosis of mucosa-associated lymphoid tissue lymphoma was confirmed by histopathology and immunohistochemical staining showed CD20+. Examinations such as chest CT, abdominal CT, bone marrow biopsy or positron emission tomography-CT to exclude lymphoma with systemic lesions. Patients with conjunctival POAML were injected through the subconjunctival infiltration. For the lacrimal gland POAML and the orbital POAML, the injection was performed through the periocular space by using a post-bulb syringe needle at multi-points with an interval of 1 cm, before the pressure dressing. All patients were injected 1.5 to 5.0 ml (15 to 50 mg) once every three weeks, 4 to 6 times as a treatment cycle. The injection range included or exceeded the lesion area. Then consider whether to supplement the number of treatments based on the remaining tumor. The patients' treatment doses, drug onset time, tumor regression, local reactions (local pain and swelling), systemic reaction indicators (fever, gastrointestinal reactions, complete blood count and the lymphocyte subsets), and systemic recurrence indicators (serum ß2 microglobulin and lactate dehydrogenase detecting) were recorded. Results: Two patients, including one patient with bilateral lesions, had conjunctival POAML, one patient had lacrimal gland POAML, and five patients had orbital POAML. They received 2 to 7 injections, and the total dose was 45 mg to 280 mg. There were tolerable local pain upon injections in all patients, local redness and swelling in one patient, fever in two patients, mild gastrointestinal reactions in one patient, fatigue in one patient, and palpitation and discomfort in one patient. After close observation or corresponding symptomatic treatment, the symptoms subsided. No special treatment was given. The follow-up time was 9 to 30 months, and the median was 20 months. The lymphomas in all patients were controlled without recurrence. The MRI showed that local lesions disappeared completely in five patients, and the conjunctiva and the extraocular muscle became thickened in three patients. There was no abnormity in complete blood count, serum ß2 microglobulin and lactate dehydrogenase. Conclusion: The intralesional rituximab administration is a simple, effective therapy for localized POAML with no systemic invasion. (Chin J Ophthalmol, 2020, 56: 839-845).

[Analysis of clinical prognosis of 68 patients with gastric mucosa-associated lymphoid tissue lymphoma].

Objective: To explore the clinical features and prognosis of gastric mucosa-associated lymphoid tissue lymphoma. Methods: The data of patients with gastric mucosa-associated lymphoid tissue lymphoma diagnosed and treated at Peking University Cancer Hospital from 2000 to 2019 were collected, and their clinical characteristics, treatment options and effects, and prognosis were retrospectively analyzed. Results: The median age of 68 patients was 56 years old, and the ratio of men to women was 0.6∶1.0. Early patients accounted for about 76% (52/68). The most commonly used treatment is Helicobacter pylori eradication, followed by radiotherapy and chemotherapy. The total effective rate is 94%, of which the complete remission rate is 72%. With a median follow-up of 44 months, the 5-year progression-free survival (PFS) rate was 73%, and the overall survival (OS) rate was 83%. Prognostic factor analysis showed that albumin level (P=0.011) and international prognostic index (IPI)(P=0.017) are independent prognostic indicators of PFS, The 5-year PFS rate of patients with normal and reduced albumin was 77% and 50%, respectively, and the 5-year PFS rate of patients with IPI score<2 points and IPI ≥ 2 points was 80% and 48%, respectively. Lactate dehydrogenase (LDH) (P=0.016) and albumin level (P=0.025) are independent prognostic indicators of OS, and the 5-year OS rates of patients with normal and elevated LDH were 87% and 67%, respectively. The 5-year OS rates of patients with normal albumin and reduced were 92% and 73%, respectively. Conclusions: Gastric mucosa-associated lymphoid tissue lymphoma is sensitive to treatment and has a good prognosis. The patient's albumin and lactate dehydrogenase levels are independent prognostic indicators.

Primary hepatic extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue.

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), is one of the specific type of low-grade B-cell lymphoma not infrequently found worldwide. It typically involves mucosal sites such as stomach and conjunctiva; however, primary hepatic MALT lymphoma has been extremely rarely reported. We describe a case of hepatic MALT lymphoma in a 70-year-old male patient who underwent left hepatectomy due to the incidentally detected liver masses at a medical checkup. The resected specimen revealed multinodular masses consisting of small-to-intermediate-sized lymphoid cells with serpentine pattern and focal lymphoepithelial lesions. The tumor cells were diffusely positive for CD20 and Bcl-2 but negative for CD3, CD10, CD5, CD23, CD43, and cyclinD1. The Ki-67 labeling index was 10% and immunoglobulin heavy chain gene rearrangement study confirmed monoclonal proliferation. In this paper, we discuss several unique clinicopathologic characteristics which will be helpful to the differential diagnosis of hepatic MALT lymphoma.

[Diagnosis and Treatment of Gastric Mucosa-associated Lymphoid Tissue Lymphoma].

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.

[Paediatric nodal marginal zone lymphoma: a clinicopathological study of seven cases].

Objective: To investigate the pathological features and clinical manifestation of pediatric nodal marginal zone lymphoma(NMZL). Methods: Histological morphology and immunophenotype of 7 cases of pediatric NMZL were retrospectively reviewed at Beijing Friendship Hospital Affiliated to Medical University between January 2008 to October 2018. Clonal rearrangement analysis was performed. Clinical information including patient follow-up data were analyzed. Results: All 7 patients were male with a median age of 15 years aged from 10 to 26 years. All patients presented with only lymph node enlargement without B symptoms, including cervical lymph node (5 cases), preauricular lymph node (1 case) and retroauricular lymph node (1 case). Histologically, all cases showed irregular large follicles on the edges with widened marginal areas and intervesicular areas, and lesional cells were uniform with progressive transformation of germinal center centers along with a small amount of intrinsic lymphoid tissue. All 7 cases showed diffuse CD20 positivity both follicle and interfollicular region along with 30%-40% positivity in the interfollicular region (pathological region). Markers of other B-cell lymphomas werenot expressed. All 7 cases were positive for immunoglobulin(Ig) gene rearrangement. None of the patients showed no recurrence up on after follow-up for an average of 13 months. Conclusions: Pediatric NMZL is a rare type of lymphoma that has a unique morphology and occurs almost exclusively in male children and young adults and often in head and neck lymph nodes. It has an excellent prognosis. Therefore, awareness of the disease with accurate diagnosis is important.

[Clinical Efficacy of Radiotherapy in Helicobacter pylori Negative or Unresponsive to Eradication Therapy Primary Gastric Mucosa-Associated Lymphoid Tissue Lymphoma].

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.

Discordant lymphoma: report of one case and review of literature / 白血病·淋巴瘤

Objective@#To investigate the clinical characteristics, diagnosis, treatment and prognosis of discordant lymphoma.@*Methods@#The clinical data of one patient with discordant lymphoma at the PLA Strategic Support Force Characteristic Medical Center were retrospectively analyzed, and the related literatures were reviewed.@*Results@#The patient was treated for thrombocytopenia and the examination showed splenomegaly. After hormone treatment, the platelet rebounded and thrombocytopenia occurred during hormone reduction. Splenectomy was performed. Postoperative pathological diagnosis of splenic marginal lymphoma was made and observed. Axillary lymph node enlargement occurred nine months later. Pathological diagnosis of diffuse large B-cell lymphoma was made by using lymph node biopsy, and the disease condition was alleviated after immunotherapy combined with chemotherapy.@*Conclusions@#Discordant lymphoma is rare and shows no special clinical manifestations. Its diagnosis should rely on pathological examination. Immunotherapy combined with chemotherapy may be more effective.

Paediatric nodal marginal zone lymphoma: a clinicopathological study of seven cases / 中华病理学杂志

Objective@#To investigate the pathological features and clinical manifestation of pediatric nodal marginal zone lymphoma(NMZL).@*Methods@#Histological morphology and immunophenotype of 7 cases of pediatric NMZL were retrospectively reviewed at Beijing Friendship Hospital Affiliated to Medical University between January 2008 to October 2018. Clonal rearrangement analysis was performed. Clinical information including patient follow-up data were analyzed.@*Results@#All 7 patients were male with a median age of 15 years aged from 10 to 26 years. All patients presented with only lymph node enlargement without B symptoms, including cervical lymph node (5 cases), preauricular lymph node (1 case) and retroauricular lymph node (1 case). Histologically, all cases showed irregular large follicles on the edges with widened marginal areas and intervesicular areas, and lesional cells were uniform with progressive transformation of germinal center centers along with a small amount of intrinsic lymphoid tissue. All 7 cases showed diffuse CD20 positivity both follicle and interfollicular region along with 30%-40% positivity in the interfollicular region (pathological region). Markers of other B-cell lymphomas werenot expressed. All 7 cases were positive for immunoglobulin(Ig) gene rearrangement. None of the patients showed no recurrence up on after follow-up for an average of 13 months.@*Conclusions@#Pediatric NMZL is a rare type of lymphoma that has a unique morphology and occurs almost exclusively in male children and young adults and often in head and neck lymph nodes. It has an excellent prognosis. Therefore, awareness of the disease with accurate diagnosis is important.

Clinical Efficacy of Radiotherapy in Helicobacter pylori Negative or Unresponsive to Eradication Therapy Primary Gastric Mucosa-Associated Lymphoid Tissue Lymphoma / 대한소화기학회지

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.