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Background: Lymphoma and dysplasia are rare complications of long-standing Crohn's disease. We report an exceptional case of a synchronous intestinal marginal zone B-cell lymphoma (MALT lymphoma) and colonic adenoma in a Crohn's disease patient., Case Presentation: A 50-year-old male patient presented with right lower quadrant for the last 9 months. He also had associated weight loss and diarrhea alternating with constipation. Ileo-colonoscopy revealed a pseudopolypoid appearance of the colonic and ileal mucosa with many discontinuous ulcerations with a 3 cm sessile polypoid mass at 17 cm from the anal verge. Histological examination of the polypoid lesion revealed an adenoma with high grade dysplasia, while the biopsies of colonic mucosa showed histologic features of Crohn's disease. Abdominal computed tomography scan (CT scan) and magnetic resonance imaging (MRI) showed circumferential wall thickening of the colon and ileum, enlarged mesenteric lymph nodes and a sessile polypoid mass of the rectosigmoid junction. The patient was scheduled for an ileocoletectomy with resection of the upper rectum and ileorectostomy. The histological examination of the resected segment showed histologic features of Crohn's disease, a recto-sigmoid polyp with high grade. dysplasia and extensive small lymphocytic infiltrate in both colonic and ileal wall which is strongly stained by CD20 and BCL2. The diagnosis of MALT lymphoma with adenoma on a background of Crohn's disease was made. The patient successfully completed 8 cycles of Rituximab+ chlorambucil chemotherapy. Nowadays the patient is asymptomatic without evidence of lymphoproliferative recurrence 10 months after surgery., Conclusion: We report the first case in the literature of Malt lymphoma with colonic adenoma associated with Crohn's disease, and discuss his unique macroscopic and histological features in a patient. Without immunosuppressive therapy.

AL-amyloidoma is considered to be a variant of primary cutaneous marginal zone lymphoma (pcMZL). A 51-year-old white man presented a 2 × 2-cm erythematous to brownish waxy plaque on the back of the scalp. The plaque was first noticed 16 years ago. It was asymptomatic, and the patient was otherwise healthy. The lesion was excised. Histological examination revealed dermal deposits of amyloid and a subtle perivascular infiltrate, predominately consisting of plasma cells. Infiltrating cells expressed CD79a, bcl2, and IgG and were negative for bcl6, CD56, and IgM. A monoclonal light-chain expression of lambda (lambda:kappa = 10:1) was demonstrated by in situ hybridization. The diagnosis of pcMZL, presenting as an AL-amyloidoma, was made because staging procedures excluded systemic manifestation of lymphoma, monoclonal gammopathy, and systemic amyloidosis. Cutaneous amyloid deposits with monoclonal plasma cell proliferation can occur as a result of monoclonal gammopathy/plasmocytoma or as a rare manifestation of pcMZL. Systemic B-cell lymphoma and systemic monoclonal plasma cell proliferations have to be excluded.

Langerhans cell histiocytosis (LCH) is a clonal neoplastic proliferation of Langerhans-type cells. Orbital LCH is infrequent, typically manifesting as an isolated lytic bony lesion with an adjacent soft tissue mass in a child. Isolated lacrimal gland involvement by LCH is extremely rare, with only 2 previously reported cases. The authors describe a 37-year-old woman with a 6-month history of painless right upper eyelid swelling and diffuse right lacrimal gland enlargement without bony changes on computed tomography scan. Excisional biopsy of the lacrimal gland demonstrated concurrent LCH, extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue, and increased IgG4-expressing plasma cells. Work-up was negative for systemic hematolymphoid malignancy and IgG4-related disease. This case illustrates the association between LCH, mucosa-associated lymphoid tissue lymphoma, and elevated IgG4 plasma cells in the lacrimal gland, and we review the emerging theories proposed to explain this phenomenon.

Mucosal-associated lymphoid tissue (MALT) lymphoma is rare in the pediatric population, but primary Sjogren syndrome is a well-established risk factor for this malignancy. This report describes 2 cases of MALT lymphoma in children with Sjogren syndrome. A 15-year-old girl developed MALT lymphoma of the parotid gland as the presenting symptom of Sjogren syndrome. In the second case, a 15-year-old boy with known Sjogren syndrome presenting mainly with arthritis was diagnosed with MALT lymphoma, also of the parotid gland. With early diagnosis and treatment, outcomes in pediatric MALT lymphoma are generally encouraging. Pediatric oncology specialists should be aware of the association of MALT lymphoma with Sjogren syndrome and have a high index of suspicion for this malignant complication.

The kidney is among the various anatomical sites involved in mucosa-associated lymphoid tissue (MALT) lymphoma. A variety of renal pathological types, including membranous glomerulopathy, membranoproliferative glomerulonephritis, crescentic IgA nephropathy, minimal change disease, and cryoglobulinemic glomerulopathy, have been reported in MALT lymphoma patients. However, cast nephropathy is extremely rare in MALT lymphoma. Herein, we describe the case of a patient with a history of MALT lymphoma of the stomach and small intestine 7 years before presenting with acute kidney failure 1 year after chemotherapy cessation. Monoclonal IgM-λ was detected in the serum, and kidney biopsy showed λ light chain deposition-based cast nephropathy. In addition, MALT recurrence was discovered in the stomach rather than intestinal tissue by gastrointestinal endoscope, and no lymphoplasmacytic infiltration was found in bone marrow. After 1 year of chemotherapy, renal function was partially restored, and the level of serum λ chain, serum IgM, and 24-hour urine protein all decreased. Our case illustrates that MALT lymphoma is prone to recurrence and grows slowly, moreover, with the characteristic of monoclonal immunoglobulin production and kidney infiltration.

A 46-year-old male presented with anemia, vitamin B12 deficiency and a positive fecal occult blood test. Ileocolonoscopy was performed, which identified multiple large (up to 5 cm) edematous, polypoid lesions at the terminal ileum.

Pulmonary localization of B-cell lymphoma associated with deposits of amyloid material is a rare finding in the thoracic disease spectrum. This report describes a rare case of nodular pulmonary amyloidosis in a 50-year-old patient. He underwent left upper lobectomy for mucosa-associated lymphoid tissue lymphoma that originated from bronchial lymphoid tissue., (Copyright © 2019 The Society of Thoracic Surgeons. Published by Elsevier Inc. All rights reserved.)

Large cysts in the orbital lobe of the lacrimal gland are rare and are associated with Sjögren syndrome and B-cell mucosa-associated lymphoid tissue lymphoma. The authors describe 4 new cases of large orbital lobe lacrimal gland cysts. The first 2 patients, both with Sjögren syndrome, had unilateral cysts associated with chronic inflammation. Mucosa-associated lymphoid tissue lymphoma was also identified in the cyst wall of the second case and could not be completely excluded in the first case. The third patient, with a history of rheumatoid arthritis, had bilateral cysts, again associated with mucosa-associated lymphoid tissue lymphoma. The fourth patient, with no history of systemic disease, had a unilateral cyst associated with reactive lymphoid hyperplasia. Finally, the authors report the long-term outcomes of 3 previously reported cases.

Background: In Helicobacter pylori (H. pylori) positive stage I gastric low-grade MALT lymphoma, eradication is the accepted first-line therapy. The role of eradication therapy in lymphoma > stage IE is still unclear. However, about 20% of patients show persistent lymphoma following successful eradication or primary H. pylori-negative lymphoma. A prospective study for salvage radiation therapy with standard 36 Gy in comparison to a reduced dose of 25.2 Gy is still missing., Methods: A prospective, multicentre study investigated the efficacy of eradication in H. pylori-positive gastric low-grade MALT lymphoma stages IE and II1E (HELYX I). Refractory lymphoma or H. pylori-negative patients were treated in a prospective, randomised, multicentre, phase II study to receive either 25.2 Gy or 36 Gy radiotherapy (HELYX II)., Results: 102 patients (3 drop outs) were included in HELYX I: 75/99 (75.8%) showed complete remission after a median of 2.8 months. 18 (18.2%) had partial remission (PR) and 6 (6.0%) no change (NC). 29 patients (7 drop outs) were randomized in HELYX II (7 primarily H. pylori-negative, 15 patients from HELYX I with refractory disease after eradication). All patients achieved stable CR irrespective of radiation dose. Both presence of the t(11,18) translocation (OR 9.0, p = 0.01) and monoclonality of the tumour cells (OR 6.3, p = 0.006) were predictors for persistant lymphoma after eradication therapy., Conclusions: Most H. pylori-positive low grade gastric MALT lymphoma stage IE and II1E respond with stable CR after eradication therapy. In patients with refractory disease or H. pylori negative low grade gastric MALT lymphoma a dosage-reduced radiation therapy with 25.2 Gy is an effective standard dose in stage IE and II1E., Trial Registration: ClinicalTrials.gov: NCT00154440.

We report a case of splenic marginal zone B-cell lymphoma discovered in a 73-year-old man with cold agglutinin disease. PET/CT revealed splenomegaly with focally intense uptake of F-FDG and diffusely increased bone marrow uptake, which was considered to be secondary to hemolytic anemia. Splenectomy was performed. The histopathology of the spleen showed splenic marginal zone B-cell lymphoma with partial splenic infarction, which correlated with the area of focal intense FDG uptake. Depending on the time since onset, splenic infarctions can present as focal FDG accumulation.

Marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphomas of the breast with mammary amyloidosis are exceedingly rare entities. This report describes the case of women with long-standing Sjögren's syndrome presenting with breast MALT lymphoma and amyloïd light-chain (AL) amyloidosis. Breast microcalcification needle biopsy made the positive diagnosis. This unusual finding should be kept in mind. It emphasises the need for careful clinical examination of nodes and extranodal organs supposedly affected in patients with autoimmune disease., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Progressive multifocal leukoencephalopathy (PML) is caused by John Cunningham (JC) virus in immunocompromized patients such as those with human immunodeficiency virus (HIV) infection, hematological malignancy, autoimmune disorder, and immunodeficiency disorder as well as those undergoing chemotherapy or immunosuppressive therapy. No effective treatments have been established for PML, which commonly causes severe neurological sequelae. We describe the first case of PML in a patient without HIV infection who exhibited remarkable improvement following acute pyelonephritis with Escherichia coli bacteremia., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Background: Primary lymphomas of the gastrointestinal tract are rare, accounting for only 1 to 4% of malignancies arising in the stomach, small intestine, or colon. The stomach is the most common extranodal site of lymphoma and gastric mucosa-associated lymphoid tissue (MALT) lymphoma accounts for 40% of primary gastric lymphoma. Gastric MALT lymphoma reaches its peak incidence between 50 to 60 years of age, therefore, it is rarely encountered in pediatric population. The presenting symptoms of gastric MALT lymphoma are usually nonspecific and primary perforation of gastric MALT lymphoma is uncommon., Case Presentation: A 12 year-old female presented with iron deficient anemia developed gastric perforation. Emergency laparoscopic repair of the perforation was performed and tissue pathology showed gastric MALT lymphoma infiltration. Helicobacter pylori eradication and radiotherapy were sequentially performed. Complete remission was achieved at two months after radiotherapy. To our best knowledge, she is the youngest patient with gastric MALT lymphoma reported in the literature., Conclusion: Iron deficient anemia is a common presenting manifestation of malignancies in adulthood. In pediatric population, iron deficient anemia is usually caused by nutritional deficient or blood loss. In this case report, we present a teenaged female without previous gastric ulcer history who presented with a rare gastric tumor and an uncommon primary perforation. Even if there is an uncertainty about the exact diagnosis prior to the surgery, the strategy of stomach-preserving therapy by laparoscopy for primary perforation was successful and provided a good quality of life.

Purpose: To examine the clinicopathological characteristics of patients with thyroid eye disease (TED) who showed asymmetric lacrimal gland enlargement., Methods: Sixteen patients were included in this retrospective, observational case series. The following data were collected: sex, age, laterality, past medical history, laboratory results for thyroid function and other autoimmune disorders, pathological results, other systemic lesions related to lacrimal gland pathology, and the clinical course after treatment., Results: The results of pathological examinations were consistent with non-specific dacryoadenitis in seven patients, immunoglobulin G4-related dacryoadenitis in four patients, sarcoidosis in two patients, and xanthogranuloma in one patient, respectively. The remaining two patients were proven to be extra-nodal marginal zone lymphoma of mucosa-associated lymphoid tissue type (MALT lymphoma). One patient with sarcoidosis was noted to have another related systemic lesion. After treatment or observation, the lesions were either improved or stable in almost all patients, except for the two patients with MALT lymphoma., Conclusions: Patients with TED who present with asymmetric lacrimal gland enlargement need to be further evaluated. Biopsy of the lacrimal gland and full systemic work-up should be considered as these may lead to detection of other pathological entities, especially malignancies, which could help in saving patients' lives.

Background: Primary dural-based lymphoma is a rare disease entity. It has been hypothesized that patients with sarcoidosis are at increased risk of developing lymphoma, an association known as "sarcoidosis-lymphoma syndrome," although dural disease has not yet been reported in the literature., Case Description: Here we report the first case of dural-based lymphoma in a 60-year-old man with a history of sarcoidosis, in keeping with sarcoidosis-lymphoma syndrome. His dural-based tumor was surgically resected. Pathology examination revealed a marginal zone B-cell lymphoma. Subsequent neural and systemic staging confirmed a localized lymphoma. The patient received local radiotherapy to control leptomeningeal disease., Conclusions: Given the association between sarcoidosis and lymphoma, we suggest lymphoma to be considered as one of the top differentials for a dural-based mass lesion in patients with sarcoidosis., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Purpose: We aimed to evaluate therapeutic results of radiotherapy for gastric mucosa-associated lymphoid tissue (MALT) lymphomas., Patients and Methods: We reviewed retrospectively the records of 11 patients presenting with gastric MALT lymphoma treated between 1993 and 2014. Patients with low-grade lymphoma in failure after helicobacter eradication had exclusive gastric external radiotherapy. Chemotherapy followed by radiotherapy were indicated in case of high grade lymphoma. Radiotherapy doses range between 30 and 40Gy (2Gy per fraction, five fractions per week)., Results: All tumours were IE stage. Seven patients with low-grade lymphoma had radiotherapy. Four patients with high-grade lymphoma received chemotherapy then radiotherapy. Ten patients are in complete remission after treatment achievement. Five and 10 years disease-free survival are 100%. No severe toxicity was seen., Conclusion: Eradication of Helicobacter pylori is the mainstay of treatment of gastric MALT. External irradiation is an effective and well-tolerated treatment modality in case of resistance to helicobacter eradication., (Copyright © 2018 Société française de radiothérapie oncologique (SFRO). Published by Elsevier Masson SAS. All rights reserved.)

A 62-year-old man presented with complaints of acute abdominal pain. Abdominal enhanced CT showed free air in the abdominal cavity, a thickened stomach wall accompanied by deep ulcer, and enlarged lymph nodes around the stomach. Emergent surgery was conducted, and a perforation in the upper part of the stomach was found. Total gastrectomy was performed. On pathological examination, a mucosa-associated lymphoid tissue lymphoma was diagnosed. Histologic examination revealed the absence of Helicobacter pylori in the gastric mucosa. After the operation, adjuvant chemotherapy was not administered. However, he has been well for 11 months without any recurrence. Gastric MALT lymphoma with spontaneous perforation is considered extremely rare. A brief review of the literature is presented.

Primary cutaneous marginal zone lymphomas (PCMZL) frequently exhibit lymphoplasmacytoid/plasmacytic differentiation, implying the capacity to produce monoclonal immunoglobulins. As these paraproteins are secreted, and thus are measurable in blood and urine, they may correlate with disease burden and serve as tumour markers reflecting therapeutic response. This study retrospectively analysed the records of 23 patients with PCMZL. During treatment and follow-up, laboratory tests, including full blood count, lactate dehydrogenase, serum protein electrophoresis and turbidimetric analyses, were conducted. Thirty-nine percent of cases showed a suspicious serum protein electrophoresis in terms of paraproteinaemia. In 44% of cases the heavy and light chain restriction in tissue samples correlated with serological findings. Altogether, 89% of the PCMZL patients with paraproteinaemia eventually experienced a relapse, in contrast to 62% of the group without paraproteinaemia. This study analysed the incidence and clinical implications of paraproteinaemia in patients with PCMZL. A clear correlation was found between paraproteinaemia, tumour relapse and therapeutic intervention.

Objective: Primary Sjögren syndrome (pSS), an autoimmune epithelitis, bears the risk of evolving to non-Hodgkin lymphoma and most frequently to the mucosa-associated lymphoid tissue (MALT) subtype. Based on the observation that pSS patients with MALT present a more atrophic and more intensely fissured tongue, we aimed to semiquantify severity of tongue atrophy and clinically assess lingual appearance in pSS patients with and without MALT, and investigate whether tongue atrophy and fissured appearance could serve as clinical indicators/signs of MALT., Methods: A blinded complete oral examination was performed in pSS patients with and without MALT. Tongue atrophy was scored using a semiquantified atrophy score. Clinical and laboratory variables were recorded for all patients., Results: After excluding pSS patients with oral candidiasis, iron deficiency, and megaloblastic anemia, 19 pSS patients with salivary MALT were matched 1:3 for age, sex, and disease duration with 57 pSS patients without MALT. The pSS-MALT patients had increased prevalence of salivary gland enlargement, lymphadenopathy, monoclonal gammopathy, rheumatoid factor positivity, higher focus and Tarpley scores in the minor salivary gland biopsy, and hyposalivation, compared to the pSS non-MALT patients. A significantly higher prevalence of tongue atrophy (68% vs 30%, p = 0.006) and fissured tongue (89% vs 33%, p < 0.001) was observed in the former group. Multivariate analysis showed that fissured tongue appearance, hyposalivation, and lymphadenopathy associate independently with salivary MALT in pSS., Conclusion: These results suggest that pSS patients with lymphoid malignancy exhibit a more atrophic and more fissured tongue. This particular clinical tongue appearance can serve as an additional clinical sign for salivary MALT lymphoma in pSS patients.

Background: Acquired C1-esterase inhibitor (C1-INH) deficiency angioedema (C1-INH-AAE) is a form of bradykinin-mediated angioedema. This rare disorder is due to acquired consumption of C1-INH, hyperactivation of the classic pathway of human complement, and potentially fatal recurrent angioedema symptoms. Clinical symptoms of C1-INH-AAE are very similar to those of hereditary angioedema (HAE) but usually appear after the fourth decade of life and induce abdominal pain less frequently. Laboratory tests are essential in establishing the diagnosis with low levels or abnormal structure and function of C1-INH. Most patients present C1-INH autoantibodies. Furthermore, C1q is reduced in AAE, contrary to HAE. The long-term prognosis is determined by associated hematologic malignancies., Patients and Methods: We report 4 cases of C1-INH-AAE associated with lymphoproliferative disorders referred to the Reference Centre for Angioedema of Besançon, France. The patients were aged between 60 and 77 years. C1 INH antibodies were found in three patients. Symptoms were triggered by angiotensin-converting enzyme (ACE) inhibitors or angiotensin II receptor blockers (ARBs) in 3 patients. Hematologic malignancy was present at diagnosis (one case of chronic lymphoid leukemia) or was diagnosed during follow-up (one case of indolent marginal zone non-Hodgkin lymphoma and two cases of monoclonal gammopathy)., Discussion: C1-INH-AAE induced by ACE inhibitors or ARBs may be associated with hematologic malignancies. This form of revelation does not necessarily indicate a diagnosis of ACE or ARBs angioedema, and screening should therefore be performed for C1 Inh and C1q. An underlying hematologic malignancy should be routinely sought and the long-term prognosis determined., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Rationale: Gastric mucosa-associated lymphoid tissue (MALT) lymphoma is the most common and best-studied extranodal marginal zone lymphoma of the MALT. It is characterized by an indolent clinical course and excellent survival compared with other malignant tumor. Complications such as obstruction, perforation or bleeding are rarely observed. The treatment strategy is still controversial., Patient Concerns: A 59-year-old man, who had been diagnosed with MALT lymphoma by gastroscopy and biopsy one month before, came to the hospital for a sudden onset of abdominal pain after breakfast., Diagnoses: MALT lymphoma; gastric perforation., Interventions: Emergency surgery., Outcomes: Gastric perforation repair and jejunostomy was performed. The patient recovered well and is preparing for combined chemotherapy., Lessons: This case report illustrates the challenges in diagnosis and treatment of MALT lymphoma. We discussed the particularity of its clinical characteristics, treatment strategies and prognosis combined with literature review, and we think that early diagnosis and timely appropriate chemotherapy is of great importance.

Over a 2-year period, swellings of all 4 eyelid margins developed in a 32-year-old woman and was accompanied by complete loss of eyelashes. An inflammatory dermatologic condition was considered the most likely cause. A full-thickness right lower eyelid biopsy revealed a multinodular lymphoid tumor at the eyelid margin which immunophenotypically and genetically was diagnosed as an extranodal marginal zone lymphoma. The mode of presentation of the disease was considered to be most unusual, as was its B cell lineage, since the majority of primary cutaneous lymphomas are of T-cell origin. Systemic workup demonstrated bilateral involvement of the external auditory canals., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by the infiltration of lymphocytes into exocrine glands, resulting in the typical sicca symptoms. Unlike adults, primary SS is a very rare condition in childhood, and the risk of malignancy in juvenile SS (JSS) has not been defined. We report the detection of extranodal marginal zone B-cell lymphoma (EMZL) occurring in two children with SS. Fine needle aspiration of the salivary glands (SG) showed nonspecific findings that led to delayed diagnosis of SS. The diagnosis of B-cell lymphoma associated with JSS was based on morphologic and immunohistochemical staining done during the biopsy. To highlight awareness of EMZL as a timely and appropriate update of an unusual complication in children with SS., (Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Reumatología y Colegio Mexicano de Reumatología. All rights reserved.)

Rationale: Mucosa-associated lymphoid tissue (MALT) lymphoma of the trachea is a rare disease that has been shown to be associated with chronic antigenic stimulation. There have been few reports of MALT lymphoma of the trachea in association with idiopathic pulmonary fibrosis (IPF)., Patient Concerns: A 73-year-old patient visited with a 2-year history of dyspnea on exertion and productive cough, which had worsened 1 month ago., Diagnoses: MALT lymphoma of the trachea associated with IPF., Interventions: After taking into consideration the age, poor performance status, and comorbidities of the patient and the extent of disease, we utilized an observational approach as a treatment strategy., Outcomes: The patient is well without any evidence of progression for 12 months since the initial diagnosis., Lessons: We present a case of MALT lymphoma of the trachea associated with IPF. A common predisposing factor may exist for tracheal MALT lymphoma and IPF. As there are no randomized clinical trials focusing on tracheal MALT lymphoma, individualized treatment decision is important, and in some cases, simply monitoring the patient might be the most appropriate approach.

The complex diversity of nonpathogenic microbes that colonize the human body, known as microbiota, exert considerable effects on physiological homeostasis, and immune regulation. Helicobacter pylori (H. pylori) is a bacterium that frequently colonizes human stomach and is a major pathogenic agent for peptic ulcer diseases, gastric cancer, and mucosa-associated lymphoid tissue (MALT) lymphoma. Due to its acidic pH and peristaltic movements, the stomach has been considered a hostile environment for most microorganisms, however various commensal microorganisms are capable of colonizing the stomach to form a stomach niche. Recent pieces of evidence indicate that commensal gastric microbes or their metabolites influence the capability of H. pylori to colonize the stomach and directly modulate its pathogenicity and carcinogenic potential. In this article, we present an overview of recent advances in the understanding of H. pylori-commensal interactions in the pathogenesis and clinical evolution of H. pylori-associated gastric malignancies., (Copyright © 2017 Elsevier B.V. All rights reserved.)

A 68-year-old man presented with a 4-day history of worsening knee and arm pain. On examination, there was erythema and swelling of the left knee and both wrists. Joint aspiration grew Neisseria meningitidis Blood tests showed an unusually high total protein level (100 g/L) and an IgM kappa paraprotein band of 45 g/L on protein electrophoresis. CT showed widespread lymphadenopathy, hepatosplenomegaly and multilevel thoracic vertebral collapse. A bone marrow biopsy revealed a lymphoplasmacytic infiltrate and a lymph node biopsy showed features of nodal marginal zone lymphoma with plasmacytic differentiation., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Salivary glands mucosa-associated lymphoid tissue lymphoma (SGML) is a distinct subtype of marginal zone B-cell type non-Hodgkin's lymphoma (NHL), which is commonly seen in middle aged females. SGML is usually associated with autoimmune diseases such as Sjögren's syndrome or with chronic infection such as hepatitis C virus (HCV) infection. Chromosomal abnormalities are frequently seen in SGML, which usually activate nuclear factor-κB molecular pathway to modulate cell survival and proliferation, resulting in lymphoma occurrence. SGML tends to arise from parotid gland, presenting frequently as a localized and indolent lesion, a long-term follow-up and biopsy are needed for accurate diagnosis. Surgery, radiotherapy and chemotherapy are usually effective disseminated diseases at multiple sites need combined treatment. SGML has a relatively better prognosis with a higher relapse rate than other types of NHLs, dissemination or higher degree of malignant transformation may occur. Thus, a long-term and close follow-up is essential for patients with SGML.

A 73-year-old male who underwent splenectomy was diagnosed with splenic non-caseating granuloma in May 201X, and sarcoidosis was disregarded from the differential diagnosis. Owing to the persisting inflammation, the patient was carefully followed up with no treatment. Four months post splenectomy, the patient was hospitalized due to progressive dyspnea. Chest computed tomography revealed an encapsulated pleural effusion and lymphocytic infiltration in the left lower lung, with subclavian and mediastinal lymphadenopathy. Although the patient was treated with antibiotics, his condition showed no improvement; therefore, prednisolone 40 mg was administered, resulting in lung lesion improvement. A re-examination of the tissue obtained from the previously removed spleen revealed splenic marginal zone lymphoma (SMZL), a specific low-grade, small B-cell lymphoma. As a result, the patient was treated with rituximab combined with chemotherapy. During the fifth course of the chemotherapy, a subcutaneous abscess appeared in the cervical region, and Mycobacterium shigaense was isolated from the pus discharge, suggesting that the splenic granulomatous lesion formed due to M.shigaense, and dissemination of the Mycobacterium infection occurred following splenectomy and chemotherapy, when the patient was immunosuppressed. Overall, we consider that SMZL developed because of chronic inflammation resulting from a nontuberculous mycobacterial infection.

Syndrome of inappropriate antidiuretic hormone secretion (SIADH) may develop in association with several malignancies. However, as an immunohistochemical analysis is not performed in the majority cases, its true cause is often uncertain. We herein report a case of SIADH following chemotherapy due to tumor-derived ADH production in a patient with mucosa-associated lymphoid tissue (MALT) lymphoma. A retrospective immunohistochemical analysis demonstrated ADH expression by lymphoma cells. These findings highlight the importance of using an immunohistochemical analysis to determine ADH production by tumor cells in patients with SIADH. Such analyses play an important role in elucidating the pathogenesis of SIADH and determining the most appropriate treatment.

Pulmonary amyloidosis is a rare disease that incorporates deposition of amyloid microfibril material in the lung parenchyma. The condition generally presents as an indolent subacute-to-chronic pulmonary disease and requires tissue biopsy to establish the diagnosis. Nodular pulmonary amyloidosis, a subtype of pulmonary amyloidosis, is characterised by special radiographic and pathological features. While the disease can be associated with inflammatory conditions; its association with mucosal-associated lymphoid tissue (MALT lymphoma) is unusual and carries management challenges. Herein, we illustrate a case study of nodular pulmonary amyloidosis associated with underlying MALT lymphoma in a patient with known systemic lupus erythematosus. The aim of this article is to share the management experience of this complex condition with the medical community and to conduct an up-to-date literature review on nodular pulmonary amyloidosis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Background: The inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. hypomyopathic/amyopathic DM. Anti-MDA5 dermatomyositis (DM) is a rare form of dermatomyositis that is frequently amyopathic; the prognosis is linked mainly to pulmonary involvement., Patients and Methods: A 69-year-old woman treated for mucosa-associated lymphoid tissue (MALT) gastric lymphoma was referred for a bullous eruption. Based on the investigations performed, a diagnosis was made of bullous pemphigoid. At the same time, amyopathic dermatomyositis was discovered together with interstitial lung disease. Systemic steroids were introduced in combination with rituximab. A favourable outcome was achieved., Discussion: Anti-MDA5 dermatomyositis must be considered systematically in all cases of pulmonary involvement associated with cutaneous signs of dermatomyositis, in which no muscular involvement is generally seen. This condition accounts for up to 7% of DM and carries a severe prognosis due to pulmonary involvement., (Copyright © 2017 Elsevier Masson SAS. All rights reserved.)

Extra-nodal mucosa-associated lymphoid tissue (MALT) lymphoma is frequently involved with the upper gastrointestinal tract, but rarely involved with the rectum. We report a case of rectal MALT lymphoma treated by radiotherapy (RT) alone. A 74-year-old woman with lower abdominal pain was diagnosed with MALT lymphoma by endoscopic mucosal resection (EMR). She was diagnosed as stage IE (Ann Arbor) MALT lymphoma by diagnostic work-up and review of EMR specimens. Definitive RT was performed with curative intent, totaling 30 Gy in 15 fractions. Complete response was confirmed by colonoscopy after RT with no progression observed at 5 years. Definitive RT is effective for rectal MALT lymphoma.