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Key Clinical Message: A unique ER/PR‐positive mucinous adenocarcinoma of breast but CK7/CK20 negativity. A rare finding of Lymphoplasmacytic Infiltration is noted. Successful treatment included modified radical mastectomy and tamoxifen‐based therapy, leads to remission. Mucinous carcinoma, which is characterized by the presence of abundant extracellular mucin, is a rare and distinctive subtype of invasive breast cancer. This subtype accounts for less than 5% of all invasive breast cancers, with pure mucinous carcinoma representing only around 2%. It is most commonly found in older patients, typically in the sixth to early eighth decade of life. We present the case of a 55‐year‐old female with a 15‐year history of a painless, gradually enlarging palpable lump on her left breast. Upon examination, a 5 × 5 cm lump with well‐defined margins and a hard consistency was found in the lower outer quadrant of the left breast. Histopathological examination confirmed a diagnosis of Mucinous Adenocarcinoma of the Left Breast. The patient underwent modified radical mastectomy and received adjuvant endocrine therapy with tamoxifen due to hormone receptor‐positive status. Mucinous carcinoma of the breast is a unique entity with specific histological features including the presence of extracellular mucin pools. Immunohistochemical staining is crucial for determining hormone receptor status, which can guide treatment decisions. Although surgical intervention is the primary approach, the extent of surgery may vary, ranging from lumpectomy to mastectomy. Adjuvant therapies such as chemotherapy and radiotherapy may also be considered based on individual cases. This case underscores the importance of a multidisciplinary approach in managing rare subtypes of breast cancer, such as mucinous carcinoma. Accurate diagnosis, appropriate surgical intervention, adjuvant therapy, and long‐term follow‐up are critical components of treatment. [ABSTRACT FROM AUTHOR]

Key Clinical Message A unique ER/PR‐positive mucinous adenocarcinoma of breast but CK7/CK20 negativity. A rare finding of Lymphoplasmacytic Infiltration is noted. Successful treatment included modified radical mastectomy and tamoxifen‐based therapy, leads to remission. Abstract Mucinous carcinoma, which is characterized by the presence of abundant extracellular mucin, is a rare and distinctive subtype of invasive breast cancer. This subtype accounts for less than 5% of all invasive breast cancers, with pure mucinous carcinoma representing only around 2%. It is most commonly found in older patients, typically in the sixth to early eighth decade of life. We present the case of a 55‐year‐old female with a 15‐year history of a painless, gradually enlarging palpable lump on her left breast. Upon examination, a 5 × 5 cm lump with well‐defined margins and a hard consistency was found in the lower outer quadrant of the left breast. Histopathological examination confirmed a diagnosis of Mucinous Adenocarcinoma of the Left Breast. The patient underwent modified radical mastectomy and received adjuvant endocrine therapy with tamoxifen due to hormone receptor‐positive status. Mucinous carcinoma of the breast is a unique entity with specific histological features including the presence of extracellular mucin pools. Immunohistochemical staining is crucial for determining hormone receptor status, which can guide treatment decisions. Although surgical intervention is the primary approach, the extent of surgery may vary, ranging from lumpectomy to mastectomy. Adjuvant therapies such as chemotherapy and radiotherapy may also be considered based on individual cases. This case underscores the importance of a multidisciplinary approach in managing rare subtypes of breast cancer, such as mucinous carcinoma. Accurate diagnosis, appropriate surgical intervention, adjuvant therapy, and long‐term follow‐up are critical components of treatment.

Key Clinical Message: Waldenström's macroglobulinemia may begin with constitutional symptoms that are common in primary care settings and it is crucial for physicians to be aware of the potential complications of hyperviscosity syndrome and to employ the appropriate diagnostic methods in order to achieve better outcomes. Waldenström's macroglobulinemia (WM) refers to a type of lymphoplasmacytic lymphoma distinguished by the hyperproliferation of plasma cells, lymphocytes, and plasmacytoid lymphocytes. The disease is primarily diagnosed by increased monoclonal immunoglobulin M (IgM) levels and lymphoplasmacytic cell infiltration into the bone marrow. Individuals exhibit a high risk for hyperviscosity syndrome (HVS) as immunoglobulin levels increase. In addition to constitutional symptoms (fever, night sweats, and unintentional weight loss), clinical findings such as cytopenia, hepatosplenomegaly, and lymphadenopathy, this condition may cause hyperviscosity‐related organ failures. Here we discuss a patient with WM who presented with neurological complaints and blurry vision and developed necrosis at distal portions of his body during the 6‐month course of the disease. [ABSTRACT FROM AUTHOR]

Key Clinical Message Waldenström's macroglobulinemia may begin with constitutional symptoms that are common in primary care settings and it is crucial for physicians to be aware of the potential complications of hyperviscosity syndrome and to employ the appropriate diagnostic methods in order to achieve better outcomes. Abstract Waldenström's macroglobulinemia (WM) refers to a type of lymphoplasmacytic lymphoma distinguished by the hyperproliferation of plasma cells, lymphocytes, and plasmacytoid lymphocytes. The disease is primarily diagnosed by increased monoclonal immunoglobulin M (IgM) levels and lymphoplasmacytic cell infiltration into the bone marrow. Individuals exhibit a high risk for hyperviscosity syndrome (HVS) as immunoglobulin levels increase. In addition to constitutional symptoms (fever, night sweats, and unintentional weight loss), clinical findings such as cytopenia, hepatosplenomegaly, and lymphadenopathy, this condition may cause hyperviscosity‐related organ failures. Here we discuss a patient with WM who presented with neurological complaints and blurry vision and developed necrosis at distal portions of his body during the 6‐month course of the disease.

Ultrasonographic intestinal muscularis thickening has not been described as an imaging feature of canine inflammatory bowel disease. In this retrospective case series, patients were identified by searching sonographic reports for "muscularis" and/or "muscular layer." Patients were included if small intestinal muscularis thickening was reported, and sonographic images and histopathological samples of the small intestine were available for review. Cases with small intestines nodules, masses, or complete loss of wall layering were excluded. Sonographic images were retrospectively evaluated for jejunal muscularis layer thickness, and ratios of intestinal layer measurements were performed. Histological samples were retrospectively reviewed. Thirteen dogs met inclusion criteria: all dogs had sonographic intestinal muscularis thickening relative to the submucosa (>1.0, range of 1.3–2.5), and most dogs had muscular layer thickness above normal published ranges (11/13; all 13/13 above the weight‐specific mean). More than half of the patients had overall normal wall thickness (11/13) and several had normal mucosal echogenicity (6/13). Therefore, in some dogs, the only sonographic abnormality in the small intestine was muscularis thickening. No dogs had lymphadenomegaly. Endoscopic partial‐thickness (n = 11, duodenum and/or ileum) or surgical full‐thickness (n = 2) samples confirmed inflammatory bowel disease. Direct comparison between jejunum sonographic characteristics and histology features was limited due to both partial thickness biopsies and lack of direct comparison between anatomical locations of ultrasonographic assessment and biopsy site. However, no cases that met the inclusion criteria had normal small intestinal histology. Comparable to cats, dogs with ultrasonographic intestinal muscularis thickening may have inflammatory bowel disease, and further workup for enteropathy is indicated. [ABSTRACT FROM AUTHOR]

BackgroundEffective treatments are needed for idiopathic chronic rhinitis in dogs, but assessment of efficacy requires a practical, quantifiable method for assessing severity of disease.ObjectivesTo develop and perform initial validity and reliability testing of an owner-completed questionnaire for assessing clinical signs and dog and owner quality of life (QOL) in canine chronic rhinitis.AnimalsTwenty-two dogs with histopathologically confirmed chronic rhinitis and 72 healthy dogs.MethodsIn this prospective study, an online questionnaire was created based on literature review and feedback from veterinarians, veterinary internists with respiratory expertise, and owners of dogs with rhinitis. Owners of affected dogs completed the questionnaire twice, 1 week apart, to test reliability. Healthy dogs were assessed once. Data were analyzed using the Rasch Rating Scale Model, and results were interpreted using Messick's framework for evaluating construct validity evidence.ResultsInitial item generation resulted in 5 domains: nasal signs, paranasal signs, global rhinitis severity, and dog's and owner's QOL. A 25-item questionnaire was developed using 5-point Likert-type scales. No respondent found the questionnaire difficult to complete. Strong psychometric evidence was available to support the substantive, generalizability, content, and structural aspects of construct validity. Statistical differences were found between responses for affected and control dogs for all but 2 items. These items were eliminated, resulting in the 23-item Severity of Nasal Inflammatory Disease (SNIFLD) questionnaire.Conclusions and clinical importanceThe SNIFLD questionnaire provides a mechanism for repeated assessments of disease severity in dogs with chronic rhinitis.

Although IgG4‐RD has CNS manifestations, cerebellar involvement has only been reported in three cases. Our patient presented with cerebellar symptoms, several cerebellar infarcts were evident on the brain MRI, and CT abdomen revealed retroperitoneal tumor. Endoscopic biopsy confirmed IgG4‐RD. Steroids are the first‐line therapy for IgG4‐RD, but our patient was lost to follow‐up before treatment. [ABSTRACT FROM AUTHOR]

Abstract Although IgG4‐RD has CNS manifestations, cerebellar involvement has only been reported in three cases. Our patient presented with cerebellar symptoms, several cerebellar infarcts were evident on the brain MRI, and CT abdomen revealed retroperitoneal tumor. Endoscopic biopsy confirmed IgG4‐RD. Steroids are the first‐line therapy for IgG4‐RD, but our patient was lost to follow‐up before treatment.

Abstract Background Our case of a patient with untreated lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia with extramedullary pleural effusion is the first documented case of pleural fluid MYD88 L265P mutation status in a community hospital setting. Our patient was intolerant to 420 mg ibrutinib, but still achieved a lasting complete remission, as defined by National Comprehensive Cancer Network guidelines, with a dose reduction to 240 mg of ibrutinib. Case presentation A 72-year-old Caucasian (white) man diagnosed with monoclonal immunoglobin M kappa lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia monitored without treatment for 2 years, presented with dyspnea and a left pleural effusion. At presentation, computed tomography scans of his chest, abdomen, and pelvis showed layering left pleural effusion and para-aortic lymphadenopathy. Pleural fluid cytology demonstrated B-cell lymphoma of the lymphoplasmacytic subtype, with monoclonal kappa B-cell population on flow and a positive MYD88 L265P mutation. The pleural effusion recurred post-thoracentesis and he achieved a lasting complete remission as defined by National Comprehensive Cancer Network guideline with 240 mg ibrutinib. Conclusions Our discussion details a comprehensive literature review of extramedullary pulmonary involvement in Waldenstrom’s macroglobulinemia. Establishing a malignant etiology for pleural effusion in Waldenstrom’s macroglobulinemia can be challenging, as standard techniques may be insensitive. Allele-specific polymerase chain reaction for detecting MYD88 L265P mutations is more sensitive for confirming lymphoplasmacytic lymphoma/Waldenstrom’s macroglobulinemia in pleural fluid. Extramedullary pulmonary involvement usually presents post-diagnosis of Waldenstrom’s macroglobulinemia and responds well to Waldenstrom’s macroglobulinemia-directed treatment regimens. Allele-specific polymerase chain reaction is a sensitive assay for detecting MYD88 L265P mutations in pleural fluid to support the diagnosis of malignant pleural effusion in the setting of Waldenstrom’s macroglobulinemia and helps guide the treatment decision to use ibrutinib. Although intolerant of ibrutinib 420 mg, our patient achieved complete and sustained remission of pleural effusion with a dose of 240 mg with progression free survival of over 30 months.

Waldenstrom's macroglobulinemia (WM) is a rare and slowly progressive disorder, a variant of lymphoplasmacytic lymphoma, which needs therapy only when patient becomes symptomatic. WM presents usually with constitutional symptoms, organomegaly, cytopenias, and hyperviscosity syndrome. This neoplasm is composed of small lymphocytes, plasmacytoid lymphocytes, and plasma cells that typically involve the bone marrow, and it is associated with an immunoglobulin M (IgM) gammopathy. Here we report the case a 60-year-old male with WM who initially presented with anemia and fatigue. The patient had no lymphadenopathy or any organomegaly. The diagnosis of WM was made after morphological and immunohistochemical examination of bone marrow of the patient along with an elevated serum IgM level. The patient responded well to plasmapheresis and chemotherapy. This case is unusual because the patient lacked the common clinical features of WM. A thorough clinical and hematological work up including serum electrophoresis, bone marrow study, and immunohistochemistry helps in distinguishing WM from other lymphomas and plasma cell dyscrasias.

Background: Waldenstrom macroglobulinemia (WM) is a rare mature B cell Neoplasm, characterized by monoclonal Immunoglobulin M (IgM) in the serum and lymphoplasmacytic infiltration in the bone marrow. It accounts for approx 2% of Non Hodgkin Lymphomas. The disease is slightly more common in elderly males, the median age at diagnosis being 63 years. The disease is very infrequent in India and only a few studies have been published on the disease. Hence, we bring out this study on Clinico-Pathological Profile of WM. Aims and Objectives: To study clinico-pathological profile of WM cases and to distinguish them from other plasma cell neoplasms as both the diseases have different treatment protocols and prognosis. Materials and Methods: A cross sectional observational study was conducted at tertiary care center over a period of two years. Six cases of WM were encountered and enrolled for the study due to their rarity and morphological overlap with plasma cell neoplasms. The WHO criteria were followed and patients fulfilling those criteria were included in the study. The diagnosis of WM was based on the morphologic and immunohistochemistry findings of biopsy specimens, the presence of a monoclonal IgM, and the presence of distinctive clinical characteristics. Results: Median age of patients was 60.5 years with a male preponderance. 33% of cases presented with B symptoms and 100% cases presented with moderate to severe anaemia. 50% of the cases had thrombocytopenia and hepatomegaly. All cases had albumin:globulin ratio reversal. Peripheral smear showed background staining, rouleaux formation, relative lymphocytosis. Plasmacytoid cells were present in two cases. Bone marrow aspirate smears showed features of lymphoplasmacytic infiltration with suppression of other lineages. BM biopsies were hypercellular and showed a diffuse pattern of marrow infiltration by lymphoplasmacytic cells. The immunostaining of the infiltrating lymphocytes were CD 20+ and plasma cells were CD138+. Serum protein electrophoresis and immunofixation electrophoresis confirmed IgM type of monoclonal gammopathy. Conclusion: WM is relatively a rare mature B cell neoplasm which at times can have morphological overlap with other plasma cell neoplasms. We conclude that correlation with clinical laboratory test results while making diagnostic decision is an integral part of recognition of WM due to low grade indolent course of the disease and better outcome.

Background: Our case of a patient with untreated lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia with extramedullary pleural effusion is the first documented case of pleural fluid MYD88 L265P mutation status in a community hospital setting. Our patient was intolerant to 420 mg ibrutinib, but still achieved a lasting complete remission, as defined by National Comprehensive Cancer Network guidelines, with a dose reduction to 240 mg of ibrutinib.Case Presentation: A 72-year-old Caucasian (white) man diagnosed with monoclonal immunoglobin M kappa lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia monitored without treatment for 2 years, presented with dyspnea and a left pleural effusion. At presentation, computed tomography scans of his chest, abdomen, and pelvis showed layering left pleural effusion and para-aortic lymphadenopathy. Pleural fluid cytology demonstrated B-cell lymphoma of the lymphoplasmacytic subtype, with monoclonal kappa B-cell population on flow and a positive MYD88 L265P mutation. The pleural effusion recurred post-thoracentesis and he achieved a lasting complete remission as defined by National Comprehensive Cancer Network guideline with 240 mg ibrutinib.Conclusions: Our discussion details a comprehensive literature review of extramedullary pulmonary involvement in Waldenstrom's macroglobulinemia. Establishing a malignant etiology for pleural effusion in Waldenstrom's macroglobulinemia can be challenging, as standard techniques may be insensitive. Allele-specific polymerase chain reaction for detecting MYD88 L265P mutations is more sensitive for confirming lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia in pleural fluid. Extramedullary pulmonary involvement usually presents post-diagnosis of Waldenstrom's macroglobulinemia and responds well to Waldenstrom's macroglobulinemia-directed treatment regimens. Allele-specific polymerase chain reaction is a sensitive assay for detecting MYD88 L265P mutations in pleural fluid to support the diagnosis of malignant pleural effusion in the setting of Waldenstrom's macroglobulinemia and helps guide the treatment decision to use ibrutinib. Although intolerant of ibrutinib 420 mg, our patient achieved complete and sustained remission of pleural effusion with a dose of 240 mg with progression free survival of over 30 months. [ABSTRACT FROM AUTHOR]

Background: Waldenstrom macroglobulinemia (WM) is a rare mature B cell Neoplasm, characterized by monoclonal Immunoglobulin M (IgM) in the serum and lymphoplasmacytic infiltration in the bone marrow. It accounts for approx 2% of Non Hodgkin Lymphomas. The disease is slightly more common in elderly males, the median age at diagnosis being 63 years. The disease is very infrequent in India and only a few studies have been published on the disease. Hence, we bring out this study on Clinico-Pathological Profile of WM. Aims and Objectives: To study clinico-pathological profile of WM cases and to distinguish them from other plasma cell neoplasms as both the diseases have different treatment protocols and prognosis. Materials and Methods: A cross sectional observational study was conducted at tertiary care center over a period of two years. Six cases of WM were encountered and enrolled for the study due to their rarity and morphological overlap with plasma cell neoplasms. The WHO criteria were followed and patients fulfilling those criteria were included in the study. The diagnosis of WM was based on the morphologic and immunohistochemistry findings of biopsy specimens, the presence of a monoclonal IgM, and the presence of distinctive clinical characteristics. Results: Median age of patients was 60.5 years with a male preponderance. 33% of cases presented with B symptoms and 100% cases presented with moderate to severe anaemia. 50% of the cases had thrombocytopenia and hepatomegaly. All cases had albumin:globulin ratio reversal. Peripheral smear showed background staining, rouleaux formation, relative lymphocytosis. Plasmacytoid cells were present in two cases. Bone marrow aspirate smears showed features of lymphoplasmacytic infiltration with suppression of other lineages. BM biopsies were hypercellular and showed a diffuse pattern of marrow infiltration by lymphoplasmacytic cells. The immunostaining of the infiltrating lymphocytes were CD 20+ and plasma cells were CD138+. Serum protein electrophoresis and immunofixation electrophoresis confirmed IgM type of monoclonal gammopathy. Conclusion: WM is relatively a rare mature B cell neoplasm which at times can have morphological overlap with other plasma cell neoplasms. We conclude that correlation with clinical laboratory test results while making diagnostic decision is an integral part of recognition of WM due to low grade indolent course of the disease and better outcome. [ABSTRACT FROM AUTHOR]

Waldenstrom's macroglobulinemia (WM) is a rare and slowly progressive disorder, a variant of lymphoplasmacytic lymphoma, which needs therapy only when patient becomes symptomatic. WM presents usually with constitutional symptoms, organomegaly, cytopenias, and hyperviscosity syndrome. This neoplasm is composed of small lymphocytes, plasmacytoid lymphocytes, and plasma cells that typically involve the bone marrow, and it is associated with an immunoglobulin M (IgM) gammopathy. Here we report the case a 60-year-old male with WM who initially presented with anemia and fatigue. The patient had no lymphadenopathy or any organomegaly. The diagnosis of WM was made after morphological and immunohistochemical examination of bone marrow of the patient along with an elevated serum IgM level. The patient responded well to plasmapheresis and chemotherapy. This case is unusual because the patient lacked the common clinical features of WM. A thorough clinical and hematological work up including serum electrophoresis, bone marrow study, and immunohistochemistry helps in distinguishing WM from other lymphomas and plasma cell dyscrasias. [ABSTRACT FROM AUTHOR]

We report a rare case of a 68-year-old male who presented with fever, weight loss, nasal blockage, and epistaxis. Examination revealed cervical and axillary lymphadenopathy with no evidence of organomegaly. On evaluation, bone marrow aspiration showed lymphoplasmacytic infiltration. The computed tomography of the neck showed nasopharyngeal mass and the biopsy of this mass and cervical lymph node showed lymphoplasmacytic lymphoma (LPL) with high serum IgM level. Hence, a diagnosis of Waldenstrom's macroglobulinemia (WM) was made. The patient received six cycles of chemotherapy with a combination of cyclophosphamide, vincristine, and prednisolone (COP regimen). Currently, the patient is under follow-up and in complete remission (CR), one year after completion of therapy. Nasopharyngeal involvement is extremely rare in WM, and hence we report this case. [ABSTRACT FROM AUTHOR]

Lymphoplasmacytic gastritis is a concern for toxicological pathologists reading preclinical, non-human primate toxicity studies because subtle gastric changes which could be treatment-related may be masked and gastritis lesions may be confused with treatment-related effects and thus a gastric finding may be incorrectly assigned as a treatment-related lesion. This paper discusses the incidence of lymphoplasmacytic gastritis in cynomolgus monkeys at a contract research organization. The incidence of lymphoplasmacytic gastritis in the fundus and antrum of control cynomolgus monkeys on 18 non-gastric compound studies, was scored. The average fundus score ranged from 0.3 to 1.5 and the average antral score ranged from 0.9 to 3.5 in the cynomolgus monkey stomachs examined. The number of affected control animals in a study ranged from 0 to 5 control animals. No correlation between the route of vehicle administration and the severity or incidence of the lesions was noted. The percentage incidence of affected animals ranged from 0 to 100%. An increased incidence lymphoplasmatic gastritis from 2000 to 2004 was noted. The implications of lymphoplasmacytic gastritis in cynomolgus monkeys used for acute toxicity studies are discussed. [ABSTRACT FROM AUTHOR]

Abstract: Autoimmune pancreatitis (AIP) is a form of chronic pancreatitis that typically affects adults with a male predilection. Jaundice, weight loss, anorexia and abdominal pain are the most common presenting symptoms. Serum IgG and IgG4 are frequently elevated. Imaging studies show diffuse enlargement of the pancreas with diffuse or segmental narrowing of the main pancreatic duct. When pancreatic involvement is focal, it can mimic adenocarcinoma on imaging. Histologically, the disease is characterized by a duct-centric fibroinflammatory process, dense lymphoplasmacytic infiltrates and phlebitis. High numbers of IgG4-positive plasma cells are frequently present. The absence of duct dilatation, pseudocyst, fat necrosis or calcification aids in the distinction from alcoholic pancreatitis. AIP can involve other sites in the pancreaticobilairy tree and is frequently associated with other autoimmune and fibrosclerosing diseases. This has led to the concept that AIP is the pancreatic manifestation of systemic IgG4-related immunological disease. The prognosis is generally favourable with an excellent response to steroids. [Copyright &y& Elsevier]

Chronic inflammatory rhinitis is commonly found in dogs with chronic nasal disease and is characterized by lymphoplasmacytic infiltrates in the nasal mucosa in the absence of an obvious etiologic process. The pathogenesis of lymphoplasmacytic rhinitis remains unknown. Animals respond poorly to antibiotics, oral glucocorticoids, and antihistamines, making primary infectious, immune-mediated, or allergic etiologies unlikely. Aberrant immune response to inhaled organisms or allergens may induce inflammation in some animals. Common clinical signs include nasal discharge, sneezing, coughing, epistaxis, and stertor. Diagnosis is made by performing a thorough history, physical examination, radiography or advanced imaging (via computed tomography or magnetic resonance imaging), rhinoscopy, and nasal mucosal biopsy to rule out primary etiologies of nasal discharge. Treatment strategies have included various antibiotics, antihistamines, oral and inhalant steroids, nonsteroidal antiinflammatories, and antifungal medications. Some dogs may respond partially to doxycycline or azithromycin, although it is unclear whether response is related to antimicrobial or antiinflammatory properties of these drugs. Hydration of the nasal cavity through nasal drops or aerosols may limit nasal discharge, and some animals may improve with inhalant (but rarely oral) glucocorticoids. [Copyright &y& Elsevier]

Human monoclonal IgM associated with a demyelinating peripheral neuropathy often feature a distinct antibody activity directed against a glucuronyl sulphate epitope shared by myelinassociated glycoprotein (MAG), nerve glycolipids and low molecular weight peripheral nerve polypeptides. Earlier studies showed that these IgM use a diverse repertoire of VH and VL genes which exhibit somatic mutations, possibly indicative of an antigen-driven process. Here, we investigated whether such monoclonal IgM may react with environmental bacterial antigens. We found that six patients' sera and purified monoclonal IgM, as well as IgM from supernatants of three clonal anti-MAG-secreting cell lines reacted with unique 90-100 kD polypeptides from extracts of two out of 10 bacterial species. Purified MAG was able to inhibit this reactivity. These results indicate molecular mimicry as a possible mechanism of this immunomediated neuropathy and associated clonal lymphoid disease. [ABSTRACT FROM AUTHOR]

Abstract: We present the unusual case of a 33-year-old woman who presented with a 2year history of amenorrhoea and an expanding sellar lesion. Initial MRI revealed a lesion in the pituitary fossa, thought to be a pituitary adenoma. One year later, the lesion had enlarged by 5mm, with associated enhancement of the dura of the planum sphenoidale and pituitary stalk. Histopathology revealed a lymphocytic and plasma cell inflammatory infiltrate suggestive of lymphoplasmacytic hypophysitis associated with immunoglobulin G4. [Copyright &y& Elsevier]

Introduction: Due to recent advancements in the understanding of the molecular pathogenesis of B-cell malignancies, there has been an explosion of innovative agents in development. The purpose of this review is to efficiently summarize novel therapies with activity in indolent non-Hodgkin lymphoma (iNHL) targeting surface antigens, signaling pathways, and the tumor microenvironment. Areas covered: A literature search was performed to identify preclinical data and clinical trials focused on the use of targeted therapies in iNHL subtypes including follicular lymphoma, marginal zone lymphoma, small lymphocytic lymphoma, and lymphoplasmacytic lymphoma/Waldenström macroglobulinemia. Classes reviewed include monoclonal antibodies, antibody-drug conjugates, immunomodulatory agents, B-cell receptor pathway inhibitors, Bcl-2 inhibitors, checkpoint inhibitors, chromatin and epigenetic modulating agents, and CAR T-cells. Expert commentary: Opinions regarding strategies to address the prioritization of novel agents entering clinical development, the determination of rational combination therapy, the development of novel endpoints to expedite clinical development, and the movement towards novel consolidative approaches with immuno- and cellular therapy in an attempt to provide curative treatment options are provided. Also, the economic impact of indefinite therapy is discussed.

B-cell non-Hodgkin lymphomas with plasmacytic differentiation are a diverse group of entities with extremely variable morphologic features. Diagnostic challenges can arise in differentiating lymphoplasmacytic lymphoma from marginal zone lymphoma and other low-grade B-cell lymphomas. In addition, plasmablastic lymphomas can be difficult to distinguish from diffuse large B-cell lymphoma or other high-grade lymphomas. Judicious use of immunohistochemical studies and molecular testing can assist in appropriate classification., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Lymphoplasmacytic infiltrates in the breast, a modified skin appendage, include lymphocytic lobulitis, other nonspecific benign proliferations, and mucosa-associated lymphoid tissue (MALT)-type lymphoma. Distinguishing these entities, all of which may be B-cell rich and may have associated sclerosis, can be difficult. In addition, the proportion that represents IgG4-related disease is unknown, and the similarity of MALT lymphomas to primary cutaneous marginal zone lymphoma is uncertain. To address these questions, the clinical, histologic, and immunohistochemical features of 50 benign and malignant breast lymphoplasmacytic infiltrates (10 lymphocytic lobulitis, 1 granulomatous, 19 not otherwise specified, 20 MALT lymphomas) were evaluated. Compared with the MALT lymphomas, benign cases had a less dense infiltrate (P < .001), fewer but more histologically apparent germinal centers (P < .001), and more marked fibrosis (P < .0001). Greater than 60% B cells were present in 23% (7/30) benign cases versus 75% (15/20) MALT lymphomas (P = .0003). Plasma cells were predominantly IgG+ in 83% (24/29) benign cases and predominantly IgM+ in 73% (14/19) MALT lymphomas (P < .0001). None of the benign cases had greater than 50 IgG4+ plasma cells/high-power field, and only 1 lymphocytic lobulitis case had an IgG4/IgG ratio exceeding 40% and no clinical evidence for extramammary IgG4-related disease. Although there may be some overlapping features, routine histopathology together with limited immunohistochemical stains can distinguish benign from neoplastic lymphoplasmacytic infiltrates in the breast. Despite frequent sclerosis, the breast is not a common site of unrecognized IgG4-related sclerosing disease. Although there are similarities, breast MALT lymphomas can be separated from cutaneous marginal zone lymphoma., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Primary sclerosing cholangitis (PSC), first described in the mid-1850s, is a complex liver disease that is heterogeneous in its presentation. PSC is characterized by chronic cholestasis associated with chronic inflammation of the biliary epithelium, resulting in multifocal bile duct strictures that can affect the entire biliary tree. Chronic inflammation leads to fibrosis involving the hepatic parenchyma and biliary tree, which can lead to cirrhosis and malignancy. The etiology of PSC is not fully understood, which in part explains the lack of effective medical therapy for this condition. However, we have begun to better understand the molecular pathogenesis of PSC. The recognition of specific clinical subtypes and their pattern of progression could improve phenotypic and genotypic classification of the disease. We review our current understanding of this enigmatic disorder and discuss important topics for future studies., (Copyright © 2013 AGA Institute. Published by Elsevier Inc. All rights reserved.)