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Clinical Medicine (London, England) Nov 2023Lymphocytic oesophagitis is a rare inflammatory condition that was first described in 2006. Although it is being increasingly diagnosed, it remains poorly described and... (Review)
Review
Lymphocytic oesophagitis is a rare inflammatory condition that was first described in 2006. Although it is being increasingly diagnosed, it remains poorly described and characterised. There is limited research on the natural history, diagnosis and management of this condition. The most common presenting symptoms are dysphagia, chest pain and heartburn. Endoscopic features can mimic eosinophilic oesophagitis. International consensus is needed to secure a histological definition, to agree on an endoscopic severity scoring system and to determine an appropriate management algorithm. This review summarises the main evidence for the diagnosis and management of lymphocytic oesophagitis, thus setting the scene for the future directions needed to improve the management of this condition.
Topics: Humans; Eosinophilic Esophagitis; Gastritis
PubMed: 38065611
DOI: 10.7861/clinmed.2023-0440 -
Frontiers in Immunology 2022Due to the various clinical and pathological manifestations of kidney involvement in lymphoproliferative disorder (LPD), the whole spectrum of kidney disease in LPD is...
BACKGROUND
Due to the various clinical and pathological manifestations of kidney involvement in lymphoproliferative disorder (LPD), the whole spectrum of kidney disease in LPD is still unclear, and data on kidney prognosis is scarce.
METHODS
We retrospectively reviewed the renal pathology profiles from January 2010 to December 2021, and 28 patients with B-cell LPD combined with intact renal biopsy data were included.
RESULTS
There were 20 men and eight women aging 41 to 79 years at the time of renal biopsy (median age 62 years). According to hematological diagnosis, patients were classified into four groups: chronic lymphocytic leukemia (CLL) (group1, n=7), Waldenström macroglobulinemia/lymphoplasmacytic lymphoma (WM/LPL) (group 2, n=8; WM, n=6; LPL, n=2), Other non-Hodgkin's lymphomas (NHL) (group3, n=7; diffuse large B-cell lymphoma (DLBCL), n=2; mucosa-associated lymphoid tissue (MALT) lymphoma, n=4; Low grade B-cell lymphoma, n=1), and monoclonal gammopathy of undetermined significance/monoclonal gammopathy of renal significance (MGUS/MGRS) (group 4, n=6). Median serum creatinine (Scr) level was 129 (range,59-956) umol/L. Eight patients (29%) were presented with acute kidney injury (AKI), and five patients (18%) required hemodialysis upon admission. Twenty-three patients (82%) presented with proteinuria (median protein excretion, 2.14 g/d), 11(39%) of whom had the nephrotic syndrome. Interstitial malignant infiltration was the most frequent renal lesion (n=6). Eight patients underwent immunohistochemistry of renal tissues, of which three patients (CLL, n=1; LPL, n=1; WM, n=1) had confirmed lymphoma infiltrates, and the infiltrating cells in the remaining five patients (CLL, n=1; MALT lymphoma, n=2; MGUS, n=2) were considered unrelated to lymphoma. The most common glomerular diseases were renal amyloidosis (n=4) and membranous nephropathy (n=4). Only 20 patients were treated, 13 of whom were treated with rituximab separately or in combination. The median follow-up time was 11 months. Of these, six had achieved hematological response, complete response in five cases. Eight had achieved renal response. At the end-of-study visit, four patients died and two progressed to end stage kidney disease (ESKD).
CONCLUSION
In conclusion, the clinicopathological spectrum of renal involvement in BLPD is diverse. Renal biopsy and immunohistochemistry are required for early diagnosis and prognostic assessment.
Topics: Acute Kidney Injury; Creatinine; Female; Humans; Kidney; Leukemia, Lymphocytic, Chronic, B-Cell; Leukocyte Disorders; Lymphoma, B-Cell, Marginal Zone; Lymphoproliferative Disorders; Male; Middle Aged; Monoclonal Gammopathy of Undetermined Significance; Retrospective Studies; Rituximab; Waldenstrom Macroglobulinemia
PubMed: 36172352
DOI: 10.3389/fimmu.2022.903315 -
Canadian Medical Association Journal Oct 1964
Topics: Alpha-Globulins; Biopsy; Blood Protein Electrophoresis; Complement Fixation Tests; Diethylcarbamazine; Drug Therapy; Eosinophilia; Fever; Humans; Leukocyte Disorders; Male; Muscles; Prostatitis; Skin Manifestations; Trichinellosis
PubMed: 14217252
DOI: No ID Found -
Archives of Disease in Childhood Dec 1985We report two children with eosinophilic gastroenteritis--a 14 month old atopic boy with persistent vomiting and aspiration pneumonitis illustrates the mucosal variety...
We report two children with eosinophilic gastroenteritis--a 14 month old atopic boy with persistent vomiting and aspiration pneumonitis illustrates the mucosal variety of the disorder, and a 9 year old boy with eosinophilic ascites typifies serosal involvement.
Topics: Child; Eosinophilia; Food Hypersensitivity; Gastroenteritis; Humans; Infant; Male
PubMed: 4091587
DOI: 10.1136/adc.60.12.1186 -
Archives of Pathology & Laboratory... Apr 2007Kimura disease is a benign rare chronic inflammatory disorder of unknown etiology that involves the lymph nodes and subcutaneous tissue of the head and neck regions.... (Review)
Review
Kimura disease is a benign rare chronic inflammatory disorder of unknown etiology that involves the lymph nodes and subcutaneous tissue of the head and neck regions. Elevated serum immunoglobulin E levels and peripheral blood eosinophilia are also common. This disease is most common in middle-aged Asian men. Although the etiology is unknown, it most probably represents an aberrant chronic immune response. Treatment for Kimura disease includes surgical resection and regional or systemic steroid therapy. Cytotoxic therapy and radiation have also been utilized. The disease has an excellent prognosis, although it may recur locally.
Topics: Angiolymphoid Hyperplasia with Eosinophilia; Diagnosis, Differential; Female; Humans; Male; Sex Factors
PubMed: 17425383
DOI: 10.5858/2007-131-650-KD -
Journal of Gastrointestinal and Liver... Mar 2020Eosinophilic esophagitis (EoE) is an eosinophil-rich, Th2 antigen-mediated disease of increasing worldwide prevalence. Originally considered common in children and young... (Review)
Review
Eosinophilic esophagitis (EoE) is an eosinophil-rich, Th2 antigen-mediated disease of increasing worldwide prevalence. Originally considered common in children and young adults, it can be seen at any age, with the highest prevalence between 30 and 40 years. Symptoms reflect esophageal dysfunction, and typical endoscopic pictures consist of rings, furrows, exudates and edema. Progressive disease leads to pathologic tissue remodeling, with ensuing esophageal rigidity and loss of luminal diameter caused by strictures. The definitive diagnosis is histological (at least 15 eosinophils/HPF, high power field), upper gastrointestinal endoscopy with multiple esophageal biopsies being mandatory. Current therapeutic options include dietary and pharmacologic treatments. Despite being successful in a high proportion of patients, elemental diet has multiple disadvantages. Therefore, a step-up approach (using a two-, four- and six food elimination diets) is preferred, being globally effective in up to 79% of cases and avoiding unnecessary restrictions. Drug therapy relies on proton pump inhibitors and topical corticosteroids. Esophageal dilation may be required to increase luminal patency, leading to immediate symptomatic improvement in 95% of EoE patients, who have strictures or narrow caliber esophagus. The chronic nature of the disease necessitates long-term therapy. In this review, current diagnostic and treatment options are discussed and a treatment algorithm is proposed.
Topics: Disease Management; Eosinophilic Esophagitis; Humans
PubMed: 32176746
DOI: 10.15403/jgld-768 -
Ugeskrift For Laeger Aug 2022Eosinophilic oesophagitis (EoE) is a clinicopathological condition characterised by symptoms of oesophageal dysfunction and eosinophilic inflammation. The EoE incidence... (Review)
Review
Eosinophilic oesophagitis (EoE) is a clinicopathological condition characterised by symptoms of oesophageal dysfunction and eosinophilic inflammation. The EoE incidence is increasing rapidly as described in this review. In Denmark, EoE among children is significantly underdiagnosed. Untreated EoE is associated with low quality of life, and development of complications. EoE is effectively treated with proton pump inhibitors, diet, or topical corticosteroids. However, evaluation of the treatment response requires histological samples, since conflicting symptoms and histology is observed in 40%.
Topics: Child; Enteritis; Eosinophilia; Eosinophilic Esophagitis; Gastritis; Humans; Proton Pump Inhibitors; Quality of Life
PubMed: 35959825
DOI: No ID Found -
Blood May 2019Primary immunodeficiencies affecting the function of neutrophils and other phagocytic leukocytes are notable for an increased susceptibility to bacterial and fungal... (Review)
Review
Primary immunodeficiencies affecting the function of neutrophils and other phagocytic leukocytes are notable for an increased susceptibility to bacterial and fungal infections as a result of impaired leukocyte recruitment, ingestion, and/or killing of microbes. The underlying molecular defects can also impact other innate immune responses to infectious and inflammatory stimuli, leading to inflammatory and autoimmune complications that are not always directly related to infection. This review will provide an update on congenital disorders affecting neutrophil function in which a combination of host defense and inflammatory complications are prominent, including nicotinamide dinucleotide phosphate oxidase defects in chronic granulomatous disease and β2 integrin defects in leukocyte adhesion deficiency.
Topics: Animals; Autoimmune Diseases; Genetic Variation; Granulomatous Disease, Chronic; Humans; Immunity, Innate; Inflammation; NADPH Oxidases; Neutrophils
PubMed: 30898864
DOI: 10.1182/blood-2018-11-844563 -
Orphanet Journal of Rare Diseases Sep 2007Hypereosinophilic syndromes (HES) constitute a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia (> 1.5 x 10(9)/L for more... (Review)
Review
Hypereosinophilic syndromes (HES) constitute a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia (> 1.5 x 10(9)/L for more than six consecutive months) associated with evidence of eosinophil-induced organ damage, where other causes of hypereosinophilia such as allergic, parasitic, and malignant disorders have been excluded. Prevalence is unknown. HES occur most frequently in young to middle-aged patients, but may concern any age group. Male predominance (4-9:1 ratio) has been reported in historic series but this is likely to reflect the quasi-exclusive male distribution of a sporadic hematopoietic stem cell mutation found in a recently characterized disease variant. Target-organ damage mediated by eosinophils is highly variable among patients, with involvement of skin, heart, lungs, and central and peripheral nervous systems in more than 50% of cases. Other frequently observed complications include hepato- and/or splenomegaly, eosinophilic gastroenteritis, and coagulation disorders. Recent advances in underlying pathogenesis have established that hypereosinophilia may be due either to primitive involvement of myeloid cells, essentially due to occurrence of an interstitial chromosomal deletion on 4q12 leading to creation of the FIP1L1-PDGFRA fusion gene (F/P+ variant), or to increased interleukin (IL)-5 production by a clonally expanded T cell population (lymphocytic variant), most frequently characterized by a CD3-CD4+ phenotype. Diagnosis of HES relies on observation of persistent and marked hypereosinophilia responsible for target-organ damage, and exclusion of underlying causes of hypereosinophilia, including allergic and parasitic disorders, solid and hematological malignancies, Churg-Strauss disease, and HTLV infection. Once these criteria are fulfilled, further testing for eventual pathogenic classification is warranted using appropriate cytogenetic and functional approaches. Therapeutic management should be adjusted to disease severity and eventual detection of pathogenic variants. For F/P+ patients, imatinib has undisputedly become first line therapy. For others, corticosteroids are generally administered initially, followed by agents such as hydroxycarbamide, interferon-alpha, and imatinib, for corticosteroid-resistant cases, as well as for corticosteroid-sparing purposes. Recent data suggest that mepolizumab, an anti-IL-5 antibody, is an effective corticosteroid-sparing agent for F/P-negative patients. Prognosis has improved significantly since definition of HES, and currently depends on development of irreversible heart failure, as well as eventual malignant transformation of myeloid or lymphoid cells.
Topics: Animals; Humans; Hypereosinophilic Syndrome
PubMed: 17848188
DOI: 10.1186/1750-1172-2-37 -
World Journal of Gastroenterology Jun 2009Eosinophilic colitis (EC) is a rare form of primary eosinophilic gastrointestinal disease with a bimodal peak of prevalence in neonates and young adults. EC remains a... (Review)
Review
Eosinophilic colitis (EC) is a rare form of primary eosinophilic gastrointestinal disease with a bimodal peak of prevalence in neonates and young adults. EC remains a little understood condition in contrast to the increasingly recognized eosinophilic esophagitis. Clinical presentation of EC is highly variable according to mucosal, transmural, or serosal predominance of inflammation. EC has a broad differential diagnosis because colon tissue eosinophilia often occurs in parasitic infection, drug-induced allergic reactions, inflammatory bowel disease, and various connective tissue disorders, which require thorough searching for secondary causes that may be specifically treated with antibiotics or dietary and drug elimination. Like eosinophilic gastrointestinal disease involving other segments of the gastrointestinal tract, EC responds very well to steroids that may be spared by using antihistamines, leukotriene inhibitors and biologics.
Topics: Colitis; Colon; Eosinophilia; Humans
PubMed: 19554649
DOI: 10.3748/wjg.15.2975