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Lymphoproliferative Disorders - Science topic

Disorders characterized by proliferation of lymphoid tissue, general or unspecified.
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Publications related to Lymphoproliferative Disorders (10,000)
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Background Castleman’s Disease (CD) is a diverse group of non-neoplastic lymphoproliferative disorders characterized by lymphoid endothelial and follicular hyperplasia. Human Immunodeficiency Virus (HIV)-associated Human Herpes Virus type-8 (HHV-8) CD is a form of CD affecting HIV-positive individuals with concomitant HHV-8 infection as the aetiolo...
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Atypical lymphoproliferative disorders (LPDs) related with autoimmune disease (AID) show marked clinicopathological diversity, which are defined as three distinct clinicopathological subtypes such as those resembling Castleman disease (CD), atypical paracortical hyperplasia with lymphoid follicles (APHLF), and atypical lymphoplasmacytic and immunob...
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We report a case of donor-derived diffuse large B-cell lymphoma (DLBCL), which developed 5 years after stem cell transplantation from a human leukocyte antigen (HLA)-haploidentical donor for acute myeloid leukemia (AML). A 51-year-old male was diagnosed with AML with variant KMT2A translocation involving t(6;11)(q13;q23). After 12 cycles of azaciti...
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The last 2 decades have seen great progress in understanding the pathogenesis of cold agglutinin disease (CAD) and development of effective therapies. Cold agglutinins can cause hemolytic anemia as well as peripheral circulatory symptoms such as acrocyanosis. We distinguish CAD, a well-defined clinicopathologic entity, from secondary cold agglutini...
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Etiological diagnosis of mediastinal lymphadenopathy represents a daily challenge. Endosonography (transesophageal and transbronchial ultrasound-guided needle aspiration) is the recommended technique in the first diagnostic work-up and in the mediastinal staging of lung cancer. Despite a good sensitivity, limited amount of collected tissue may hamp...
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In this study, we investigated the usefulness of FDG-PET/CT for predicting spontaneous regression in methotrexate-associated lymphoproliferative disorder (MTX-LPD). Twenty patients with rheumatoid arthritis who were diagnosed with MTX-LPD were enrolled in the study. These patients were divided into those who showed spontaneous regression (SR group:...
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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 patient...
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Background This case reveals a novel presentation of drug rash with eosinophilia and systemic symptoms syndrome that mimics a lymphoproliferative disorder. The heterogeneous clinical presentation of drug rash with eosinophilia and systemic symptoms syndrome gives rise to a broad differential diagnosis that includes a multitude of infectious, inflam...
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Castleman disease constitutes a rare class of lymphoproliferative disorders, with an estimated incidence of 21 to 25 per million patient years. The idiopathic subtype exhibits a significantly diverse clinical presentation, which can imitate many autoimmune, malignant, and infectious diseases. Cutaneous manifestations are uncommon and require in-dep...
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Lymphomas represent a heterogeneous and widely diversified group of neoplastic diseases rising from a variety of lymphoid subsets at heterogeneous differentiation stages. These lymphoproliferative disorders lead to the clinicopathological complexity of the classification of lymphoid neoplasms, describing to date more than 40 categories of non-Hodgk...
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Heng Zhang, Kun Li, Jianzhong Zhang, Xue Chen Department of Dermatology, Peking University People’s Hospital, Beijing, People’s Republic of ChinaCorrespondence: Xue Chen, Department of Dermatology, Peking University People’s Hospital, # 11. Xizhimen South St, Beijing, 100044, People’s Republic of China, Tel +8610 88325472, Fax +8610 88325474, Email...
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The application of intracellular and extracellular Epstein–Barr virus (EBV) DNA in allogeneic hematopoietic stem cell transplantation (allo-HSCT) has been poorly characterized. We conducted a combined prospective-retrospective study of 300 patients who underwent allo-HSCT between 2016 to 2019 in our center and monitored for EBV DNA within the first...
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The therapeutic landscape for lymphomas is quite diverse and includes active surveillance, chemotherapy, immunotherapy, radiation therapy, and even stem cell transplant. Advances in the field have led to the development of targeted therapies, agents that specifically act against a specific component within the critical molecular pathway involved in...
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Introduction Post-transplant lymphoproliferative disorders(PTLD) include a mix of rare yet life endangering complications. Case presentation and conclusion Here, we report a case of a 63-year-old man who was the victim of post-transplant lymphoproliferative disorder (Hodgkin's lymphoma). The patient was initially diagnosed with multiple myeloma, f...
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Access to antiretroviral therapy (ART) led to epidemiological changes in human immunodeficiency virus (HIV) associated lymphoma in high-income countries such as reductions in diffuse large B-cell lymphoma (DLBCL) and stable or increased Hodgkin lymphoma (HL) and Burkitt lymphoma (BL). In 2016, Malawi implemented a universal ART (UART) policy, expan...
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Castleman’s disease (CD) is a primary lymphoproliferative disorder of the lymph nodes with rare extra-nodal primary affection. Solid organ involvement is rare, and isolated liver involvement is extremely rare. Here we presented a case of a 59-year-old woman with a hepatic lesion accidentally found by ultrasound. The MRI result indicated primary liv...
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Mantle cell lymphoma (MCL) is a rare and aggressive non-Hodgkin’s B cell lymphoma characterized by the translocation t(11;14) (q13;32) and overexpression of CCND1 . MCL is immunophenotypically identified as CD20 ⁺ , CD5 ⁺ , CyclinD1+, CD43 ⁺ , CD10 ⁻ , BCL6 ⁻ , and CD23 ⁻ . It is often distinguished from B cell lymphomas of germinal center cell ori...
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Primary EBV-positive mucocutaneous ulcer (EBVMCU) is a rare and indolent disorder occurring in the oropharynx, skin, and gastrointestinal tract, with remission after removal of the immunosuppressive causes. We present a 69-year-old woman with heartburn, regurgitation of gastric acid, enlarged lymph nodes, and parotid glands. The endoscopic examinat...
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Compared to the general population the incidence of lymphoproliferative disorders (LPDs) is significantly elevated among people living with HIV (PLHIV). In high-income countries LPDs have become the most common HIV-associated cause of death among PLHIV. Lymphomas are one of the most frequent triggers of Hemophagocytic Lymphohistiocytosis (HLH), a l...
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B cell chronic lymphoproliferative diseases (B-CLPD) are associated with secondary antibody deficiency and other innate and adaptive immune defects, whose impact on infectious risk has not been systematically addressed. We performed an immunological analysis of a cohort of 83 B-CLPD patients with recurrent and/or severe infections to ascertain the...
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Objective Granuloma etiology includes infections, vasculitis, chemicals, malignancies, lymphoproliferative disorders, and immunological diseases. We hypothesized that patients with granuloma have an underlying primary immunodeficiency disease (PIDD).Patients and Methods We retrospectively enrolled 82 patients with immunological evaluation among 294...
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Background: Patients with a history of solid organ transplantation (SOT) or hematopoietic stem cell transplantation (HSCT) are at an increased risk of developing post-transplant lymphoproliferative disorder (PTLD). The gastrointestinal (GI) tract is commonly affected as it has an abundance of B and T cells. Aim: To determine typical GI-manifesta...
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Intestinal T/NK-cell lymphomas include enteropathy-associated T-cell lymphoma (EATL), monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL), indolent T-cell lymphoproliferative disorders of the GI tract (ITCLPD), extranodal NK/T-cell lymphoma, nasal type (ENKTL), and intestinal T-cell lymphoma NOS (ITCL-NOS). Here we describe a case of sur...
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The ZEBRA (Z EBV replication activator) protein is the major transcription factor of EBV, expressed upon EBV lytic cycle activation. An increasing body of studies have highlighted the critical role of EBV lytic infection as a risk factor for lymphoproliferative disorders, such as post-transplant lymphoproliferative disease (PTLD). We studied 108 tr...
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Autoimmune hemolytic anemia (AIHA) is a rare complication following heart transplantation and has been attributed to several etiologies including infections, immunosuppressive medications, and post-transplant lymphoproliferative disorders. We report a 23-year-old male presenting 22 years after heart transplantation with severe AIHA. Laboratory find...
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The prospective multicentre Phase II PTLD-2 trial (NCT02042391) tested modified risk-stratification in adult SOT recipients with CD20-positive PTLD based on principles established in the PTLD-1 trials: sequential treatment and risk-stratification. After rituximab monotherapy induction, patients in complete remission as well as those in partial remi...
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Epstein-Barr virus (EBV) infects more than 90% of the world’s adult population and accounts for a significant cancer burden of epithelial and B cell origins. Glycoprotein B (gB) is the primary fusogen essential for EBV entry into host cells. Here, we isolated two EBV gB-specific neutralizing antibodies, 3A3 and 3A5; both effectively neutralized the...
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Background Castleman disease (CD), classified as unicentric CD (UCD) or multicentric CD (MCD), is a rare non-neoplastic lymphoproliferative disorder of unknown origin. Owing to its rarity, the clinical characteristics, therapeutic modalities, treatment outcomes, and prognostic factors related to UCD or MCD are not well defined. Method We retrospec...
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Hydroa vacciniforme-like lymphoproliferative disorder (HV-LPD) is a cutaneous form of chronic active Epstein-Barrvirus (EBV) infection, which can develop into the extremely rare systemic lymphoma. Patients with Inborn errors of immunity (IEI), such as common variable immunodeficiency (CVID), are at higher risk of developing a severe course of infec...
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Methotrexate (MTX) is increasingly used in the treatment of rheumatoid arthritis. Many recent reports have identified MTX-related lymphoproliferative disorder (MTX-LPD) as lymphoma that develops during MTX therapy. However, spinal lesions, which are extremely rare, can be misdiagnosed as spinal metastases or pyogenic spondylitis. Here, we describe...
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Introduction Follicular lymphoma (FL) is an indolent, yet heterogeneous, B-cell lymphoproliferative disorder. Although most FL patients respond well to treatment, few with specific traits have a poor prognosis; the latter are difficult to define. Patients and methods We retrospectively analyzed data from 143 FL patients treated at the University o...
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BACKGROUND Lymphomatoid granulomatosis (LyG) is a rare lymphoproliferative disorder associated with Epstein-Barr virus (EBV) in which there is an infection of B cells and numerous reactive T cells. The lymphoproliferative disorder progresses to organ infiltration and resultant dysfunction of affected organs. Histologically, it is characterized by a...
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Chronic active Epstein-Barr virus (CAEBV) infection is usually a fatal disease associated with clonal proliferation of EBV-infected T or NK cells. We present the case of a 33-year-old Peruvian patient who developed a multisystem CAEBV, notably responsible for exceptional ophthalmological and renal damage. We describe the clinicopathological feature...
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Shashank Cingam,1 Surbhi Sidana2 1Division of Hematology and Oncology, University of New Mexico Comprehensive Cancer Center, Albuquerque, NM, 87102, USA; 2Division of BMT and Cell Therapy, Stanford University School of Medicine, Stanford, CA, 94305, USACorrespondence: Surbhi Sidana, Division of Blood and Marrow Transplant and Cellular Therapy, Stan...
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Epstein–Barr virus (EBV)⁺ diffuse large B-cell lymphoma (DLBCL) has specific tumour cell characteristics, and these patients have worse outcomes than EBV-negative DLBCL patients. We compared 38 EBV⁺ DLBCL patients with 43 methotrexate-associated EBV⁺ B-cell lymphoproliferative disorders (MTX⁺/EBV⁺ BLPDs) and 30 non-germinal centre (GC) subtype DLBC...
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Introduction and importance The management of large malignant tracheo-esophageal fistulas (TEF) is not standardized. Herein, we report a case with a malignant TEF associated with esophageal post-transplant lymphoproliferative disorder (PTLD) for whom we successfully performed a surgical repair. This contributes to the knowledge on how to treat larg...
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Rationale: Chronic active Epstein-Barr virus (EBV) infection (CAEBV) is a rare but life-threatening EBV-positive lymphoproliferative disorder. Currently, treatment options for CAEBV are limited. Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the only way to cure CAEBV. Here, we report a rare case of CAEBV manifesting as massive...
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Introduction . Lymphomas are a heterogenic group of tumors of the lymphatic and hematopoietic systems, and in many cases tumor process develops in the area of the head and neck including skin and mucosa, orbit, nasal cavity and paranasal sinuses, oral cavity, oropharynx, salivary glands, thyroid, and cervical lymph nodes. Differential diagnosis of...
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Pityriasis lichenoides et varioliformis acuta (PLEVA) and lymphomatoid papulosis (LyP) are uncommon inflammatory skin disorders that occasionally share clinicopathological features. Differentiating between the two entities remains problematic, and a definitive diagnosis usually requires multi-step investigations, which is an enormous challenge to p...
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Indolent NK-cell lymphoproliferative disorder of the gastrointestinal tract is a new provisional entity listed in the structure of the forthcoming fifth edition of the World Health Organization (WHO) Classification of Hematolymphoid Tumors. It was first named as “NK-cell enteropathy” and “Lymphomatoid gastropathy” by two independent series a decade...
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Pneumocystis jirovecii is an opportunistic fungus that is classically associated with pneumonia in immunocompromised patients, particularly those with human immunodeficiency virus and acquired immunodeficiency syndrome (HIV/AIDS). However, this infection is now more commonly seen in those with malignancy, particularly lymphoproliferative disorders....
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Methotrexate-associated lymphoproliferative disorder (MTX-LPD) occurs in patients with rheumatoid arthritis (RA) treated with methotrexate (MTX). MTX-LPD is typically associated with Epstein-Barr virus (EBV) infection and regresses with MTX discontinuation. On the other hand, EBV-negative MTX-LPDs are less common and are more likely to show partial...
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Background: Erythema induratum of Bazin is a rare manifestation of cutaneous tuberculosis. It may mimic some systemic disorders like sarcoidosis, poly arteritis nodosa, lymphoproliferative disorder, Histoplasmosis, and lupus profundus. Case Presentation: A 38-year young female, diabetic, and hypertensive presented with multiple painful skin lesion...
Presentation
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Post Transplant Lymphoproliferative disorder
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The current gold standard to prevent allograft rejection for maintenance immunosuppression in kidney transplantation currently consists in glucocorticoids, an antiproliferative agent and a calcineurin inhibitor (CNI), with better outcome for tacrolimus than cyclosporin. Although, CNI drastically improved early graft survival, so far, CNI have faile...
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Ferroptosis is a type of regulated cell death catalyzed by the iron-dependent accumulation of lipid hydroperoxides to lethal levels. Chronic lymphocytic leukemia (CLL) is a chronic lymphoproliferative disorder. However, the understanding of ferroptosis in CLL remains largely poor. In this study, we investigated the stratification and prognostic rol...
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Post-transplant lymphoproliferative disease (PTLD) is a well-recognized complication after transplant. This study aimed to develop and validate a risk score to predict PTLD among solid organ transplant (SOT) recipients. Poisson regression identified predictors of PTLD with the best fitting model selected for the risk score. The derivation cohort co...
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It takes only 1 profound experience to change someone’s life. We invite you to read about how an experience of struggle led to a motivation in medicine. Through a first-person perspective, the author details the sequence of diagnosis and treatment of his own case of cardiac transplantation secondary to viral cardiomyopathy.
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Human T-cell leukemia virus type 1 (HTLV-1) is the causative agent of adult T-cell leukemia/lymphoma (ATL) and HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP). The effects of HTLV-1 on health are not fully elucidated. Epidemiological studies have shown that the prevalence of HTLV-1 infection is high in patients with rheumatic di...
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Mutations in the X-linked gene MAGT1 cause a Congenital Disorder of Glycosylation (CDG), with two distinct clinical phenotypes: a primary immunodeficiency (XMEN disorder) versus intellectual and developmental disability. It was previously established that MAGT1 deficiency abolishes steady-state expression of the immune response protein NKG2D (encod...
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Review: Interleukin-18 (IL-18) is a proinflammatory cytokine that promotes various innate immune processes related to infection, inflammation, and autoimmunity. Patients with systemic juvenile idiopathic arthritis and adult-onset Still’s disease exhibit chronic excess of serum IL-18, which is associated with a high incidence of macrophage activatio...
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Epstein-Barr virus (EBV) establishes a lifelong latent infection in healthy humans, kept under immune control by cytotoxic T cells (CTLs). Following paediatric haematopoetic stem cell transplantation (HSCT), a loss of immune surveillance leads to opportunistic outgrowth of EBV-infected cells, resulting in EBV reactivation, which can ultimately prog...
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Autoimmune lymphoproliferative syndrome (ALPS) is a chronic non-malignant lymphoproliferative disorder caused by mutations in the genes involved in programmed cell death. It is inherited as an autosomal dominant pattern with variable penetrance. In this paper we present the first report of a Macedonian family with ALPS, caused by a novel heterozygo...
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Primary cutaneous lymphomas are lymphoproliferative disorders having multiple heterogenous subtypes with a primary cutaneous manifestation in the absence of systemic involvement of the lymph nodes, bone marrow, or visceral organs at the time of diagnosis. Herein, we report the case of a 75-year-old male who presented with a single plaque on the bac...