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Diagnostic and disease severity determination criteria for hydroa vacciniforme lymphoproliferative disorders and severe mosquito bite allergy.

Hydroa vacciniforme lymphoproliferative disorder (HV-LPD) and severe mosquito bite allergy (SMBA) are both cutaneous forms of Epstein-Barr virus (EBV)-associated T/natural killer (NK) cell LPDs and are closely related to chronic active EBV disease (CAEBV) and EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH). HV-LPD is further divided into classic HV, a benign subtype mediated by EBV-positive γδT cells, and systemic HV, another life-threatening subtype mainly associated with EBV-positive αβT or γδT cells. The vast majority of patients with SMBA have increased numbers of EBV-infected

Case Report: Non-negligible Epstein-Barr virus-associated posttransplant lymphoproliferative disorders in a lung transplant recipient.

BACKGROUND: Posttransplant lymphoproliferative disorders (PTLDs) are uncommon but serious complications in patients following solid organ transplantation. Primary Epstein-Barr virus (EBV) infection is a risk factor for the development of PTLD, especially early-onset PTLD, in EBV-negative recipients. To date, however, there are no specific guidelines on the threshold of EBV-DNA load for therapeutic intervention, the source for measurement (e.g., blood, bronchoalveolar fluid), or the use of antiviral agents as prophylaxis for early PTLD prevention in EBV-mismatched patients. METHODS: The presen…

Unrelated cord blood transplantation for adult-onset EBV-associated T-cell and NK-cell lymphoproliferative disorders.

Adult-onset EBV-associated T-cell and NK-cell lymphoproliferative disorders (EBV-T/NK-LPDs) often progress rapidly, and require allogeneic stem cell transplantation early in the course of treatment. Unrelated cord blood transplantation (UCBT) is a readily available option for patients without HLA-matched donors. We retrospectively analyzed the outcomes of 12 UCBT in adult patients with chronic active EBV infection (CAEBV, n = 8), EBV-positive hemophagocytic lymphohistiocytosis following primary EBV infection (n = 2), hydroa vacciniforme-like lymphoproliferative disorder (n = 1), and systemic E…

The challenge of early diagnosis of autoimmune lymphoproliferative syndrome in children with suspected autoinflammatory/autoimmune disorders.

OBJECTIVES: To test the usefulness of an extended panel of lymphocyte subsets in combination with Oliveira's diagnostic criteria for the identification of autoimmune lymphoproliferative syndrome (ALPS) in children referred to a paediatric rheumatology centre. METHODS: Patients referred from 2015 to 2018 to our rheumatology unit for an autoimmune or autoinflammatory condition were retrospectively analysed. Oliveira's required criteria [chronic lymphoproliferation and elevated double-negative T (DNT)] were applied as first screening. Flow cytometry study included double-negative CD4-CD8-TCRαβ+

Peripheral T-cell lymphoma with gastrointestinal involvement and indolent T-lymphoproliferative disorders of the gastrointestinal tract.

The 2017 WHO classification includes a new provisional entity of indolent T-lymphoproliferative disorders of the gastrointestinal tract (ITLPD-GIT). We investigated GI involvement of peripheral T-cell lymphoma (PTCL). Eighty-two patients were diagnosed with PTCL during 2007-2017. Eleven patients (13 %) had histologically-confirmed GI tract involvement {3 monomorphic epitheliotropic intestinal lymphoma (MEITL), 3 extranodal NK-/T-cell lymphoma nasal type (ENKL), 2 PTCL, not otherwise specified, 1 adult T-cell leukemia-lymphoma, 2 ITLPD-GIT}. Three patients each had lesions in the small intestin…

Prevalence and spectrum of T-cell lymphoproliferative disorders in patients with Hypereosinophilia: A reference laboratory experience.

Hypereosinophilia (HE) is defined as persistently elevated absolute eosinophil count (AEC) ≥ 1.5 × 109/L, which can be due to a variety of underlying causes. In this study, we investigated the prevalence and spectrum of T-cell lymphoproliferative disorders in 124 consecutive patients with HE by flow cytometric immunophenotyping. Available medical records, pathology reports and T-cell receptor (TCR) gene rearrangement were reviewed. Fifteen patients (12%) with HE had abnormal T-cell populations that were initially detected by flow cytometry. The presence of immunophenotypically abnormal T cells…

Epstein-Barr Virus (EBV)-Related Lymphoproliferative Disorders in Ataxia Telangiectasia: Does ATM Regulate EBV Life Cycle?

Epstein-Barr virus (EBV) is an ubiquitous herpesvirus with a tropism for epithelial cells (where lytic replication occurs) and B-cells (where latency is maintained). EBV persists throughout life and chronic infection is asymptomatic in most individuals. However, immunocompromised patients may be unable to control EBV infection and are at increased risk of EBV-related malignancies, such as diffuse large B-cell lymphomas or Hodgkin's lymphomas. Ataxia telangiectasia (AT) is a primary immunodeficiency caused by mutations in the ATM gene and associated with an increased incidence of cancers, parti

Local Disease Control in Ocular Adnexal Lymphoproliferative Disorders: Comparative Outcomes of MALT Versus Non-MALT Histologies.

INTRODUCTION: Ocular adnexal lymphoproliferative disorders (OALDs) are almost exclusively of B-cell origin, with the majority being extra-nodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT). The comparative efficacy of involved field radiation therapy (IFRT) in MALT vs. non-MALT OALDs is not known. MATERIALS AND METHODS: We present a single-center, large cohort, retrospective study of the efficacy of IFRT in OALDs. Failure-free survival (FFS), complete remission, and local, regional, and distant failure were determined for 112 patients with MALT OALDs (n = 71) and non-MA…

Intralymphatic cutaneous anaplastic large cell lymphoma/lymphomatoid papulosis: expanding the spectrum of CD30-positive lymphoproliferative disorders.

Intravascular large B-cell lymphomas and EBV NK/T-cell lymphomas commonly follow an aggressive clinical course. We recently reported an enti … s. All 12 intravascular large T-cell lesions were intralymphatic; the majority (9) were CD30 T-cell lymphoproliferative disorders (TLPDs), 5 further classified as intravascular ALK ALCL. One ALK ALCL and 2 benign microscopic intravascular T-cell proliferations were also intralymphatic. A single case of otherwise typical cutaneous follicle center lymphoma contained intralymphat …

Molecular lesions in B-cell lymphoproliferative disorders: recent contributions from studies utilizing high-throughput sequencing techniques.

Next-generation sequencing techniques are powerful high-throughput methods that have enabled the comprehensive documentation of genetic lesions in numerous hematological malignancies. In recent times, the genomes of multiple different B-cell lymphoproliferative disorders including chronic lymphocytic leukemia, diffuse large B-cell lymphoma, Burkitt lymphoma, splenic marginal zone lymphoma, mantle cell lymphoma, hairy cell leukemia and Waldenström macroglobulinemia have been documented. Between them, these studies have reinforced and provided insight into the mechanisms for the dysregulation of…

Корреляции гематологических параметров с обнаружением костного мозга в хронических лимфопролиферативных расстройствах, связанных с гепатитовыми вирусами.

Гепатит В и вирусы С часто ассоциируются с гематологическими расстройствами в эволюции, что говорит о том, что эти вирусы имеют тропизм для периферийных клеток крови и/или костного мозга. AIM: Анализ гематологических параметров и результатов исследования костного мозга у группы пациентов с хроническими лимфопролиферативными расстройствами (CLD) и гепатитовыми вирусами B, C, D, которые были включены в грант на исследования (акроним LIMFO-VIR) в период с декабря 2007 года по май 2010 года в отделении гематологии госпиталя Университета неотложной помощи Бухареста. МЕТОДЫ И РЕЗУЛЬТАТЫ: У пациентов

Совпадение в аннотации оригинала
BACKGROUND: Hepatitis B and C viruses' infections are often associated with hematological disorders in evolution, suggesting that these viruses have a tropism for peripheral blood and/or bone marrow cells. AIM: To analyze the hematological parameters and bone marrow findings in a group of patients diagnosed with chronic lymphoproliferative disorders (CLD) a…

Lymphoproliferative disorders of the kidney on fine-needle aspiration: cytomorphology and radiographic correlates in 33 cases.

BACKGROUND: Unless renal lesions present in the setting of widespread lymphoma, biopsy can be indicated to differentiate from metastases, hypovascular renal cell carcinoma, urothelial carcinoma or infection. We review our experience with lymphoproliferative disorders in the kidney diagnosed by fine-needle aspiration (FNA), and focus on clinicopathologic and radiographic features. DESIGN: All cases of non-Hodgkin lymphoma diagnosed on renal FNA at 2 academic institutions between 1989 and 2011 were reviewed. Clinical history, radiographic and cytomorphologic features, and follow-up were assesse…

Cytotoxic T lymphocytes for the treatment of viral infections and posttransplant lymphoproliferative disorders in transplant recipients.

PURPOSE OF REVIEW: The continuous and successful expansion of organ transplants is unfortunately associated with increased incidence of severe opportunistic viral infections and Epstein-Barr virus (EBV)-related lymphomas secondary to immunosuppression. Here, we review the strengths and limitations of T-cell-based strategies used to treat viral infections in immunocompromised individuals. RECENT FINDINGS: While current antiviral drugs are often suboptimal because of associated toxicities, a promising approach in the management of infections with viruses like cytomegalovirus (CMV), adenovirus (

Gamma heavy-chain disease: defining the spectrum of associated lymphoproliferative disorders through analysis of 13 cases.

Gamma heavy-chain disease (gHCD) is defined as a lymphoplasmacytic neoplasm that produces an abnormally truncated immunoglobulin gamma heavy-chain protein that lacks associated light chains. There is scant information in the literature regarding the morphologic findings in this rare disorder, but cases have often been reported to resemble lymphoplasmacytic lymphoma (LPL). To clarify the spectrum of lymphoproliferative disorders that may be associated with gHCD, this study reports the clinical, morphologic, and phenotypic findings in 13 cases of gHCD involving lymph nodes (n=7), spleen (n=2), b…

Clinicopathologic spectrum and EBV status of post-transplant lymphoproliferative disorders after allogeneic hematopoietic stem cell transplantation.

Post-transplant lymphoproliferative disorders (PTLDs) are serious, life-threatening complications of solid-organ transplantation (SOT) and bone marrow transplantation, and are associated with high mortality. PTLDs represent a heterogeneous group of lymphoproliferative diseases, which show a spectrum of clinical, morphologic, and molecular genetic features ranging from reactive polyclonal lesions to frank lymphomas. We describe clinicopathologic features of 17 cases of PTLD after allogeneic hematopoietic stem cell transplantation (allo-HSCT), which were analyzed by in situ hybridization for EBV…

Myasthenia gravis, an autoimmune manifestation of lymphoma and lymphoproliferative disorders: case reports and review of literature.

Myasthenia gravis (MG) is an autoimmune disease mediated by antibodies to acetylcholine receptors (AChRs) or muscle specific tyrosine kinase (MuSK). While the frequent association of MG with thymoma in patients aged 40-60 years is well recognized, its occurrence in patients with lymphoma has not been well studied. We review the literature on the association of MG and lymphoid malignancies and report two new patients. MG can occur in a synchronous or non-synchronous fashion with lymphoma. The pathogenesis of MG in lymphoid malignancies is probably heterogeneous and likely relates to perturbatio

Folliculocentric B-cell-rich follicular dendritic cells sarcoma: a hitherto unreported morphological variant mimicking lymphoproliferative disorders.

We report three cases of follicular dendritic cell sarcoma (FDCS) showing a hitherto undescribed histological pattern consisting of nodular tumor growth associated with small B lymphocytes. FDCS tumor cells consistently showed large epithelioid features and were intermingled with small lymphocytes in the nodules in two cases, whereas they formed cohesive aggregates surrounded by lymphocyte mantle in the other. These features were easily confused with lymphomatous proliferations and, in particular, subtypes of Hodgkin lymphoma, high-grade follicular lymphoma, and germinotropic large B-cell lymp

Epstein-Barr virus-related atypical lymphoproliferative disorders in Waldeyer's ring: a clinicopathological study of 9 cases.

BACKGROUND AND STUDY AIM: Because of the small biopsy specimens in the Waldeyer's ring (WR) the differential diagnosis between Epstein-Barr virus (EBV)-associated lymphoproliferative disorder (LPD) and malignant lymphoma is occasionally difficult. We report here clinicopathological, immunohistochemical and genotypic findings of 9 cases of EBV-associated LPDs in WR. PATIENTS AND METHODS: Using formalin-fixed paraffin-embedded sections, histological analyses, immunohistochemistry, in situ hybridization and polymerase chain reaction were performed. RESULTS: Clinically, all 9 cases showed more t

Ofatumumab, a second-generation anti-CD20 monoclonal antibody, for the treatment of lymphoproliferative and autoimmune disorders.

BACKGROUND: Lymphoproliferative and autoimmune disorders share monoclonal dysregulation and survival advantage of B-lymphocytes. Thus, therapies directed towards eliminating B-cells will play an important role as CD20 is exclusively expressed in B-lymphocytes and its modulation by monoclonal antibodies such as rituximab has improved outcomes in lymphoproliferative and autoimmune disorders. Ofatumumab is a new, fully human anti-CD20 antibody and has been shown to be effective and safe, but its role in these conditions is still unclear. OBJECTIVES: To describe the preclinical and clinical data

Epstein-Barr virus-associated post-transplant lymphoproliferative disorders presented as interstitial pneumonia; successful recovery with rituximab.

We describe a patient that developed Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disorders (PTLD), which presented as interstitial pneumonia. He had received allogeneic bone marrow transplantation for the treatment of acute myeloid leukemia 17 months before, when he developed hypoxemia requiring emergent admission. Chest computed tomography revealed pulmonary interstitial shadows, but neither hepatomegaly nor lymphadenopathy were detected. Bronchoscopy with lung biopsy revealed a lymphomatous proliferation of EBV-infected B cells. The interstitial pneumonia rapidly …