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Клиническое значение тестов FLC у пациентов без других признаков гематологического расстройства.

Клиническое значение испытания на аномальную свободную цепочку освещения (ФЛК), проводимого в связи с неконкретными жалобами в отсутствие известной дискразии плазмы (ПХД) или лимфопролиферативной болезни (ЛДП), не в полной мере прояснено. Мы изучили значение аномального соотношения FLC (FLC-R) в этой ситуации. Пациенты, зарегистрированные в базе данных медицинского обслуживания " Маккаби " 2007-2023 Был проведен обзор без учета ранее зарегистрированных ПХД/БЗП или повышения общего уровня белка (ОП). Были зарегистрированы демографические данные, случаи сопереживания и лабораторные тес

Temporal trends in hematopoietic stem cell transplantation in Argentina between 2009 and 2018: A collaborative study by GATMO-TC and INCUCAI.

IntroductionHematopoietic stem cell transplantation is the only curative treatment for many disorders and international data shows a growing trend.MethodWe aimed to evaluate the temporal trends in HSCT transplant rates in Argentina. A time-series analysis was performed for the period 2009 to 2018 using the national database from the National Central Coordinating Institute for Ablations and Implants. Crude and standardized transplant rates were calculated. A permutation joinpoint regression model analysis was used to identify significant changes over time.ResultsAltogether, 8,474 transplants we

Combined Selection System to Lower the Cutoff for Plasma Cell Enrichment Applied to iFISH Analysis in Multiple Myeloma.

Multiple myeloma (MM) is a very heterogeneous disease, characterized by multiple cytogenetic aberrations on plasma cells (PC) that have been traditionally used to predict the outcome of the disease. A mayor issue on the analysis of PC is the sometimes low infiltration of these cells in the bone marrow that hampers cytogenetic studies. To solve this problem we have optimized a selection strategy based on PC immunomagnetic isolation that has allowed us to lower to 1% the minimal PC infiltration requirement without loss of purity, enabling to perform genetic analysis. In this study, we have analy

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… In lymphoproliferative disorders of bone marrow, it represents 42% of B-cell tumors and affects mainly older patients in their sixth or seventh decades of life, where, according to the IMF, death rates have been falling on average 0.8% yearly. …

HBV Reactivation During the Treatment of Non-Hodgkin Lymphoma and Management Strategies.

Hepatitis B virus reactivation (HBV-R), which can lead to HBV-related morbidity and mortality, is a common and well-known complication that occurs during the treatment of non-Hodgkin lymphoma (NHL) patients with current or past exposure to HBV infection. HBV-R is thought to be closely associated with chemotherapeutic or immunosuppressive therapies. However, immunosuppressive agents such as anti-CD20 antibodies (e.g., rituximab and ofatumumab), glucocorticoids, and hematopoietic stem cell transplantation (HSCT) administered to NHL patients during treatment can cause deep immunodepression and pl

Факторы, связанные с результатами аутологической трансплантации стволовых клеток при множественном склерозе.

: Автономная трансплантация стволовых клеток (AHSCT) вылилась в высокоэффективное лечение повторно излучаемого многократного склероза (RRMS), хотя отбор пациентов по-прежнему сопряжен с трудностями. Неясно, в какой степени осложнения, связанные с модификацией заболеваний (ДМР), и осложнения, связанные с процедурой, влияют на результаты лечения. Цель этого исследования заключалась в изучении факторов, которые могут повлиять на результаты АХСТТ.МЕТОДЫ: Были проанализированы данные многоцентрального ретроспективного исследования Haematopoietic Stem Transplanation для лечения множественных склероз

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… While EBV reactivation has previously been linked to complications following AHSCT for MS, including EBV-related lymphoproliferative disorders, 9 34 a direct relationship with disease-related outcomes has not been demonstrated. …

Insights into Facilitated Subcutaneous Immunoglobulin Use in Patients with Secondary Immunodeficiency Diseases: A FIGARO Subgroup Analysis.

The Facilitated Immunoglobulin Administration Registry And Outcomes (FIGARO) Study was a European, multicenter, prospective, observational study conducted across Europe designed to provide insights on the clinical use and tolerability of facilitated subcutaneous immunoglobulin (fSCIG). Data herein are reported for the cohort of patients with secondary immunodeficiency (SID), with a subgroup analysis by age. The SID cohort included 31 patients: 1 pediatric, 15 adult, and 15 older adult patients. Over the 36-month observation period, the median monthly dose of fSCIG (30 g) and median monthly inf

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… Subcutaneous immunoglobulin in lymphoproliferative disorders and rituximab-related secondary hypogammaglobulinemia: A single-center experience in 61 patients Haematologica 2014 99 1101 1106 10.3324/haematol.2013.101261 24682509 PMC4040915 32. Li Z. Follman K. Freshwater E. Engler F. Yel L. …

Brazilian consensus recommendations on the diagnosis and treatment of light chain amyloidosis.

INTRODUCTION: Systemic immunoglobulin light chain amyloidosis is characterized by the extracellular deposition of misfolded light chains, leading to progressive dysfunction of multiple organs. Diagnosis remains a significant clinical challenge and is frequently delayed due to the presence of nonspecific symptoms, coupled with limited access to specialized diagnostic tools and expertise. Currently, no established national recommendations for the diagnosis and management of this condition exist in Brazil. METHODS: These expert recommendations were developed following a structured advisory meeti

Прогрессивная многофукальная лейкоэнцефалопатия как осложнение лечения множественным склерозом, которое может привести к изменению болезней

Обзор дает современное представление о патогенезе прогрессивной многофукальной лейкоэнцефалопатии (ПМЛ), которая представляет собой серьезную и потенциально фатальную форму расстройства белых позвоночников головного мозга. Анализируется информация о частотности его развития у пациентов, которые проходят курс лечения множественным склерозом. В этой категории пациентов с множественным склерозом подробно описаны алгоритмы оптимизации рисков PML. Обобщаются данные о наиболее важных биомаркерах PML, поиск которых в настоящее время ведется во многих центрах по всему миру. Первый случай PML в России

Calculated globulin (CG) as a screening test for antibody deficiency.

Calculated globulin (total protein - albumin) is usually tested as part of a liver function test profile in both primary and secondary care and determines the serum globulin concentration, of which immunoglobulins are a major component. The main use hitherto of calculated globulin is to detect paraproteins when the level is high. This study investigated the potential to use low levels of calculated globulin to detect antibody deficiency. Serum samples with calculated globulin cut-off < 18 g/l based on results of a pilot study were collected from nine hospitals in Wales over a 12-month period.

HIV-Associated Lymphomas: Updates from Pathogenesis to Treatment Strategies.

HIV-associated lymphoma (HAL) is an aggressive malignancy directly linked to HIV infection and accounts for more than 30% of cancer-related deaths in people living with HIV (PLWH). HAL subtypes, including diffuse large B-cell lymphoma (DLBCL), Burkitt lymphoma (BL), primary effusion lymphoma (PEL), and plasmablastic lymphoma (PBL), exhibit five to ten times higher incidence rates and distinct molecular profiles compared to HIV-negative lymphomas. Pathogenesis involves HIV-driven CD4+ T-cell depletion, chronic B-cell activation, and oncogenic viral coinfection. First-line therapy combines antir

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… The latest WHO classification (5 th edition) refines the categorization of immunodeficiency-associated lymphoproliferative disorders (IDD-LPDs), encompassing subtypes such as post-transplant lymphoproliferative disorders (PTLD), HIV-associated lymphomas (HAL), primary immune deficiency-related LPDs, …

Rare central nervous system lymphomas.

Central nervous system (CNS) lymphomas are rare malignancies characterised by lymphoid infiltration into the brain, spinal cord, cranial nerves, meninges and/or eyes in the presence or absence of previous or concurrent systemic disease. Most CNS lymphomas are of the diffuse large B-cell lymphoma (DLBCL) subtype for which treatment strategies, particularly the use of high-dose methotrexate-based protocols and consolidation with autologous stem cell transplantation, are well established. Other histopathological subtypes of CNS lymphoma are comparatively less common with published data on these r

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… disorder, resulting in the production of a monoclonal immunoglobulin M (IgM) paraprotein by lymphoplasmacytoid cells that infiltrate the bone marrow. 87 Although neurological signs and symptoms may occur in the context of LPL, they are not always the direct result of neoplastic infiltration into the … MYD88 L265P in Waldenström macroglobulinemia, immunoglobulin M monoclonal gammopathy, and other B‐cell lymphoproliferative disorders using conventional and quantitative allele‐specific polymerase chain reaction . …

Management of cutaneous T cell lymphoma: new and emerging targets and treatment options.

Cutaneous T cell lymphomas (CTCL) clinically and biologically represent a heterogeneous group of non-Hodgkin lymphomas, with mycosis fungoides and Sézary syndrome being the most common subtypes. Over the last decade, new immunological and molecular pathways have been identified that not only influence CTCL phenotype and growth, but also provide targets for therapies and prognostication. This review will focus on recent advances in the development of therapeutic agents, including bortezomib, the histone deacetylase inhibitors (vorinostat and romidepsin), and pralatrexate in CTCL.

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… disorders, subcutaneous panniculitis-like T cell lymphoma, and the group of primary cutaneous peripheral T cell lymphomas that includes the provisional entities of cutaneous aggressive epidermotropic CD8 + T cell lymphoma, cutaneous γ/δ T cell lymphoma, and cutaneous CD4 + small/medium-sized pleomorphic … WHO-EORTC | Frequency (%) | 5-year survival (%) Cutaneous T cell and natural killer cell lymphoma Indolent | | Mycosis fungoides | 44 | 88 Follicular mycosis fungoides | 4 | 80 • Pagetoid reticulosis | <1 | 100 • Granulomatous slack skin | <1 | 100 CD30 + lymphoproliferative disorders | | • Anaplastic …

Development and implementation of the International AIDA Network Castleman's disease registry.

Castleman's disease (CD) consists of a wide spectrum of rare disorders classified into unicentric CD and multicentric CD (MCD), based on the diffusion of disease distribution and the severity of clinical manifestations. While unicentric CD is characterized by a single lymph node involvement, MCD is defined by multiple lymph node station involvement with more prominent systemic symptoms. MCD is further subdivided into HHV-8 associated MCD, polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS)-associated MCD, and idiopathic MCD (iMCD), which is also

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… These include the rarity of the disease, overlapping clinical features with mimicker diseases such as autoimmune and lymphoproliferative disorders, and the need for histological confirmation, which may not always be uniformly available across centers. …

Epidemiology, genetics and treatment of multiple myeloma and precursor diseases.

Multiple myeloma (MM) is a hematological malignancy caused by the clonal expansion of plasma cells. The incidence of MM worldwide is increasing with greater than 140 000 people being diagnosed with MM per year. Whereas 5-year survival after a diagnosis of MM has improved from 28% in 1975 to 56% in 2012, the disease remains essentially incurable. In this review, we summarize our current understanding of MM including its epidemiology, genetics and biology. We will also provide an overview of MM management that has led to improvements in survival, including recent changes to diagnosis and therapi

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… Risk of plasma cell and lymphoproliferative disorders among 14621 first‐degree relatives of 4458 patients with monoclonal gammopathy of undetermined significance in Sweden . …

Post-pandemic recommendations for the management of COVID-19 in patients with haematological malignancies or undergoing cellular therapy, from the European Conference on Infections in Leukaemia (ECIL-10).

In the post-pandemic years, SARS-CoV-2 morbidity and mortality declined due to less pathogenic variants, active and passive immunization, and antiviral therapies. However, patients with hematological malignancies and/or undergoing hematopoietic cell transplantation (HCT) remain at increased risk for poor outcomes. Therefore, adherence to contact and droplet precautions is essential to avoid transmission, especially during epidemic waves. Detection of viral RNA by nucleic acid testing of naso-oro-pharyngeal samples is the gold standard for diagnosis due to its high sensitivity and specificity.

Treatment-free survival to bortezomib combined with dexamethasone in patients with refractory/relapsed pure red cell aplasia: a prospective clinical study.

BackgroundImmunosuppressive treatments induce and maintain remissions in a majority of patients with acquired pure red cell aplasia (aPRCA); however, salvage therapy for non-responders remains unsatisfactory.MethodsIn this prospective, open-label, clinical trial (NCT04423367), patients with refractory/relapsed aPRCA were recruited and received subcutaneous bortezomib at 1.3 mg/m2 in combination with dexamethasone 40 mg weekly(BD regimen) for 12 weeks. The primary endpoint was the overall response rate at 12 weeks.ResultsIn total, 18 patients were enrolled, including 10 (55.6%) with primary aPR

A retrospective review of the management of patients following a malignancy diagnosis on biologic therapies for the treatment of dermatological disorders.

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Long-Term Safety of Rapid Daratumumab Infusions in Multiple Myeloma Patients.

Multiple myeloma survival has significantly improved in recent years, due to novel agents that are available for treatment. The anti-CD38 monoclonal antibody Daratumumab is particularly efficient for patients with relapse/refractory disease, and many studies have shown its unprecedented efficacy also as a first treatment. However, to avoid the incidence of infusion reactions, long infusion schedules of 8 h at first dose and 4 h in the following doses are required, which can reduce the compliance of patients and health care professionals. A reduced infusion time of 90 min has been reported prev

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… Rapid infusions have been tested with other monoclonal antibodies in the past; for example, rapid 60 min infusions of rituximab (IgG1 chimeric anti CD20 antibody) for B cell lymphoproliferative disorders have been adopted at our institution and many others since 2006 ( 22 , 23 ). …

International Recommendations for Screening and Preventative Practices for Long-Term Survivors of Transplantation and Cellular Therapy: A 2023 Update.

As hematopoietic cell transplantation (HCT) and cellular therapy expand to new indications and international access improves, the number of HCTs performed annually continues to rise. Parallel improvements in HCT techniques and supportive care entails more patients surviving long term, creating further emphasis on survivorship needs. Survivors are at risk for developing late complications secondary to pretransplantation, peritransplantation, and post-transplantation exposures and other underlying risk factors. Guidelines for screening and preventive practices for HCT survivors were originally p

Autologous hematopoietic stem cell transplant at a tertiary care centre in India: achieving comparable outcomes with adaptations.

Autologous stem cell transplantation (ASCT) is the standard treatment for many high-risk solid tumors. Patients undergoing ASCT should be managed in a dedicated hematopoietic stem cell transplantation (HSCT) unit with isolation rooms, high-efficiency particulate air (HEPA) filters, and positive pressure. We report the outcomes of the first 20 pediatric patients who underwent ASCT in isolation rooms with no HEPA filters or positive pressure. Moreover, the isolation rooms were not part of a dedicated HSCT unit. Data from 20 patients were analyzed. All patients included in the study underwent ASC