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Amyloidosis with Cardiac Involvement: Identification, Characterization, and Management.

Purpose of reviewAmyloidosis is a protein deposition disease whereby a variety of precursor proteins form insoluble fibrils that deposit in tissues, causing organ dysfunction and, many times, death. Accurate characterization of the disease based on the nature of the precursor protein, organ involvement, and extent of disease is paramount to guide management. Cardiac amyloidosis is critical to understand because of its impact on prognosis and new treatment options available.Recent findingsNew imaging methods have proven to be considerably valuable in the identification of cardiac amyloid infilt

The Use of Immune Checkpoint Inhibitors in Oncology and the Occurrence of AKI: Where Do We Stand?

Immune checkpoint inhibitors (ICIs) are a novel class of immunotherapy drugs that have improved the treatment of a broad spectrum of cancers as metastatic melanoma, non-small lung cancer or renal cell carcinoma. These humanized monoclonal antibodies target inhibitory receptors (e.g. CTLA-4, PD-1, LAG-3, TIM-3) and ligands (PD-L1) expressed on T lymphocytes, antigen presenting cells and tumor cells and elicit an anti-tumor response by stimulating immune system. Nevertheless, the improved overall survival is complicated by the manifestation of Immune-related Adverse Effects (irAEs). During treat

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… Lowering the dose of immunosuppressant has been considered a crucial management strategy in some cancers, such as post-transplant lymphoproliferative disorders and skin cancer. …

Quality of Online Information on Multiple Myeloma Available for Laypersons.

Online information can increase patients' competence and engagement. However, there are concerns regarding invalid information. Overall, 300 websites and 50 YouTube videos on multiple myeloma (MM) were evaluated. The websites did not differ between the search engines or search ranks. The median time since the last update was 9 months. The 63 unique websites showed a poor general quality (median JAMA score 2 of 4, only 18% with a valid HON certificate). The patient- (user-) focused quality was medium to poor (median sum DISCERN score 41 out of 80 points). The overall reading level was difficult

Contemporary Monoclonal Antibody Utilization in Glomerular Diseases.

Therapeutic monoclonal antibodies (MAbs) have been one of the fastest growing drug classes in the past 2 decades and are indicated in the treatment of cancer, autoimmune disorders, solid organ transplantation, and glomerular diseases. The Food and Drug Administration has approved 100 MAbs between 1986 and 2021, and MAbs account for 20% of Food and Drug Administration's new drug approval every year. MAbs are preferred over traditional immunosuppressive agents because of their high specificity, reduced number of drug-drug interactions, and low toxicity, which make them a prime example of persona

The Art of Bioimmunogenomics (BIGs) 5.0 in CAR-T Cell Therapy for Lymphoma Management.

PurposeLymphoma, the most predominant neoplastic disorder, is divided into Hodgkin and Non-Hodgkin Lymphoma classifications. Immunotherapeutic modalities have emerged as essential methodologies in combating lymphoid malignancies. Chimeric Antigen Receptor (CAR) T cells exhibit promising responses in chemotherapy-resistant B-cell non-Hodgkin lymphoma cases.MethodsThis comprehensive review delineates the advancement of CAR-T cell therapy as an immunotherapeutic instrument, the selection of lymphoma antigens for CAR-T cell targeting, and the conceptualization, synthesis, and deployment of CAR-T c

Biologics in Kidney Transplantation: Why and How Should We Personalize Their Dosage?

The therapeutic arsenal of immunosuppressive drugs in kidney transplantation has recently expanded with the introduction of biologics, approved or used off-label, that specifically target the immune system. Although therapeutic drug monitoring is well established for calcineurin inhibitors, its utility for biological immunosuppressants remains unclear. The objective of this narrative review was to evaluate whether biologics used to prevent or treat renal allograft rejection could be suitable candidates for therapeutic drug monitoring. This review provides (1) a comprehensive and up-to-date syn

Анализ многократной миеломы (ММ) и Моноклональной гаммопатии неопределенных знаков (MGUS)

История семьи является известным фактором риска для множественной миеломы (ММ) и ее предшественника, моноколональной гаммопатии неопределённого значения (МГУС). Предыдущие исследования в рамках всей ассоциации генома (ОСГ) выявили 35 общие loci, связанные с риском MM и 21 связанные с MGS. Цель этого исследования заключалась в выявлении менее распространенных и редких генетических loci, предвосхищающих MM/MGUS, путем проведения анализа последовательности связей на основе целых экспозиций (WES).МЕТОДЫ: Мультиточечный анализ связей был проведен с использованием многоточечного двигателя быстрого с

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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 Blood 2009 114 791 795 10.1182/blood-2008-12-191676 19182202 PMC2716021 16. Landgren O. Linet M.S. McMaster M.L. Gridley G. …

Case Report: Endothelial-targeted bridging therapy for a TTP-like phenotype in fulminant iMCD-TAFRO.

BACKGROUND: iMCD-TAFRO (the TAFRO clinical subtype of idiopathic multicentric Castleman disease (iMCD)) is characterized by thrombocytopenia, anasarca, fever, reticulin fibrosis, and organomegaly. Diagnosis is challenging due to its rarity, non-specific early presentation, and overlap with sepsis, lymphoma, and thrombotic microangiopathy (TMA). Endothelial injury is increasingly recognized as a central driver of its severe complications. CASE PRESENTATION: A previously healthy 20-year-old woman presented with rapid progression of fever, anasarca, jaundice, and respiratory failure. Laboratory

Prevalence of functional iron deficiency in patients with malignancies and its impact on fatigue-specific quality of life.

BackgroundFunctional iron deficiency (FID) as defined by the European Society for Medical Oncology (ESMO) occurs in patients with malignant neoplasms and might be a target to improve fatigue-specific quality of life (QoL). The prevalence of FID in that population, especially in patients with a haemoglobin (Hb) of 10-11 g/dl and Hb > 11 g/dl, is unclear. The aim of this study was to obtain data on the prevalence of FID in patients with solid tumours and haematological malignancies independent of Hb and age, and to investigate the potential of FID as a target to improve fatigue-specific QoL.Pati

CXCR4-directed endoradiotherapy with [177Lu]Pentixather added to total body irradiation for myeloablative conditioning in patients with relapsed/refractory acute myeloid leukemia.

Rationale: Despite recent advances in the targeted therapy of AML, the disease continues to have a poor prognosis. Allogeneic hematopoietic stem cell transplantation (alloSCT) remains to be the curative therapy option for fit patients with high-risk disease. Especially patients with relapsed or refractory (r/r) AML continue to have poor outcomes. Myeloablative total body irradiation (TBI) based conditioning can be used in AML patients refractory to multiple lines of standard therapy, but the optimal conditioning regimen remains unclear for patients considered to be chemotherapy- refractory. Fe

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… Integrating CXCR4-directed ERT into auto- or alloSCT strategies for heavily pretreated lymphoproliferative disorders, including lymphomas and multiple myeloma, has achieved remissions in all evaluated patients 33 , 38 - 40 . …

Hematopoietic progenitor cell mobilization for autologous transplantation - a literature review.

The use of high-dose chemotherapy with autologous support of hematopoietic progenitor cells is an effective strategy to treat various hematologic neoplasms, such as non-Hodgkin lymphomas and multiple myeloma. Mobilized peripheral blood progenitor cells are the main source of support for autologous transplants, and collection of an adequate number of hematopoietic progenitor cells is a critical step in the autologous transplant procedure. Traditional strategies, based on the use of growth factors with or without chemotherapy, have limitations even when remobilizations are performed. Granulocyte

Update on the role of autologous hematopoietic stem cell transplantation in multiple myeloma.

Autologous stem cell transplantation is considered the standard of care for multiple myeloma patients aged < 65 years with no relevant comorbidities. The addition of drugs acting both on bone marrow microenvironment and on neoplastic plasma cells has significantly increased the proportion of patients achieving a complete remission after induction therapy, and these results are mantained after high-dose melphalan, leading to a prolonged disease control. Studies are being carried out in order to evaluate whether short term consolidation or long-term maintenance therapy can result into disease er

Антибиотическое управление в случае предполагаемой нейтральной лихорадки (испытание ASTERIC): мультицентр, тип 1 гибридный протокол проведения экспериментальных исследований по вопросам эффективности, ступенчатый, выборочный и контролируемый.

ВВЕДЕНИЕ: Нейтропеническая лихорадка (НФ) имеет общий коэффициент смертности: 3-18%. Международные руководящие принципы рекомендуют всем пациентам, имеющим NF, получать антибиотики сверхширокого спектра (UBSA) в пределах 1 час регистрации аварийного отделения (ЭД). Тем не менее, 70% пациентов, поступающих в больницу с подозрением в наличии NF (SNF), не могут получить доступ к абсолютному числу нейтрофильов (ANC) в пределах 1 час, не имеет NF и не требует UBSA. В скорой и госпитализированных пациентах, имеющих SNF, мы предполагаем, что протокол ASTERIC эффективно и безопасно сокращает использов

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… Management of febrile neutropenia--a German prospective hospital cost analysis in lymphoproliferative disorders, non-small cell lung cancer, and primary breast cancer. Onkologie. 2011;34:241–6. doi: 10.1159/000327711. 7. Pasikhova Y, Ludlow S, Baluch A. Fever in Patients With Cancer. …

Efficacy and safety of biosimilar Peg-filgrastim after autologous stem cell transplant in myeloma and lymphoma patients: a comparative study with biosimilar Filgrastim, Lenograstim, and originator Peg-filgrastim.

Data about biosimilar Peg-filgrastim (bioPEG) in autologous stem cell transplant (ASCT) are still scarce. The aim of this study has been to assess efficacy and safety of bioPEG among lymphoma and myeloma patients undergoing ASCT, comparing these data with historical controls receiving other G-CSFs. Furthermore, an economic evaluation has been included to estimate the savings by using bioPEG. This is a prospective cohort study comparing lymphoma and myeloma patients undergoing ASCT and receiving bioPEG (n = 73) with three historical consecutive cohorts collected retrospectively who received oth

A systematic review of the cost and cost-effectiveness of immunoglobulin treatment in patients with hematological malignancies.

ObjectivesPatients with hematological malignancies are likely to develop hypogammaglobulinemia. Immunoglobulin (Ig) is commonly given to prevent infections, but its overall costs and cost-effectiveness are unknown.MethodsA systematic review was conducted following the PRISMA guidelines to assess the evidence on the costs and cost-effectiveness of Ig, administered intravenously (IVIg) or subcutaneously (SCIg), in adults with hematological malignancies.ResultsSix studies met the inclusion criteria, and only two economic evaluations were identified; one cost-utility analysis (CUA) of IVIg versus

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… Subcutaneous immunoglobulin in lymphoproliferative disorders and rituximab-related secondary hypogammaglobulinemia: a single-center experience in 61 patients. Haematologica. 2014;99(6):1101–1106. 34. Gerth WC, Betschel SD, Zbrozek AS. …

Trends in mortality from infection among patients with hematologic malignancies: differences according to hematologic malignancy subtype.

BackgroundInfection is the most important cause of non-relapse mortality in hematologic malignancy patients, leading to increased costs and prolonged hospitalization times. However, comprehensive and comparable reports on infection-specific mortality (ISM) trends in hematologic malignancy patients are lacking.ObjectivesWe aimed to provide updated ISM trends and factors associated with ISM among hematologic malignancy patients.DesignThis is a retrospective study.MethodsPatients diagnosed with the five most common hematologic malignancies from 1983 to 2016 from the Surveillance, Epidemiology, an

MicroRNAs - novel biomarkers for malignant pleural effusions.

Lung cancer is one of the most common causes of cancer death. Its poor prognosis can be attributed to the patients' advanced or metastatic presentation at the time of diagnosis. To improve and accelerate the diagnosis, better therapeutic and diagnostic methods are constantly being sought. MicroRNAs (miRNAs) are short nucleotide sequences of single-stranded, non-coding RNA that function as critical post-transcriptional regulators of gene expression. They are identified not only intracellularly, but also in physiological and pathological body fluids. These molecules are responsible for the regul

Feasibility, Tolerability and Efficacy of Carfilzomib in Combination with Lenalidomide and Dexamethasone in Relapsed Refractory Myeloma Patients: A Retrospective Real-Life Survey of the Sicilian Myeloma Network.

Background: The ASPIRE (NCT01080391) phase 3 trial showed the efficacy of carfilzomib, lenalidomide and dexamethasone (KRd) triplet for relapse and refractory multiple myeloma (RRMM). However, little is known about safety and efficacy of KRd outside a clinical trial context. Methods: Herein we report real life results of KRd given to 130 RRMM patients from 12 Sicilian Centers. Results: Median age was 62 years; patients had received a median of two previous lines of treatment (range 1-10) and 52% were refractory to previous treatment. Median number of KRd cycles was 12 (2-29), with a mean durat

Glucosylsphingosine (Lyso-Gb1) as a reliable biomarker in Gaucher disease: a narrative review.

BACKGROUND: Gaucher disease (GD) is a rare, inherited, autosomal recessive disorder caused by a deficiency of the lysosomal enzyme, acid β-glucosidase. Its diagnosis is achieved via measurements of acid β-glucosidase activity in either fresh peripheral blood leukocytes or dried blood spots, and confirmed by identifying characteristic mutations in the GBA1 gene. Currently, several biomarkers are available for disease monitoring. Chitotriosidase has been used over the last 20 years to assess the severity of GD, but lacks specificity in GD patients. Conversely, the deacylated form of glucosylcera

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… Furthermore, lyso-Gb1 has an important role in the pathogenetic mechanism of PD due to its cerebral accumulation, and in B-cell lymphoproliferative disorders, such as multiple myeloma, due to humoral immunity dysregulation by chronic antigenic stimulation. …

Immunotherapy targets in pediatric cancer.

Immunotherapy for cancer has shown increasing success and there is ample evidence to expect that progress gleaned in immune targeting of adult cancers can be translated to pediatric oncology. This manuscript reviews principles that guide selection of targets for immunotherapy of cancer, emphasizing the similarities and distinctions between oncogene-inhibition targets and immune targets. It follows with a detailed review of molecules expressed by pediatric tumors that are already under study as immune targets or are good candidates for future studies of immune targeting. Distinctions are made b

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… Rituximab is now integrated into standard therapies for B-cell lymphomas (Coiffier et al., 2002 ; Thomas et al., 2006 ; Meinhardt et al., 2010 ; Kumar et al., 2011 ), chronic lymphocytic leukemia (Woyach et al., 2011 ), post-transplant lymphoproliferative disorder (Evens et al., 2010 ), and various autoimmune … Post-transplantation lymphoproliferative disorders: diagnosis, prognosis, and current approaches to therapy . Curr. Oncol. Rep . 12 , 383 – 394 10.1007/s11912-010-0132-1 20963522 Falini B. Pulford K. Pucciarini A. Carbone A. De Wolf-Peeters C. Cordell J. Fizzotti M. Santucci A. Pelicci P. G. …