Результаты поиска

Электронные платформы отчетов о результатах лечения, предоставляемых пациентами, используемые в онкологии крови: систематический обзор.

ЦЕЛЬ: Использование электронных показателей результатов (ЭПРОМ), сообщаемых пациентами, продемонстрировало свои преимущества в онкологии; однако их использование в гематологических отклонениях по-прежнему ограничено. Мы поставили перед собой цель выявить платформы, которые используются в гематологии, и описать их особенности, приемлемость, удобство использования, осуществимость, темпы осуществления и поглощения, а также данные о результатах. МЕТОДЫ: Систематический обзор был проведен после ПРИСМА 2020 Руководящие принципы. Электронные базы данных (MedLINE, EMBASE, CINAHL, PsycINFO, Cochrane) и

Pleural effusion aetiology, presentation, treatment and outcome in haematological diseases: a review.

BACKGROUND AND AIM: Pleural effusions (PE) can complicate the course of hematologic disorders (HD) and may arise in the form of malignant PE or as a consequence of non-neoplastic complications. While a certain amount of data has been published regarding infectious and iatrogenic HD-associated PE (HPE), no comprehensive review regarding the other types of HPE has ever been conducted. To address this issue, we performed a systematic review of the literature regarding HPE, focusing on the clinical and chemical characteristics of PE, therapeutic approaches and ì outcomes at the one-year follow-up.

Expert Perspective: Hematologic Malignancies and Vasculitis.

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Personalized Management of Chronic Rhinosinusitis in the Context of Immune Deficiency.

Primary and secondary immunodeficiency as a cause of recalcitrant or difficult to treat chronic rhinosinusitis (CRS) and recurrent acute rhinosinusitis (RARS) are under-appreciated etiologies. Patients with persistent symptoms, frequent exacerbations despite appropriate medical therapy, or other sinopulmonary conditions known to be related to an immunodeficient state warrant further testing for immunodeficiency, as up to 20% of this cohort of patients may have underlying immunodeficiency. Testing for immunodeficiency includes CBC with differential, immunoglobulin levels, and pneumococcal vacci

Exploring the role of viral hepatitis in plasma cell disorders.

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… Hepatitis C virus-related lymphoproliferative disorders: an overview . World J Gastroenterol . 2007 ; 13 ( 17 ): 2467 - 2478 . 17552031 10.3748/wjg.v13.i17.2467 PMC4146766 9. Teng CJ Liu HT Liu C Y . …

Simultaneous Onset of Haematological Malignancy and COVID: An Epicovideha Survey.

Background: The outcome of patients with simultaneous diagnosis of haematological malignancies (HM) and COVID-19 is unknown and there are no specific treatment guidelines. Methods: We describe the clinical features and outcome of a cohort of 450 patients with simultaneous diagnosis of HM and COVID-19 registered in the EPICOVIDEHA registry between March 2020 to February 2022. Results: Acute leukaemia and lymphoma were the most frequent HM (35.8% and 35.1%, respectively). Overall, 343 (76.2%) patients received treatment for HM, which was delayed for longer than one month since diagnosis in 57 (1

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… disorders (CLD) (4/11, 36.4%) and Hodgkin lymphoma (HL) (7/14, 50%). … Considering lymphoproliferative disorders, a wide range of mortality rates has been observed among MM, lymphoma and CLD patients in previous EPICOVIDEHA studies [ 1 ], with the worst prognosis for MM. …

Association of Supporting Trial Evidence and Reimbursement for Off-Label Use of Cancer Drugs.

ImportanceIn many health systems, access to off-label drug use is controlled through reimbursement restrictions by health insurers, especially for expensive cancer drugs.ObjectiveTo determine whether evidence from randomized clinical trials is associated with reimbursement decisions for requested off-label use of anticancer drugs in the Swiss health system.Design, setting, and participantsThis cross-sectional study used reimbursement requests from routinely collected health records of 5809 patients with drug treatment for cancer between January 2015 and July 2018 in 3 major cancer centers, cov

Multicentre adaptive randomised trial of GvHD prophylaxis following unrelated donor stem cell transplantation comparing Thymoglobulin versus calcineurin inhibitor-based or sirolimus-based post-transplant cyclophosphamide (Methods of T cell Depletion, MoTD trial).

IntroductionGraft-versus-host disease (GvHD) remains a major complication of allogeneic stem cell transplantation (allo-SCT), affecting 30-70% of patients (representing 800 new patients per year in the UK). The risk is higher in patients undergoing unrelated allo-SCT. About 1 in 10 patients die as a result of GvHD or through complications of its treatment. In patients who survive GvHD and or the complications of treatment, about 1 in 3 patients develop chronic GvHD which has a negative impact on quality of life (QoL). Many transplant protocols use alemtuzumab or anti-thymocyte globulin (ATG) i

Bridging the Gap in Secondary Antibody Deficiencies: Current Evidence and Unmet Needs in Diagnosis and Management with Immunoglobulin Replacement.

Secondary antibody deficiency (SAD) represents a substantial yet under-recognised global healthcare burden. It is more prevalent than primary antibody deficiency, but frequently under-diagnosed and variably managed worldwide. Prompt diagnosis is often hindered by insufficient awareness among clinicians, lack of global consensus on screening/monitoring for SAD among at-risk patients, inadequate clinical immunology services and lack of standardised referral pathways/protocols. Management practices vary widely, with little international agreement, particularly regarding threshold to initiate immu

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… disorders and rituximab-related secondary hypogammaglobulinemia: a single-center experience in 61 patients[ 117 ] Lymphoproliferative disorders with secondary antibody deficiency ( N = 61, with 49 of them treated with rituximab) Yes (Sequential) SCIg, mean dose 0.075 g/kg/week (33 patients had previous … disorders and rituximab-related secondary hypogammaglobulinemia: a single-center experience in 61 patients[ 117 ] | Lymphoproliferative disorders with secondary antibody deficiency ( N = 61, with 49 of them treated with rituximab) | Yes (Sequential) | SCIg, mean dose 0.075 g/kg/week (33 patients had …

Monocentric Analysis of the Effectiveness and Financial Consequences of the Use of Lenograstim versus Filgrastim for Mobilization of Peripheral Blood Progenitor Cells in Patients with Lymphoma and Myeloma Receiving Chemotherapy and Autologous Stem Cell Transplantation.

PurposeGranulocyte-colony stimulating factors (G-CSFs) are widely used to mobilize CD34+ stem cells and to support the engraftment after hematopoietic stem cell transplantation (HSCT). A budget impact analysis and an incremental cost-effectiveness study of two G-CSFs (Lenograstim and Filgrastim biosimilar), considering engraftment, number of hospitalization days and number of G-CSF vials administered were performed.Patients and methodsBetween 2009 and 2016, 248 patients undergoing autologous HSCT have been evaluated and divided into three groups (100 Leno-Leno, 93 Leno-Fil, 55 Fil-Fil) accordi

Advanced Diagnostic Techniques

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Subcutaneous immunoglobulins replacement therapy in secondary antibody deficiencies: Real life evidence as compared to primary antibody deficiencies.

Secondary antibody deficiencies (SAD) may require immunoglobulin replacement therapy (IgRT). While the intravenous route (IVIG) is broadly considered effective in SAD, the use of subcutaneous immunoglobulins (SCIG) is mainly adopted from the experience in primary antibody deficiencies (PAD), where SCIG have been shown to perform as effective as IVIG. However, evidence-based data on SCIG administration in SAD patients are still insufficient. Herein we retrospectively evaluated the efficacy and safety profile of SCIG treatment in 131 SAD patients as compared to a group of 102 PAD patients. We fo

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… Subcutaneous immunoglobulin in lymphoproliferative disorders and rituximab-related secondary hypogammaglobulinemia: a single-center experience in 61 patients. Haematologica. 2014. June;99(6):1101–6. 10.3324/haematol.2013.101261 32. …

Primary choroidal lymphoma with extrascleral extension and bone marrow involvement: a case report.

Primary extranodal marginal zone B-cell lymphoma (EMZL) of the choroid is a rare condition with a handful of cases reported in the literature. Here, we report a challenging case of primary EMZL of the choroid with extrascleral extension and bone marrow involvement. An 86-year-old male presented with 2 years of progressive vision loss in his left eye. Fundus examination revealed yellow-white subretinal infiltrates at the posterior pole. Ultrasonography detected diffuse choroidal thickening with retrobulbar hypoechoic mass. One year later, an inferior exudative retinal detachment developed. Ultr

Bleeding Propensity in Waldenström Macroglobulinemia: Potential Causes and Evaluation.

Waldenström macroglobulinemia (WM) is a rare, incurable, low-grade, B cell lymphoma. Symptomatic disease commonly results from marrow or organ infiltration and hyperviscosity secondary to immunoglobulin M paraprotein, manifesting as anemia, bleeding and neurological symptoms among others. The causes of the bleeding phenotype in WM are complex and involve several intersecting mechanisms. Evidence of defects in platelet function is lacking in the literature, but factors impacting platelet function and coagulation pathways such as acquired von Willebrand factor syndrome, hyperviscosity, abnormal

Exploring the Rare Etiology of Severe Anemia in an Immunocompromised Patient.

Pure red cell aplasia (PRCA) is a rare cause of profound anemia, marked by very low reticulocyte count and near to complete absence of erythroid precursor cells in the bone marrow. PRCA can be congenital such as in the case of children with Diamond- Blackfan anemia or acquired, which is often triggered by exposure to certain viruses or drugs. Management depends on the underlying etiology of PRCA. Here, we present the case of a young male with underlying acquired immunodeficiency syndrome, who presented with a hemoglobin of 2.6 g/dL, initially thought to be secondary to gastrointestinal blood l

Autoimmune Diseases and Plasma Cells Dyscrasias: Pathogenetic, Molecular and Prognostic Correlations.

Multiple myeloma and monoclonal gammopathy of undetermined significance are plasma cell dyscrasias characterized by monoclonal proliferation of pathological plasma cells with uncontrolled production of immunoglobulins. Autoimmune pathologies are conditions in which T and B lymphocytes develop a tendency to activate towards self-antigens in the absence of exogenous triggers. The aim of our review is to show the possible correlations between the two pathological aspects. Molecular studies have shown how different cytokines that either cause inflammation or control the immune system play a part i

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… Patients with primary SS have an increased relative chance of having B cell lymphoproliferative disorders compared to the normal population. It has been estimated that one-third of patients with SS develop a B cell lymphoma. …

How to manage Waldenstrom's macroglobulinemia.

Waldenstrom's macroglobulinemia (WM) is very distinct from other indolent lymphoma subtypes: by definition it is accompanied by a monoclonal IgM gammopathy; it presents always with bone marrow infiltration and often with clinical symptoms such as neuropathy or hyperviscosity. These disease characteristics and the frequently advanced age of the WM patient pose a major challenge to the treating clinician even today. Recently, there has been not only substantial progress in our understanding of the biology of WM, but we have also significantly improved our tools to prognostify and to treat patien

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… Besides treatment aspects, correct diagnosis of WM might be a challenge, in particular with other chronic lymphoproliferative disorders associated with IgM paraproteinemia, such as marginal zone lymphoma. … disorders because of its excellent toxicity profile (for example, in patients with renal insufficiency) and high antilymphoma activity. …

Editorial: COVID-19 and hematological malignancies: Volume II.

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… Similarly, the retrospective study by Gung et al. evaluates mRNA-based vaccine (either Pfizer or Moderna) responses in patients with lymphoproliferative disorders or plasma cell dyscrasias. …

"Look Beyond the Skin": A case report about chronic pruritus.

A healthy 23-year-old female developed generalized pruritus over a year that began on her feet and gradually progressed to involve more than 50% of her entire body surface area. Punch skin biopsies were inconclusive, whereas a two-view chest x-ray was suspicious for lymphadenopathy. A chest computed tomography scan with contrast identified an anterior mediastinal mass which was biopsied and diagnosed as a nodular sclerosis type of Hodgkin's lymphoma. Subsequently, appropriate therapy was initiated resulting in complete resolution of the patient's chronic itch. This case underscores the clinica

Secondary antibody deficiencies in the modern era: emerging trends, diagnostic pitfalls, and advances in personalised management.

Secondary antibody deficiencies (SADs) are a significant but frequently under-recognised group of acquired immunodeficiencies. They may arise in various clinical settings, including haematological malignancies, immunosuppressive therapies, and protein-losing conditions. SADs are associated with an increased risk of recurrent and severe infections, hospitalisation, and impaired quality of life. Despite this, diagnostic and treatment pathways remain inconsistent across healthcare settings and regions. Recent advances in the use of structured clinical data, including electronic health records and

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… Building on lessons from the identification of primary immunodeficiency within B-cell lymphoproliferative disorders ( 27 , 39 , 40 ), these collaborations will help to ensure equitable, evidence-based care for all patients with SAD. … Dissecting secondary immunodeficiency: identification of primary immunodeficiency within B-cell lymphoproliferative disorders . J Clin Immunol . ( 2024 ) 45 : 32 . doi: 10.1007/s10875-024-01818-2 , PMID: 39441407 PMC11499357 26 McQuilten ZK Weinkove R Thao LTP Crispin P Degelia A Dendle C . …