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Infectious diseases in allogeneic haematopoietic stem cell transplantation: prevention and prophylaxis strategy guidelines 2016.

Infectious complications after allogeneic haematopoietic stem cell transplantation (allo-HCT) remain a clinical challenge. This is a guideline provided by the AGIHO (Infectious Diseases Working Group) of the DGHO (German Society for Hematology and Medical Oncology). A core group of experts prepared a preliminary guideline, which was discussed, reviewed, and approved by the entire working group. The guideline provides clinical recommendations for the preventive management including prophylactic treatment of viral, bacterial, parasitic, and fungal diseases. The guideline focuses on antimicrobial

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… Risk of lymphoproliferative disorders after bone marrow transplantation: a multi-institutional study. Blood. 1999;94(7):2208–2216. 192. Bertz H, et al. … Antiviral prophylaxis and the Epstein Barr virus-related post-transplant lymphoproliferative disorder. Clin Transplant. 1995;9(1):53–59. 195. Liu Q, et al. Molecular monitoring and stepwise preemptive therapy for Epstein-Barr virus viremia after allogeneic stem cell transplantation. …

Pilot Study on the Cost of Some Oncohematology Diseases in Bulgaria.

The goal of the current study is to perform a pilot study of the cost of some oncohematology diseases in Bulgaria. This is a pilot broader burden of disease research. The official report of the National health insurance fund provided information about the total expenditures paid for medicines, ambulatory services, and hospitalizations in 2015 and 2016. To evaluate the costs from a patient perspective, an internet inquiry was organized with the support of the patient organization. The inquiry contained questions regarding the patients' demography, type of oncohematology disease, year of diagnos

Novel targeted treatments in hairy cell leukemia and other hairy cell-like disorders.

In the category of mature B-cell neoplasms, splenic B-cell lymphoma and leukemia were clearly identified and include four distinct entities: hairy cell leukemia (HCL), splenic marginal zone lymphoma (SMZL), splenic diffuse red pulp lymphoma (SDRPL) and the new entity named splenic B-cell lymphoma/leukemia with prominent nucleoli (SBLPN). The BRAFV600E mutation is detected in nearly all HCL cases and offers a possibility of targeted therapy. BRAF inhibitors (BRAFi) represent effective and promising therapeutic approaches in patients with relapsed/refractory HCL. Vemurafenib and dabrafenib were

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… BRAF V600E was detected in 48 other HCL patients by Sanger sequencing and was not found in 195 patients with other B-cell chronic lymphoproliferative disorders (B-CLPD), suggesting BRAF V600E mutation could be an early and genetic driver in HCL. … Chemokine receptor expression in b-cell lymphoproliferative disorders . Leuk Lymphoma ( 2004 ) 45 : 2491–6 . doi: 10.1080/10428190410001723449 15621766 38 Sivina M Burger JA . The importance of the tissue microenvironment in hairy cell leukemia . …

Прогрессивная многофукальная лейкоэнцефалопатия в реуматоидном артрите и биологической терапии: доклад по делу и обзор литературы.

Прогрессивная многофукальная лейкоэнцефалопатия - это редкое, но потенциально смертельное заболевание, вызванное инфекцией центральной нервной системы с полиомавирусом Джона Каннингема. Прогрессивная многофукальная лейкоэнцефалопатия встречается главным образом у иммунокомпромиссированных пациентов, включая пациентов с биологическими и адресными синтетическими препаратами, такими, как множественный склероз и ревматоидный артрит. Ранняя диагностика прогрессивной многофукальной лейкоэнцефалопатии имеет решающее значение для выживания пациента. Мы описываем случай прогрессивной многофукальной лей

Concurrent Chylothorax, Chylopericardium, and Superior Vena Cava Syndrome in a Young Male With Primary Mediastinal Large B-cell Lymphoma: A Report of a Rare Case.

Primary mediastinal large B-cell lymphoma (PMBCL) is an uncommon and aggressive subtype of non-Hodgkin lymphoma (NHL) that predominantly affects young adults and typically presents with a bulky anterior mediastinal mass. We report the case of a 23-year-old male who presented with progressive dyspnea, orthopnea, and facial swelling, subsequently found to have superior vena cava (SVC) syndrome, chylothorax, and chylopericardium, an exceedingly rare triad of complications. Diagnostic evaluation required integration of advanced imaging, fluid analysis, and histopathological confirmation, ultimatel

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… Most of the available literature discusses these complications in isolation, either as secondary to thoracic duct disruption following trauma or surgery or as rare sequelae of lymphoproliferative disorders [ 10 ]. …

Prevention of CMV/EBV reactivation by double-specific T cells in patients after allogeneic stem cell transplantation: results from the randomized phase I/IIa MULTIVIR-01 study.

INTRODUCTION: Allogeneic stem cell transplantation is used to cure hematologic malignancies or deficiencies of the hematopoietic system. It is associated with severe immunodeficiency of the host early after transplant and therefore early reactivation of latent herpesviruses such as CMV and EBV within the first 100 days are frequent. Small studies and case series indicated that application of herpes virus specific T cells can control and prevent disease in this patient population. METHODS: We report the results of a randomized controlled multi centre phase I/IIa study (MULTIVIR-01) using a new

Abstracts From the 42nd Annual Scientific Meeting of the Transplantation Society of Australia and New Zealand held on June 16-18, 2024

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Assisted Peritoneal Dialysis: A Feasibility and Quality Improvement Project.

Despite often offering a better quality of life than in-center hemodialysis, peritoneal dialysis (PD) at home can be challenging for many. From May 2021 to October 2024, we offered PD assistance at 2 home dialysis clinics in Dialysis Clinic, Inc, a mid-size national dialysis provider. Eligible patients could be incident to or established on PD, with anticipated need for either permanent or temporary assistance. Over 3 years, 6 patients received assisted PD. Three established PD patients needed assistance when their care partners were unavailable. Three other patients required assistance to ini

Economic impact of immunoglobulin replacement therapy in secondary immunodeficiency to hematological cancer: a single center observational study.

This is the first report of the health economic benefits derived from preventing infections through Immunoglobulin Replacement Therapy (IgRT) in patients with secondary immunodeficiency due to hematological malignancies. We conducted a retrospective population-based cohort study using patient medical history and pharmacy data from the Hospital Clínico San Carlos for 21 patients between 2011 and 2020. The pharmacoeconomic impact of using prophylactic IgRT was assessed by comparing characteristics of the SID patients 1 year before and after initiating IgRT measured by direct medical and tangible

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… All patients had hematological malignancies such as NHL, CLL, or other lymphoproliferative disorders. At the start of IgRT, all but two patients were in complete remission, a status that was maintained throughout the following year, and none had other life-threatening conditions. …

Catch the Calcium: T-Cell Histiocyte-Rich B-Cell Lymphoma Presenting as Hypercalcemia.

T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is an extremely rare and aggressive subtype of diffuse large B-cell lymphoma (DLBCL) that typically presents in middle-aged patients and carries a poor prognosis. Hypercalcemia presenting as the initial manifestation of the disease is rare, with only one other case reported in the literature. We report a case of a 90-year-old male who presented with progressive lethargy and unintentional weight loss. Initial workup showed elevated serum calcium of 14.6 mg/dL, corrected for albumin, and creatinine of 1.51 mg/dL. He had a suppressed iPTH of

Raised Serum Levels of Syndecan-1 (CD138), in a Case of Acute Idiopathic Systemic Capillary Leak Syndrome (SCLS) (Clarkson's Disease).

BACKGROUND Systemic capillary leak syndrome (SCLS) (Clarkson's disease) is a rare disorder of unknown etiology, characterized by transient episodes of hypotension, and the microvascular leak of fluids into the peripheral tissues, resulting in edema. Between 80-90% of patients with SCLS have a concomitant monoclonal gammopathy. Although translational in vitro studies have implicated vascular endothelial barrier dysfunction in the etiology of SCLS, the etiology and disease associations in clinical cases remain unknown. CASE REPORT We report a case of SCLS in a 49-year-old woman who initially pre

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… In this case, a family history of lymphoproliferative disorders, cardiovascular disease, cancer, and diabetes was identified. …

VEXAS Syndrome in a Patient with Myeloproliferative Neoplasia.

VEXAS syndrome stands for vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome. The syndrome is a combined hematological and rheumatological condition caused by a somatic mutation in the UBA1. There is an association between VEXAS and hematological conditions such as myelodysplastic syndrome (MDS), monoclonal gammopathies of uncertain conditions (MGUS), multiple myeloma (MM), and monoclonal B-cell lymphoproliferative conditions. There are not many descriptions of patients having VEXAS in combination with myeloproliferative neoplasm (MPN). With this article, we want to present a ca

Frequency and causes of anemia in Lymphoma patients.

ObjectivePurpose of this study was to find out frequency of anemia and its causes in newly diagnosed treatment naive lymphoma patients.MethodsWe retrospectively studied all lymphoma patients (> 18 years age) diagnosed and treated at Shaukat Khanum Memorial Cancer Hospital and Research Centre, from January 2016 till January 2017. The data was collected from electronic Hospital Information System. Descriptive statistics were done by using summary measures for categorical variables as well as continuous variables.ResultsOut of a total 408 patients, 272 were males and 136 females. Median age of pa

Symptomatic Hypoalbuminemia as an Indication for Treatment of Waldenström Macroglobulinemia: A Case Report and Review of the Literature.

Hypoalbuminemia has been reported as common in patients with symptomatic Waldenström macroglobulinemia (WM), but it is not typically mentioned as a manifestation of the disease in review articles or articles discussing indications for treatment. We present the case of a patient with WM for whom symptomatic hypoalbuminemia was the primary reason for initiating treatment. Except in rare cases of WM with renal or gastrointestinal involvement, hypoalbuminemia is thought to be due to the effects of elevated levels of inflammation-associated cytokines, and it has been associated with greater likelih

Multiple myeloma presenting as an unhealed extraction socket: Report of a case with brief review of literature.

Multiple myeloma (MM) is a relatively rare malignant hematological disease, which is characterized by a monoclonal malignant proliferation of plasma cells that causes osteolytic lesions. Maxillofacial presentations in patients with MM are not uncommon, but because the symptoms are varied, it is very difficult to diagnose MM in this region especially in patients with initial oral involvement. Furthermore, maxillofacial manifestations as an initial sign or symptom are scarce. We report a case of a 40-year-old male patient who presented with an unhealed socket in lower left back tooth region for

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… An increase in vascular endothelial growth factor, transforming growth factor beta 1 and interleukin 6 (IL-6) levels have been found in patients with lymphoproliferative disorders. IL-6 is considered as the principal growth factor in the pathogenesis of MM. …

Filgrastim XM02 (Tevagrastim®) after autologous stem cell transplantation compared to lenograstim: favourable cost-efficacy analysis.

PurposeGranulocyte colony-stimulating factors (G-CSFs), filgrastim and lenograstim, are recognised to be useful in accelerating engraftment after autologous stem cell transplantation. Several forms of biosimilar non-glycosylated G-CSF have been approved by the European Medicines Agency, with limited published data supporting the clinical equivalence in peripheral blood stem cell mobilisation and recovery after autologous stem cell transplantation.MethodWith the aim of comparing cost-effective strategies in the use of G-CSF after autologous stem cell transplantation, we retrospectively evaluate

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… Aapro MS Bohlius J Cameron DA 2010 update of EORTC guidelines for the use of granulocyte-colony stimulating factor to reduce the incidence of chemotherapy-induced febrile neutropenia in adult patients with lymphoproliferative disorders and solid tumours Eur J Cancer 2011 10.1016/j.ejca.2010.10.013 21095116 …

Renal failure due to primary amyloidosis: a case report and literature review.

ContextPrimary amyloidosis, also known as AL amyloidosis, is commonly caused by clonal expansion of plasma cells in the bone marrow, thereby segregating light chains of clonal immunoglobulin that settle in tissues in the form of insoluble amyloid fibrils. The aim of this study was to report a case of primary amyloidosis with renal failure, diagnosed in Hospital São João, Porto, Portugal, focusing on the diagnostic difficulties and presenting a literature review.Case reportA 68-year-old Caucasian man was admitted to the Internal Medicine Department of the hospital with a condition of anasarca a

Role of Proteasomes in Inflammation.

The ubiquitin-proteasome system (UPS) is involved in multiple cellular functions including the regulation of protein homeostasis, major histocompatibility (MHC) class I antigen processing, cell cycle proliferation and signaling. In humans, proteasome loss-of-function mutations result in autoinflammation dominated by a prominent type I interferon (IFN) gene signature. These genomic alterations typically cause the development of proteasome-associated autoinflammatory syndromes (PRAAS) by impairing proteasome activity and perturbing protein homeostasis. However, an abnormal increased proteasomal

AL Amyloidosis: Current Chemotherapy and Immune Therapy Treatment Strategies: JACC: CardioOncology State-of-the-Art Review.

Immunoglobulin light chain (AL) amyloidosis is an incurable plasma cell disorder characterized by deposition of fibrils of misfolded immunoglobulin free light chains (FLC) in target organs, leading to failure. Cardiac involvement is common in AL amyloidosis and represents the single most adverse prognostic feature. A high index of clinical suspicion with rapid tissue diagnosis and commencement of combinatorial, highly effective cytoreductive therapy is crucial to arrest the process of amyloid deposition and preserve organ function. The clinical use of molecularly targeted drugs, such as protea

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… In a small minority of patients, an underlying lymphoproliferative disorder may not be identified. … Venetoclax BCL2 is a mitochondrial, antiapoptotic protein whose overexpression/overactivity has been shown to drive several hematologic malignancies, particularly B lymphoproliferative disorders ( 116 ). …

Recognition and management of persistent chylomicronemia: A joint expert clinical consensus by the National Lipid Association and the American Society for Preventive Cardiology.

Extreme hypertriglyceridemia, defined as triglyceride (TG) levels ≥1000 mg/dL, is almost always indicative of chylomicronemia. The current diagnostic approach categorizes individuals with chylomicronemia into familial chylomicronemia syndrome (FCS; prevalence 1-10 per million), caused by the biallelic combination of pathogenic variants that impair the lipolytic action of lipoprotein lipase (LPL), or multifactorial chylomicronemia syndrome (MCS, 1 in 500). A pragmatic framework should emphasize the severity of the phenotype and the risk of complications. Therefore, we endorse the term "persiste