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The prevalence of antifungal agents administration in patients undergoing allogeneic hematopoietic stem cell transplantation: a retrospective study.

BackgroundInvasive fungal infections (IFIs) are chief infectious complications in patients undergoing hematopoietic stem cell transplantation (HSCT). However, the diagnosis of fungal infections is difficult, and often empiric treatment initiates. Since there is no data available on the prevalence of antifungal drugs administration in allogeneic HSCT recipients in Iran, we decided to conduct this study.MethodsThis study was a retrospective review of records of patients who received allogeneic HSCT in the Hematology-Oncology, Bone Marrow Transplantation center at Shariati Hospital in Tehran, bet

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… Receive Treatment AML: Acute Myelogenous Leukemia, ALL: Acute Lymphoblastic Leukemia, AA: Aplastic Anemia, CML: Chronic Myeloid Leukemia, MM: Multiple Myeloma, MDS: Myelodysplastic syndrome, CR: Complete Remission † Other conditions consisted of one patient with the diagnosis of paroxysmal nocturnal hemoglobinuria …

Determinants and Clinical Significance of Musculoskeletal Symptoms in Patients With Chronic Graft-Versus-Host Disease.

Musculoskeletal symptoms in chronic graft-versus-host disease (cGVHD) are rare manifestations contributing to disease burden. This study assesses the frequency of muscle cramps, joint and muscle aches, and muscle weakness in a cohort of patients severely affected by cGVHD. Three hundred thirty-four patients participated in the NCI natural history study of cGVHD (NCT00092235) from October 2004 to March 2017. Five-point Lee cGVHD Symptom Scale was dichotomized (less symptom bother-0, 1, 2; severe symptom bother-3, 4) and tested for associations with: Short Form 36 (SF36), 2-minute walk test, gri

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… (0–222) Sex | Male | 190 (56.8) Female | 144 (43.2) Disease | Acute leukemias and myelodysplasia | 155 (46.4) Myeloproliferative diseases | 42 (12.6) Chronic lymphocytic leukemia | 22 (6.6) Hodgkin and Non-Hodgkin lymphomas | 82 (24.6) Multiple myeloma | 17 (5.1) Aplastic anemia, paroxysmal night hemoglobinuria …

High frequency of cutaneous manifestations including vitiligo and alopecia areata in a prospective cohort of patients with chronic graft-vs-host disease.

AimTo determine the frequency and the characteristics of cutaneous manifestations, especially vitiligo and alopecia areata, in patients with chronic graft-vs-host disease (cGVHD).Methods50 patients with cGVHD were prospectively enrolled in the observational study protocol and evaluated by an experienced dermatologist. The evaluation was focused on the clinical spectrum of skin and adnexal involvement, and the cutaneous GVHD score was determined according to National Institutes of Health (NIH) Consensus criteria. The presence of vitiligo, alopecia, xerosis, nail changes, and dyspigmentation was

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… Table 1 Demographic characteristics of the study population* *AML – acute myeloid leukemia, ALL – acute lymphoblastic leukemia, MDS – myelodysplastic syndrome, CML – chronic myeloid leukemia, MPN – myeloproliferative neoplasm, AA – aplastic anemia, PNH – paroxysmal nocturnal hemoglobinuria, NHL – Non …

Accelerated bone mass senescence after hematopoietic stem cell transplantation.

Osteoporosis and avascular necrosis (AVN) are long-lasting and debilitating complications of hematopoietic stem cell transplantation (HSCT). We describe the magnitude of bone loss, AVN and impairment in osteogenic cell compartment following autologous (auto) and allogeneic (allo) HSCT, through the retrospective bone damage revaluation of 100 (50 auto- and 50 allo-HSCT) long-term survivors up to 15 years after transplant. Current treatment options for the management of these complications are also outlined. We found that auto- and allo-HSCT recipients show accelerated bone mineral loss and micr

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… haematopoietic stem cell transplantation Infez Med 2012 1 20 26 34 23042003 [23] Risitano AM Notaro R Pascariello C Sica M Del Vecchio L Horvath CJ Fridkis-Hareli M Selleri C Lindorfer MA Taylor RP Luzzatto L Holers VM The complement receptor 2/factor H fusion protein TT30 protects paroxysmal nocturnal hemoglobinuria …

Novel Immunotherapies for Myasthenia Gravis.

Myasthenia gravis (MG), a prototype autoimmune neurological disease, had its therapy centred on corticosteroids, non-steroidal broad-spectrum immunotherapy and cholinesterase inhibitors for several decades. Treatment-refractory MG and long-term toxicities of the medications have been major concerns with the conventional therapies. Advances in the immunology and pathogenesis of MG have ushered in an era of newer therapies which are more specific and efficacious. Complement inhibitors and neonatal Fc receptor blockers target disease-specific pathogenic mechanisms linked to myasthenia and have pr

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… It was previously licensed for therapy of paroxysmal nocturnal hemoglobinuria (PNH) and atypical haemolytic uraemic syndrome (HUS) 32 , 33 and more recently for aquaporin IgG positive neuromyelitis optica spectrum disorder (NMOSD). 34 It was approved for therapy of gMG in 2019. … Efficacy and safety of eculizumab for paroxysmal nocturnal hemoglobinuria: a systematic review and meta-analysis. J Pediatr Hematol Oncol . 2021;43(6):203–210. doi: 10.1097/MPH.0000000000002178 33. Mahat U, Matar RB, Rotz SJ. …

The effect of oral chronic graft-versus-host disease on bodyweight: A cohort study.

AimThis retrospective cohort study aimed to evaluate the association between body weight and oral cGVHD (chronic graft versus host disease).MethodsPatients with oral cGVHD were compared with an age and gender-matched non-GVHD cohort in terms of demographic information, body mass index (BMI), date of transplant, length of hospitalization, and oral complications. Weight was stratified in pre-and post-transplant weight, mean weight after acquiring cGVHD for the first year, and post-oral cGVHD BMI. Each patient was matched and compared with two controls at a 1:2 ratio. Firth's penalized likelihood

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… | 28 (22.4) Acute Myeloid Leukemia (AML) | 23(18.4) Thalassemia and SCA | 6 (4.80) Hodgkin’s lymphoma (HL) | 5 (4.00) AA (Aplastic anemia) | 5 (4.00) Multiple Myeloma (MM) | 3 (2.40) Chronic Myelogenous Leukemia (CML) | 2 (1.60) T cell Prolymphocytic Leukemia (T-PLL) | 2 (1.60) Paroxysmal Nocturnal Hemoglobinuria …

Fanconi syndrome induced by the long-term use of tenofovir disoproxil fumarate: a case report and literature review.

We present the case of a woman of 50 years of age who experienced widespread bone pain along with digestive symptoms, including nausea and vomiting. She had been prescribed tenofovir disoproxil fumarate (TDF) tablets for the treatment of hepatitis B. Laboratory testing revealed low circulating phosphorus and potassium concentrations and acidosis. A whole-body bone scan revealed abnormal bone metabolism. Rheumatologic and urologic conditions were ruled out, and therefore TDF-induced Fanconi syndrome (FS) and related bone pain was diagnosed. After the TDF was discontinued, the patient's symptoms

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… The systemic diseases that cause FS include amyloidosis, multiple myeloma/light chain disease, paroxysmal nocturnal hemoglobinuria, kidney transplantation, tubulointerstitial nephritis, and membranous nephropathy with anti-tubular basement membrane antibodies. …

Neutrophil diversity and function in health and disease.

Neutrophils, the most abundant type of granulocyte, are widely recognized as one of the pivotal contributors to the acute inflammatory response. Initially, neutrophils were considered the mobile infantry of the innate immune system, tasked with the immediate response to invading pathogens. However, recent studies have demonstrated that neutrophils are versatile cells, capable of regulating various biological processes and impacting both human health and disease. Cytokines and other active mediators regulate the functional activity of neutrophils by activating multiple receptors on these cells,

Spectrum of Pancytopenia in Adults Attending a Clinical Hematology Department: A Four-Year Experience From a Tertiary Care Center of Western India.

Background Pancytopenia is a common hematological condition encountered in clinical practice. Because there is a wide variation in causes of pancytopenia even in different populations of the same geographical region, identifying the pattern of underlying etiologies is crucial for proper management. This study was conducted to evaluate the clinico-hematological profile and different etiologies of pancytopenia among adults presenting to a clinical hematology department at a tertiary care hospital of Western India. Methodology This observational study was conducted over a period of four years on

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… myelofibrosis | 12 (2.2) | 10 (1.8) | 22 (4.0) Connective tissue disorders | 6 (1.1) | 14 (2.6) | 20 (3.7) Drug-induced pancytopenia | 4 (0.7) | 9 (1.7) | 13 (2.4) Metastatic carcinoma | 9 (1.6) | 3 (0.6) | 12 (2.2) Hemophagocytic lymphohistiocytosis | 6 (1.1) | 4 (0.7) | 10 (1.8) Paroxysmal nocturnal hemoglobinuria …

From Traditional to Targeted Immunotherapy in Myasthenia Gravis: Prospects for Research.

Treatment of Myasthenia Gravis (MG) is still based on non-specific immunosuppression. Long-term high dose corticosteroids is still a major cause of side effects, in young as well as in elderly patients in whom comorbidities further increase the burden of chronic immunosuppression. Moreover, awareness of the limits of traditional therapies has led to the concept of "refractory MG." The therapeutic approach to MG is therefore progressively evolving from the classic combination of corticosteroids and immunosuppressive drugs to new biological compounds targeting different immunopathological steps.

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… Notably, ECU safety in MG was similar to that observed in neuromyelitis optica ( 31 ) as well as in the long-term use for paroxysmal nocturnal hemoglobinuria ( 32 ). …

Imbalances in serum angiopoietin concentrations are early predictors of septic shock development in patients with post chemotherapy febrile neutropenia.

BackgroundFebrile neutropenia carries a high risk of sepsis complications, and the identification of biomarkers capable to identify high risk patients is a great challenge. Angiopoietins (Ang -) are cytokines involved in the control microvascular permeability. It is accepted that Ang-1 expression maintains endothelial barrier integrity, and that Ang-2 acts as an antagonizing cytokine with barrier-disrupting functions in inflammatory situations. Ang-2 levels have been recently correlated with sepsis mortality in intensive care units.MethodsWe prospectively evaluated concentrations of Ang-1 and

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… Table 1 Patient characteristics ¥ Non-complicated sepsis; *Mann-Whitney test; **Fisher's exact test; €: Lymphoma (n = 8); Multiple Myeloma (n = 5); Myeloproliferative diseases (n = 5); Paroxysmal Nocturnal Hemoglobinuria (n = 2); HSCT: Hematopoietic stem cell transplantation; CTx: chemotherapy. £: Gram …

Editorial: The Role of Complement in Health and Disease.

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… Paroxymal nocturnal hemoglobinuria is caused by a mutation of the PIG-A gene, resulting in absence of regulatory CD55 and CD59 on affected cells. It is associated with intravascular hemolysis, thrombosis and bone marrow failure. …

Health-Related Quality of Life, Illness Perception, Stigmatization and Optimism Among Hematology Patients: Two Exploratory Path Models.

Background and Objectives: Hematological diseases can cause lasting physical and social impairments. Illness perception and emotional functioning, influenced by stigmatization and optimism, may shape these outcomes, yet their combined effects on health-related quality of life remain underexplored. This study investigates their direct and indirect links with physical and social functioning. Materials and Methods: Ninety-six hematology patients completed the EORTC QoL Scale, the Brief Illness Perception Questionnaire, the Stigma Scale for Chronic Illness, and the Revised Life Orientation Test. A

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… Definitions of abbreviations: ET = essential thrombocytosis; TTP = thrombotic microangiopathy; PNH = paroxysmal nocturnal hemoglobinuria. …

Cold versus warm autoimmune hemolytic anemia in diffuse large B cell lymphoma: pathogenic and therapeutic implications: a case series.

BACKGROUND: Autoimmune hemolytic anemia-associated diffuse large B cell lymphoma is rare, with distinct pathogenic mechanisms and therapeutic responses in cold agglutinin syndrome (CAS) and warm autoimmune hemolytic anemia subtypes poorly characterized. This report highlights two novel cases demonstrating subtype-specific molecular drivers and treatment outcomes, underscoring the necessity for precision management. CASE PRESENTATION: Two patients presented with autoimmune hemolytic anemia: a 29-year-old Han male with cold agglutinin syndrome and a 37-year-old Han female with warm autoimmune h

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… Immunoglobulins and complement levels, serum immunofixation electrophoresis, serum protein electrophoresis, serum free light chain assay, paroxysmal nocturnal hemoglobinuria (PNH) clone detection, extended immunologic panel, routine urinalysis, and stool routine with occult blood test showed no significant …

Long-Term Immune Response to SARS-CoV-2 Vaccination in Hematologic Malignancies: An Update of the ImV-HOng Trial of the East German Study Group for Hematology and Oncology.

Purpose: Evaluate long-term immunogenicity and its association with the number of vaccines and breakthrough infections in patients with hematologic malignancies compared to a healthy cohort. Methods: This study is an amendment of a multicenter study (DRKS00027372) which described the upsurge of anti-spike-IgGs on day 120 from a blunted day-35 response in patients with hematologic neoplasms. In this amendment, 191 individuals from the original study (patients with myeloid and lymphoid neoplasms and controls) were followed beyond month 12 after first SARS-CoV-2-vaccination. The long-term humoral

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… acute lymphoid leukemia (n = 7), monoclonal gammopathy of undetermined significance (n = 3), primary immune thrombocytopenia (ITP, n = 3), chronic myelomonocytic leukemia (n = 1), pure red cell aplasia (n = 1), pure graft function after allogeneic stem cell transplantation (n = 1), paroxysmal nocturnal hemoglobinuria …

Recent advances in treatments of adult immune thrombocytopenia.

Immune thrombocytopenia (ITP) is isolated thrombocytopenia characterized by autoimmune-mediated disruption of platelet without other etiologies. Treatments for chronic ITP consist of corticosteroids, intravenous immunoglobulins, anti-D immunoglobulin, rituximab, thrombopoietin receptor agonists, immunosuppressants and splenectomy. Although current therapies are effective in over two-thirds of patients, some patients are refractory to therapies or fail to achieve long-term responses. Recently, great advance has been made in identifying various mechanisms involved in ITP pathogenesis, and new tr

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… partnerID=HzOxMe3b&scp=0031905020&origin=inward 9523744 17 Gaines AR 2000 Acute onset hemoglobinemia and/or hemoglobinuria and sequelae following Rh(o)(D) immune globulin intravenous administration in immune thrombocytopenic purpura patients Blood 95 2523 9 10.1182/blood.V95.8.2523 10753830 18 Bao …

Pricing and Reimbursement Pathways of New Orphan Drugs in South Korea: A Longitudinal Comparison.

This study aimed to analyze four current pathways affecting the listing and post-listing prices of new orphan drugs (ODs) in South Korea. These mechanisms were: (1) essential OD, (2) pharmacoeconomic evaluation (PE) waiver OD, (3) weighted average price OD, and (4) PE OD. We analyzed the ratio of the listing price of 48 new ODs to the average adjusted price (AAP) of seven advanced countries and examined the change in the post-listing price. Descriptive statistics were used to analyze the listing and post-listing price changes. The mean and median ratios of the listing price of total new OD to

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… 98,766 | 100 mg | 65.2 (miglustat) | 89.8 7 | Inovelon | Lennox–Gastaut syndrome | 05/01/2010 | NA | 1280 | 400 mg | 39.7 (rufinamide) | 1.2 8 | Remodulin | Pulmonary arterial hypertension | 12/01/2010 | NA | 5,720,000 | 2.5 mg/mL | 62.5 (treprostinil sodium) | 5200.0 9 | Soliris | Paroxysmal nocturnal hemoglobinuria …

How to Choose the Right Treatment for Membranous Nephropathy

Membranous nephropathy is an autoimmune disease affecting the glomeruli and is one of the most common causes of nephrotic syndrome. In the absence of any therapy, 35% of patients develop end-stage renal disease. The discovery of autoantibodies such as phospholipase A2 receptor 1, antithrombospondin and neural epidermal growth factor-like 1 protein has greatly helped us to understand the pathogenesis and enable the diagnosis of this disease and to guide its treatment. Depending on the complications of nephrotic syndrome, patients with this disease receive supportive treatment with diuretics, AC

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… If paroxysmal nocturnal hemoglobinuria is superimposed, complement-directed therapy may be helpful [ 104 ]. In addition, secondary MN of Castleman’s disease has been successfully treated with tocilizumab from both a renal and hematological perspective [ 105 , 106 ]. … Complement Inhibition Therapy and Dialytic Strategies in Paroxysmal Nocturnal Hemoglobinuria: The Nephrologist’s Opinion. J. Clin. Med. 2020;9:1261. doi: 10.3390/jcm9051261. 105. Saiki R., Katayama K., Hirabayashi Y., Oda K., Fujimoto M., Murata T., Nakajima A., Dohi K. …

B Cells and Antibodies as Targets of Therapeutic Intervention in Neuromyelitis Optica Spectrum Disorders.

The first description of neuromyelitis optica by Eugène Devic and Fernand Gault dates back to the 19th century, but only the discovery of aquaporin-4 autoantibodies in a major subset of affected patients in 2004 led to a fundamentally revised disease concept: Neuromyelits optica spectrum disorders (NMOSD) are now considered autoantibody-mediated autoimmune diseases, bringing the pivotal pathogenetic role of B cells and plasma cells into focus. Not long ago, there was no approved medication for this deleterious disease and off-label therapies were the only treatment options for affected patient

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… Eculizumab decreased serum IL-6 levels in patients with paroxysmal nocturnal hemoglobinuria and increased B cell counts [ 166 , 167 ]. … Effects on the B cell compartment in NMOSD patients are not yet assessed in detail and may strongly differ from those in paroxysmal nocturnal hemoglobinuria due to a completely different pathophysiology. 3.6.2. …

Identification of Fatty Acid Metabolism Disorder-Related Gene Signature in Septic Cardiomyopathy.

Evans syndrome (ES) is a rare condition, defined as the presence of 2 autoimmune cytopenias, most frequently autoimmune hemolytic anemia and immune thrombocytopenia (ITP) and rarely autoimmune neutropenia. ES can be classified as primary or secondary to various conditions, including lymphoproliferative disorders, other systemic autoimmune diseases, and primary immunodeficiencies, particularly in children. In adult ES, little is known about clinical features, disease associations, and outcomes. In this retrospective international study, we analyzed 116 adult patients followed at 13 European ter

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… leukemia, n = 1), and primary immunodeficiencies (n = 5, including common variable immunodeficiency, n = 2; autoimmune lymphoproliferative syndrome, n = 1; Kabuki syndrome, n = 1; and nonfamilial hypogammaglobulinemia, n = 1); rarer associations were solid cancers (thymoma, n = 1), paroxysmal nocturnal hemoglobinuria …