Diamond-Blackfan anemia (DBA) is a rare congenital disease caused by mutations in ribosomal protein genes and is characterized by pure red cell aplasia. While the prognosis is relatively favorable, quality of life (QOL) among DBA patients is negatively impacted by the adverse effects of long-term prednisolone (PSL) therapy and blood transfusions. We describe a 43-year-old man who was diagnosed with DBA (Hb of 2.18 g/dl) at the age of two months. He was initially treated with PSL and blood transfusions, followed by cyclosporine and low-dose (6 mg/day) PSL, which resulted in a sustained hemoglobin level of 9 g/dl without severe adverse events or loss of QOL. High levels of eADA and GSH as well as a RPS19 gene mutation were confirmed. The only curative therapy is hematopoietic stem cell transplantation, which is associated with significant mortality. However, using low-dose PSL to maintain a stable hemoglobin level may improve QOL for patients who receive curative treatment.
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http://dx.doi.org/10.11406/rinketsu.58.917 | DOI Listing |
Clin Transplant
January 2025
Glickman Urological & Kidney Institute, Cleveland Clinic, Cleveland, Ohio, USA.
Background: Enhanced recovery after surgery (ERAS) protocols have gained widespread acceptance as a means to enhance surgical outcomes. However, the intricate care required for kidney transplant recipients has not yet led to the establishment of a universally recognized and dependable ERAS protocol for kidney transplantation.
Objective: We devised a customized ERAS protocol to determine its effectiveness in improving surgical and postoperative outcomes among kidney transplant recipients.
J Pediatr Hematol Oncol
January 2025
Departments of Pediatric Hematology.
Congenital thrombotic thrombocytopenic purpura (cTTP), which is associated with mutations in the gene for a disintegrin and metalloproteinase with a thrombospondin type 1 motif member 13 (ADAMTS13), is a chronic and lifelong disease. The clinical course is variable. Regularly using ADAMTS13-containing products such as fresh frozen plasma (FFP) for long-term prophylaxis is the most important treatment to prevent thrombotic microangiopathy (TMA) episodes.
View Article and Find Full Text PDFClin Transplant
January 2025
Division of Infectious Diseases, Department of Medicine, Duke University School of Medicine, Durham, North Carolina, USA.
Background: Early posttransplant cytomegalovirus (CMV) infections in CMV seronegative solid organ transplant recipients (SOTR) with CMV seronegative donors (D-/R-) are often attributed transfusion-transmitted CMV. The prevalence of false-negative donor CMV serology in D-/R- SOTR with early CMV infections has not been explored.
Methods: We determined the frequency and characteristics of CMV DNAemia that occurred within 90 days of transplant among adult SOTR classified as D-/R- who underwent a first SOT at a single center between February 25, 2014 and February 25, 2024.
Iran J Microbiol
December 2024
Blood Transfusion Research Center, High Institute for Research and Education in Transfusion Medicine, Tehran, Iran.
Background And Objectives: Recently, the anti-herpetic activities of different plant species have been investigated. This study evaluated the effects of aqueous extract on the HSV-1 virus-infected Vero cell.
Materials And Methods: The IC of the aqueous extract was obtained by the maceration of the plant in boiling water and has been measured with the MTT method, also the q-PCR was used to study viral gene expression reduction.
Front Immunol
December 2024
German Cancer Consortium (DKTK), Partner site Frankfurt/Mainz, a partnership between DKFZ and University Medical Center Frankfurt, Frankfurt, Germany.
Introduction: Posttransplant cyclophosphamide (PTCy) has revolutionized the landscape of human leukocyte antigen (HLA)-haploidentical hematopoietic cell transplantation (haplo-HCT), providing a pivotal therapeutic option for patients with hematological malignancies who lack an HLA-matched donor.
Methods: In this retrospective analysis involving 54 adult patients undergoing PTCy-based haplo-HCT, we evaluated the impact of inhibitory killer immunoglobulin-like receptor (KIR)/HLA mismatch, alongside patient, donor, and transplant factors, on clinical outcomes within a homogeneous cohort characterized by a myeloablative conditioning regimen and bone marrow graft.
Results: With a median follow-up of 73.
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