A complete list of definite, as well as possible, indications for hemopoietic stem cell transplantation in primary immunodeficiency is provided. Included are: severe combined immunodeficiency, profound T cell defects, autoimmune and autoinflammatory syndromes, innate immune defects, hemophagocytic disorders, and other conditions. Some causes and limitations are included.
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http://dx.doi.org/10.1016/j.iac.2010.03.004 | DOI Listing |
Cureus
October 2024
Emergency Medicine, Southern Health and Social Care Trust, Craigavon, GBR.
Atraumatic splenic rupture (ASR) is an unfamiliar entity that is potentially life-threatening if there is a delay in the diagnosis. Due to its rarity and its non-specific presentation, it can be a challenge to diagnose early. In this report, we present a case of a 42-year-old male patient who presented to the emergency department with nonspecific abdominal pain and had no past medical history.
View Article and Find Full Text PDFJ Clin Immunol
August 2024
Willem-Alexander Children's Hospital, Department of Pediatrics, Division of Pediatric Immunology, Hematology and Stem Cell Transplantation, Leiden University Medical Center, Leiden, The Netherlands.
Immunodeficiency-Centromeric instability-Facial dysmorphism (ICF) syndrome is an inborn error of immunity characterized by progressive immune dysfunction and multi-organ disease usually treated with antimicrobial prophylaxis and immunoglobulin substitution. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative treatment, but data on outcome are scarce. We provide a detailed description of disease characteristics and HSCT outcome in an international cohort of ICF syndrome patients.
View Article and Find Full Text PDFCD7 targeted CAR-T has demonstrated potential in the treatment of T cell malignancies but no study has been reported about its potential in the prophylaxis of GVHD in allo-HSCT. Here we reported a special case that a boy diagnosed with refractory acute T lymphoblastic leukemia (T-ALL) was treated with universal CD7 targeted CAR-T (CD7 UCAR-T) and parent-derived peripheral blood stem cells (PBSCs). Complete remission and full engraftment of donor was observed.
View Article and Find Full Text PDFIntern Med J
September 2024
School of Clinical Medicine, St Vincent's Healthcare Clinical Campus, Faculty of Medicine and Health, University of New South Wales, Sydney, New South Wales, Australia.
Background: Autologous haemopoietic stem cell transplantation (AHSCT) is an effective treatment for systemic sclerosis (SSc); however, treatment-related toxicity remains a key issue.
Aims: To investigate the perceptions of rheumatologists on the use of AHSCT for SSc.
Methods: Australian rheumatologists were asked for their opinion on the role of AHSCT, the indications for treatment and the barriers to the use of AHSCT for SSc.
Int J Surg Case Rep
September 2022
Department of Surgery, Cantonal Hospital Thurgau, Münsterlingen, Switzerland. Electronic address:
Introduction And Importance: Adrenal myelolipomas (AMLs) are rare, non-functional, benign tumours mostly diagnosed incidentally. They present as small and unilateral masses that are histologically composed of mature adipose tissue with admixed haemopoietic elements. In a small percentage of patients, pressure symptoms, retroperitoneal haemorrhage or tumour rupture may occur.
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