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Characteristics of large granular lymphocyte leukemia associated with variable common immunodeficiency disorders: A study of 12 cases.
Eur J Haematol
October 2024
Internal Medicine and Infectious diseases, Hôpital Haut-Lévêque, Pessac, France.
Objectives: Common Variable Immunodeficiency Disorders (CVID) and Large Granular Lymphocytes leukemia (LGLL) exhibit diverse clinical manifestations including infections, dysimmunity, and lymphoproliferation. Recent decades have seen the discovery of new genes in the lymphopoiesis pathway, such as JAK-STAT. This case series supplemented by a literature review aims to describe clinical and biological characteristics of patients with both CIVD and LGLL.
View Article and Find Full Text PDFStem Cell Defect in Ubiquitin-Green Fluorescent Protein Mice Facilitates Engraftment of Lymphoid-Primed Hematopoietic Stem Cells.
Stem Cells
August 2018
Institute of Pathological Physiology, First Faculty of Medicine, Charles University.
Transgenic mice expressing green fluorescent protein (GFP) are useful in transplantation experiments. When we used ubiquitin-GFP (UBC-GFP) transgenic mice to study the availability of niches for transplanted hematopoietic stem and progenitor cells, the results were strikingly different from the corresponding experiments that used congenic mice polymorphic in the CD45 antigen. Analysis of these unexpected results revealed that the hematopoiesis of UBC-GFP mice was outcompeted by the hematopoiesis of wild-type (WT) mice.
View Article and Find Full Text PDFBone marrow as a priming site for T-cell responses to blood-borne antigen.
Nat Med
September 2003
Tumor Immunology Program, German Cancer Research Center (DKFZ), Heidelberg, Germany.
Although bone marrow is known as a primary lymphoid organ, its potential to serve as a secondary immune organ has hardly been explored. Here we demonstrate that naive, antigen-specific T cells home to bone marrow, where they can be primed. Antigen presentation to T cells in bone marrow is mediated via resident CD11c+ dendritic cells.
View Article and Find Full Text PDFEffect of early fetal splenectomy on prenatal B-cell development in sheep.
Immunology
February 2001
Department of Morphology, Genetics and Aquatic Biology, Norwegian School of Veterinary Science, Oslo, Norway.
The contribution of early splenic B-cell populations to the colonization of the ileal Peyer's patch was investigated following the surgical removal of the spleen in a series of 56-day-old fetal sheep. The fetuses were killed at 140 days of gestation and the ileal Peyer's patch, the distal jejunal lymph node which drains the Peyer's patch, and a peripheral lymph node, the superficial cervical lymph node, were examined. Enzyme and immunohistochemical evaluation concluded that the distribution of B cells, T cells and stromal cells in the ileal Peyer's patch was similar in splenectomized and normal fetal sheep.
View Article and Find Full Text PDFLate onset haemolysis and red cell autoimmunisation after allogeneic bone marrow transplant.
Bone Marrow Transplant
March 1997
Department of Haematology, Royal Postgraduate Medical School, London, UK.
We reviewed the medical records of 293 patients who underwent allogeneic bone marrow transplants at the Hammersmith Hospital between 1989 and 1994. There was clinical evidence of an autoimmune reaction against red cells in nine patients. Seven of these patients had significant haemolysis; the other two had red cell autoagglutination.
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