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IgG4-related disease for the hematologist.
Hematology Am Soc Hematol Educ Program
December 2024
Division of Hematology, University of British Columbia, Vancouver, British Columbia, Canada; and Division of Hematology, Dalhousie University, Halifax, Nova Scotia, Canada.
Immunoglobulin G4-related disease (IgG4-RD) is an immune-mediated disease with many important manifestations in hematopoietic and lymphoid tissue. IgG4 is the least naturally abundant IgG subclass, and the hallmark feature of IgG4-RD is markedly increased IgG4-positive plasma cells (with an IgG4 to IgG ratio >40%) in affected tissue, along with elevated polyclonal serum IgG and IgG4 in most patients. Histological diagnosis is essential, and other key features include storiform fibrosis, lymphoplasmacytic infiltrate, tissue eosinophilia, and obliterative phlebitis.
View Article and Find Full Text PDFAdvances in Understanding and Management of Erdheim-Chester Disease.
Life Sci
July 2024
Department of Pharmaceutical Biotechnology, Manipal College of Pharmaceutical Sciences, Manipal Academy of Higher Education, Manipal, Karnataka, India. Electronic address:
Erdheim Chester Disease (ECD) is a rare histiocytic disorder marked by infiltration of organs with CD68 histiocytes. ECD stems from mutations of BRAF and MAP2K1 in hematopoietic stem and progenitor cells (HSPCs), which further differentiate into monocytes and histiocytes. Histopathology reveals lipid-containing histiocytes, which test positive for CD68 and CD133 in immunohistochemistry.
View Article and Find Full Text PDFLong-term experience in treatment of acute promyelocytic leukemia in Mexican children in a tertiary care hospital.
Front Oncol
November 2023
Myeloid Leukemia Clinic, Federico Gómez, Children's Hospital of Mexico, Mexico City, Mexico.
Introduction: Acute promyelocytic leukemia (APL) is a rare myeloid leukemia subtype affecting adult and pediatric populations. APL constitutes 15-20% of all childhood AML in Latin America, compared to 7% in the non-Latino population. This leukemia has unique characteristics, such as its association with chromosomal translocations involving the retinoid acid receptor α (RARA) gene on chromosome 17.
View Article and Find Full Text PDFIntracranial extramedullary hematopoiesis in a patient with beta-thalassemia presenting with head trauma.
Surg Neurol Int
November 2022
Department of Neurological Surgery, Icahn School of Medicine at Mount Sinai, New York City, New York, United States.
Background: Beta-thalassemia is an inherited hemoglobinopathy, whereby reduced or absent expression of beta-globin genes causes impaired erythropoiesis. Extramedullary hematopoiesis (EMH) occurs in 1% of all patients with beta-thalassemia major receiving regular transfusions and is exceedingly rare intracranially.
Case Description: We report a case of a male in his 20s with beta thalassemia who presented with head trauma found to have intracranial EMH mimicking multiple extra-axial hematomas.
MYC and TP53 Alterations but Not MAPK Pathway Mutations Are Common Oncogenic Mechanisms in Follicular Dendritic Cell Sarcomas.
Arch Pathol Lab Med
August 2023
From the Department of Pathology, Hospital Clínic of Barcelona, Barcelona, Spain (Frigola, Bühler, Marginet, Alamo, Aldecoa, Campo, Balagué).
Context.—: Despite their stromal origin, follicular dendritic cells (FDCs) share many functions with hematopoietic system cells. FDC neoplasms are currently classified by the World Health Organization along with those of a histiocytic nature.
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