China
Africa
Explore chapters and articles related to this topic
B Cells and Humoral Immunity
Published in Constantin A. Bona, Francisco A. Bonilla, Textbook of Immunology, 2019
Constantin A. Bona, Francisco A. Bonilla
The rare heavy chain diseases are additional variants of lymphoid malignancy. The malignant cells have lost the ability to synthesize a complete antibody molecule, and produce only the heavy chain or a part of it. Molecular analyses have shown deletions of large portions of Ig genes, insertions of non-Ig sequences into Ig genes, mutations, and chromosomal translocations. All of these may affect not only the functionally rearranged genes, but the non-expressed genes as well. This suggests that the underlying defect disrupts the Ig locus at some point after rearrangement.
Lymphocyte and plasma cell malignancies
Published in Gabriel Virella, Medical Immunology, 2019
Juan Carlos Varela, Gabriel Virella
Some B-cell dyscrasias are associated with the exclusive production of heavy chains (or fragments thereof) or with the synthesis of abnormal heavy chains that are not assembled as complete immunoglobulin molecules and are excreted as free heavy chains. Both types of abnormality can be on the basis of a heavy-chain disease. The heavy-chain diseases are classified according to the isotype of the abnormal heavy chain as γ, α, μ, and δ. (A single case of δ-chain disease has been reported, and ε-chain disease has yet to be described.)
The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Published in Harold R. Schumacher, William A. Rock, Sanford A. Stass, Handbook of Hematologic Pathology, 2019
Lynne V. Abruzzo, L. Jeffrey Medeiros
The heavy chain diseases are a group of rare monoclonal gammopathies that are characterized by the production of free immunoglobulin α, μ, or γ heavy chains. Alpha-chain disease, the most common of the heavy chain diseases, is now termed immunoproliferative small intestinal disease (IPSID). IPSID is a disease of young adults that is most common in the Middle East. Patients present with signs and symptoms of severe malabsorption. The small intenstine and mesenteric lymph nodes are infiltrated by malignant lymphoplasmacytic cells, which generally remain confined to these sites. Patients usually die from malabsorption.
Curative effect of cold micro forceps and CO2 laser in the treatment of laryngeal amyloidosis
Published in Acta Oto-Laryngologica, 2019
Xiufa Wu, Jing Zhang, Chunsheng Wei
Amyloidosis encompasses a group of disorders characterized by the deposition of abnormal amounts of extracellular protein in various organs. It can be localized or systemic, primary or secondary. LA represents 0.68% of benign tumors of the larynx. It usually affects individuals 0 and 70 years of age (74.6% in our study), predominately men (1.7:1 in our study). The onset is manifested by dysphonia that may develop into stridor and dyspnea [10]. In our study, the localized deposition in a single organ (larynx in the study) without generalized disease is defined as LA [4]. The systemic amyloidosis which is associated with multiple myeloma, Waldenström’s disease and heavy chain disease were not studied in the study.