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Heavy chain diseases.
- Source :
-
Best practice & research. Clinical haematology [Best Pract Res Clin Haematol] 2005; Vol. 18 (4), pp. 729-46. - Publication Year :
- 2005
-
Abstract
- Heavy chain diseases (HCDs) are rare B-cell lymphoplasma-cell proliferative disorders characterized by production of truncated monoclonal immunoglobulin heavy chains without associated light chains. HCDs involving the three main immunoglobulin classes have been described; alpha-HCD is the most common and has the most uniform presentation, gamma- and mu-HCDs have variable clinical presentations and histopathologic features. HCDs can be thought of as variant types of non-Hodgkin lymphoma: alpha-HCD presents as an extranodal marginal-zone lymphoma of mucosa-associated lymph-node tissue, gamma-HCD as lymphoplasmacytoid non-Hodgkin lymphoma, and mu-HCD as small lymphocytic non-Hodgkin lymphoma or chronic lymphocytic leukemia. Diagnosis of HCD requires documentation of a deleted immunoglobulin heavy chain without a bound light chain in the serum or urine. Prognosis is variable, and no standardized effective treatment programs are available except for alpha-HCD, which in its early stage may respond to antibiotics.
Details
- Language :
- English
- ISSN :
- 1521-6926
- Volume :
- 18
- Issue :
- 4
- Database :
- MEDLINE
- Journal :
- Best practice & research. Clinical haematology
- Publication Type :
- Academic Journal
- Accession number :
- 16026747
- Full Text :
- https://doi.org/10.1016/j.beha.2005.01.029