This disease is characterized by an excessive production of immunoglobulin heavy chains that are short and truncated. The amino acid sequence on the amino terminal is completely normal, but there is a deletion in the protein structure that extends from the middle of the variable region, through the first domain of the constant region, and ending just before the position of the first disulfide bond between the two heavy chains. This deletion causes the heavy chains to lose the ability to form disulfide bonds with the light chains. The defect in the immunoglobulins arises during the faulty coupling of the variable and constant regions during somatic recombination.
The γHCD can be divided into three categories based on the various clinical and pathological features. These categories are disseminated lymphoproliferative disease, localized proliferative disease and no apparent proliferative disease.
Composite nodular lymphocyte-predominance hodgkin disease and gamma-heavy-chain disease: A case report and review of the literature
Jun 01, 2001; * The association of Hodgkin disease with monoclonal gammopathy has rarely been reported. We present a case of a 48-year-old...
Composite Nodular Lymphocyte-Predominance Hodgkin Disease and [Gamma]-Heavy-Chain Disease: A Case Report and Review of the Literature.
Jun 01, 2001; Given the clonal B-cell origin of most non-Hodgkin lymphomas, it is not surprising that many lymphomas are associated with the...