Canterbury DHB
This is a rare B-cell neoplasm. Although the cells produce a monoclonal protein, this consists only of heavy chains, either IgG, IgA or IgM. It does not behave like a plasma cell neoplasm.
Each type of Heavy Chain disease appears to be a variant of a type of NHL. IgG heavy chain is associated with lympho-plasmacytic lymphoma, IgA heavy chain with an extra-nodal marginal zone lymphoma (MALT), and IgM heavy chains with CLL. See the WHO Classification of Tumours - Pathology and Genetics of Tumours of Haemopoietic and Lymphoid Tissues, 2001, pp 154 - 156.
Common drugs used include alkylating agents such as chlorambucil, melphalan, and cyclophosphamide, but patient responses have been inconsistent. More recently, fludarabine phosphate (Fludara) has been used.
Topic Code: 5615