Canterbury DHB

Context

Heavy Chain Disease

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.

About this Canterbury DHB document (5615):

Document Owner:

Sean Macpherson (see Who's Who)

Issue Date:

September 2014

Next Review:

September 2016

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 5615