Multiple Myeloma with Monoclonal Free IgG3 Heavy Chains and Free Kappa Light Chains

作者：Richter Alex G^*; Harding Stephen; Huissoon Aarnoud; Drayson Mark; Pratt Guy

来源：Acta Haematologica, 2010, 123(3): 158-161.

DOI：10.1159/000292899

• Heavy chain disease
• Immunoglobulins
• Light chains
• Multiple myeloma
• Truncation in heavy chains

We describe the case of a 34-year-old gentleman investigated for persistent neutropaenia following two episodes of pneumonia. Specialist investigations led to the diagnosis of multiple myeloma (MM) producing a truncated monoclonal gamma(3) heavy chain (HC) immunoglobulin molecule unattached to a light chain (LC) with atypical features for both MM and HC disease. Western blot showed gamma(3)HC was truncated with a large deletion (75 kDa). Flow cytometry of the bone marrow aspirate revealed an unusual staining pattern. This plasma cell dyscrasia was also unusual in that a subpopulation (30%) secreted large quantities of free LC (FLC) as well as truncated IgG HC. This is the first description, investigation and treatment of MM with a plasma cell population producing truncated gamma(3)HC and kappa FLC M-proteins and illustrates a number of unique immunological and clinical features.

• 出版日期2010