Unusual heavy chain disease protein in a patient with splenic marginal-zone lymphoma

Ann Clin Biochem 2006;43:161-164
doi:10.1258/000456306776021490
© 2006 Association for Clinical Biochemistry

 

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Keiko Morita,
Hiromi Kawamoto,
Honami Takada,
Shinobu Nakamura,
Kazuyoshi Ishii and
Yasuyuki Okamoto


Central Clinical Laboratory, Nara Medical University, Shijo-Cho 840, Kashihara, Nara 634-8522, Japan;
Central Clinical Laboratory, Nara Medical University, Shijo-Cho 840, Kashihara, Nara 634-8522, Japan;
Central Clinical Laboratory, Nara Medical University, Shijo-Cho 840, Kashihara, Nara 634-8522, Japan;
Department of General Medicine and Clinical Investigation, Nara Medical University, Shijo-Cho 840, Kashihara, Nara 634-8522, Japan;
Department of Haematology, Kishiwada City Hospital, Gakuhara-Cho 1001, Kishiwada, Osaka 596-8501, Japan;
Central Clinical Laboratory, Nara Medical University, Shijo-Cho 840, Kashihara, Nara 634-8522, Japan

We conducted an electrophoretic analysis of monoclonal -globulin found in the serum of a patient with splenic marginal-zone lymphoma. This monoclonal protein showed electrophoretic mobility to the region and reacted with antii-mmunoglobulin (IgG) antiserum but not with anti- or anti- light chain antisera. Sodium dodecyl sulphate-polyacrylamide gel electrophoresis (SDS-PAGE) and Western blotting of the monoclonal protein-rich -globulin fraction extracted from the sliced gel revealed the presence of two types of abnormal IgG molecule, low- and high-molecular-weight IgG, neither of which reacted with anti- or anti- light chain antisera. Additionally, an abnormal high-molecular-weight heavy chain was identified by reducing SDS-PAGE. These findings suggest that this monoclonal protein is composed only of heavy chains of normal and larger size. The presence of abnormal serum immunoglobulin composed of only heavy chain has been known as a fundamental feature of heavy chain disease (HCD). However, the unique composition of monoclonal -globulin makes our case distinct from typical HCD, which is characterized by an abnormal truncated low-molecular-weight heavy chain. Thus, the unusual monoclonal protein may havebeen produced by a somatic mutation of IgH gene associated withsplenic marginal-zone lymphoma.

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