Alpha Heavy Chain Disease Post-Diffuse Large B-cell Lymphoma Remission: A Series of Two Cases

Abstract

Abstract Introduction/Objective Heavy chain disease is the production of abnormal monoclonal immunoglobulin heavy chains. Alpha heavy chain disease is typically identified in mucosa-associated lymphoid tissue lymphomas and is secreted due to chronic infection. The alpha heavy chain can manifest as hypogammaglobulinemia or a normal serum protein electrophoresis (SPEP). Broadband may appear in the alpha-2 or beta mobility regions. Methods/Case Report We describe two cases in which a monoclonal alpha heavy chain was detected in a 60-year-old female with diffuse large B-cell lymphoma (DLBCL) of the thoracic spine and a 71-year-old female with mediastinal DLBCL. Both patients achieved clinical and radiological remission. Results (if a Case Study enter NA) A distinct IgA band without an associated light chain was detected on the first patient's serum immunofixation essay (SIFE) one year later. For the second case, there was a distortion of the beta-2 region, elevated kappa free light chain (230.6 mg/dL), and a kappa to lambda free light chain ratio of 14.23, suggesting alpha heavy chain disease and a possible monoclonal gammopathy of unknown significance. Interestingly, even with a monoclonal IgA, the patients remained symptom-free, without any gastrointestinal manifestations, and concurrent PET scans show no recurrence. Conclusion To the best of our knowledge, these are the first two cases describing the presence of alpha heavy chain disease in association with DLBCL. The significance of alpha heavy chain secretion in patients with a history of DLBCL needs to be further assessed and we need to identify the possible presence of a non-clonally related paraprotein post-treatment for DLBCL.