Gamma heavy chain disease complicated by pulmonary hypertension, which was successfully treated with lenalidomide

Abstract

Heavy chain disease (HCD) is a rare B-cell proliferative neoplasm that is characterised by the production of truncated monoclonal immunoglobulin heavy chains without light chains. Gamma HCD is a subgroup of HCD. A 67-year-old man was admitted to our hospital with dyspnoea and lower leg oedema. Based on the results of heart catheterisation, he was diagnosed with pulmonary hypertension. Laboratory tests revealed an elevated level of IgG, and serum immunoelectrophoresis showed that IgG was a monoclonal gamma heavy chain without light chains. Finally, he was diagnosed with gamma HCD complicated by pulmonary hypertension. Bortezomib and dexamethasone therapy was initiated, but became refractory within 8 months. Therefore, the treatment was switched to lenalidomide and dexamethasone therapy, and the disease has been stably controlled for more than 2 years. To the best of our knowledge, this is the first case of gamma HCD being successfully treated by lenalidomide and dexamethasone therapy.

Gamma heavy chain disease: a retrospective analysis of 6 cases

“…Perhaps patients with autoimmune disorders were more likely to be tested by immunofixation as a baseline assessment. In addition, combined pulmonary hypertension, systemic sclerosis, inflammatory myopathy, and mixed connective tissue disease were also reported in several cases [6][7][8][9].…”