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Published on: July 31, 2016
Gamma heavy chain disease complicated by pulmonary hypertension, which was successfully treated with lenalidomide
1Hematology, Tazuke Kofukai, Medical Research Institute, Kitano Hospital, Osaka, Japan ashitaharerukana21@gmail.com.
Insights
This study reports a rare case of gamma heavy chain disease (HCD) in a patient with pulmonary hypertension. Lenalidomide and dexamethasone therapy demonstrated long-term disease control, marking a potential new treatment option.
Area of Science:
- Hematology
- Oncology
- Rare Diseases
Background:
- Heavy chain disease (HCD) is a rare B-cell neoplasm characterized by truncated immunoglobulin heavy chains.
- Gamma HCD is a specific subtype, often presenting with nonspecific symptoms.
- Pulmonary hypertension is a serious complication that can affect patient prognosis.
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.
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