Abstract
IntroductionRefractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation is rarely reported and has a poor prognosis in general (a median survival of 1.6 months). Moreover, the optimum treatment for this condition is still undecided. This is the first report on the successful use of vincristine, adriamycin and dexamethasone chemotherapy for refractory pleural effusion due to systemic immunoglobulin light chain amyloidosis without cardiac decompensation.Case presentationWe report the case of a 68-year old Japanese male with systemic immunoglobulin light chain amyloidosis presenting with bilateral pleural effusion (more severe on the right side) in the absence of cardiac decompensation that was refractory to diuretic therapy. The patient was admitted for fatigue, exertional dyspnea, and bilateral lower extremity edema. He had been receiving intermittent melphalan and prednisone chemotherapy for seven years. One month before admission, his dyspnea had got worse, and his chest radiograph showed bilateral pleural effusion; the pleural effusion was ascertained to be a transudate. The conventionally used therapeutic measures, including diuretics and thoracocentesis, failed to control pleural effusion. Administration of vincristine, adriamycin, and dexamethasone chemotherapy led to successful resolution of the effusion.ConclusionTreatment with vincristine, adriamycin, and dexamethasone chemotherapy was effective for the refractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation and appears to be associated with improvement in our patient's prognosis.
Highlights
ConclusionAdriamycin, and dexamethasone chemotherapy was effective for the refractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation and appears to be associated with improvement in our patient’s prognosis
Refractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation is rarely reported and has a poor prognosis in general
Treatment with vincristine, adriamycin, and dexamethasone chemotherapy was effective for the refractory pleural effusion in systemic immunoglobulin light chain amyloidosis without cardiac decompensation and appears to be associated with improvement in our patient’s prognosis
Summary
Management of patients with systemic AL amyloidosis who present with refractory pleural effusion is extremely difficult and is associated with a poor prognosis. To the best of our knowledge, this is the first report on the administration of VAD chemotherapy for refractory pleural effusion in systemic AL amyloidosis without cardiac decompensation. This treatment may be effective and afford a high quality of life for the patient. VAD chemotherapy is known to improve the prognosis of systemic AL amyloidosis, and the Guideline Working Group of United Kingdom, Myeloma Forum, recommends VAD chemotherapy as the first-line therapy for patients aged under 70 years [17]. We suggest that VAD chemotherapy is clinically useful for regulating systemic AL amyloidosis-associated refractory pleural effusion.
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