Challenging differential diagnosis, Rare disease, Educational Purpose (only if useful for a systematic review or synthesis), Rare co-existance of disease or pathology
Meera Mohan, Murat Gokden , Neriman Gokden, Carolina Schinke
(Myeloma Institute, University of Arkansas for Medical Sciences, Little Rock, AR, USA)
Am J Case Rep 2016; 17:173-176
Light chain deposition disease is a systemic disease characterized by deposition of immunoglobin light chains in various organs. Cardiac involvement of light chain deposition disease, also known as cardiac nonamyloidotic immunoglobin deposition disease (CIDD), is a rare clinical entity, where clinical outcome is very variable and best treatment approaches are not well known.
CASE REPORT: We present the case of a 31-year-old man with a solitary thoracic plasmacytoma and cardiac light chain deposition disease with evidence of congestive heart failure by echocardiography and cardiac markers. The patient underwent surgical resection of the plasmacytoma followed by systemic therapy with 50% VDT-PACE and then VRD with near-normalization of his heart function. A melphalan-based stem cell transplant is planned in this young patient to achieve the best possible long-term remission.
CONCLUSIONS: CIDD is a very rare disease, with previous reports showing diverse manifestations with variable outcome. A high level of clinical suspicion should be maintained in such cases and early intervention, as in our patient, can restore cardiac function. There is very little literature on the optimal management of these patients. A combination of surgery and chemotherapy were pursued in our patient with very good results.
Keywords: Cardiomyopathy, Restrictive - etiology, Adult, Humans, Immunoglobulin Light Chains - metabolism, Male, Paraproteinemias - complications, Plasmacytoma - complications, Thoracic Neoplasms - complications