ABSTRACT
Light chain deposition disease (LCDD) is a rare pathologic condition distinct from amyloidosis. Amyloidosis is most often characterized by overproduction of lambda light chains, while kappa chains are overproduced in LCDD. In contrast to amyloid deposits, those of LCDD do not stain with Congo red and have a granular ultrastructure. LCDD primarily affects the kidney; clinically significant liver dysfunction is less common and less severe than renal disease. We describe a case of kappa chain deposition disease in a patient with plasma cell dyscrasia and platelet pool storage defect, which produced massive hepatomegaly and rupture of the liver leading to orthotopic liver transplantation. The liver weighed 6800 g and showed severe atrophy due to massive deposition of light chains. In this case, the deposits were composed of unbranched fibrils, which measured 12 to 20 nm in width, did not possess a hollow core, and were arranged randomly rather than in structured arrays.
Subject(s)
Hepatomegaly/metabolism , Immunoglobulin kappa-Chains/metabolism , Liver Transplantation , Adult , Female , Hepatomegaly/diagnosis , Humans , Liver/pathology , Liver/surgery , Platelet Storage Pool Deficiency/diagnosis , Platelet Storage Pool Deficiency/metabolism , Rupture, Spontaneous/etiology , Rupture, Spontaneous/surgeryABSTRACT
Primary pulmonary artery sarcomas are uncommon neoplasms with up to 20% of cases being leiomyosarcomas. In many instances, the diagnosis is difficult and delayed. We report the case of a 75-year-old male who presented with symptoms suggestive of recurrent pulmonary thromboemboli, in which a primary leiomyosarcoma of the pulmonary artery was successfully removed surgically. Three years after surgery, the patient is well and with no evidence of recurrence or metastatic disease.
